case study questions in hematology

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9. Hematology Questions and Answers

• Published: August 2013
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Chapter 9 presents multiple-choice, board review questions on hematology including anemia, myeloid malignancies, coagulation disorders, and lymphoid malignancies. Full explanations are provided with the correct answers.

Multiple Choice (choose the best answer)

Anemias and Myeloid Malignancies

1. A 67-year-old man is evaluated for exertional dyspnea. He recalls that 3 years ago he was told that he had anemia. In reviewing his records, you note that at that time his hemoglobin level was 9.5 g/dL and his hematocrit was 33% with an increased mean corpuscular volume (MCV); the remainder of his complete blood cell count was normal. On physical examination, he had conjunctival pallor, normal heart and lung findings, no lymphadenopathy, no hepatomegaly or splenomegaly, and no petechiae or ecchymoses. Diagnostic testing results are shown in Table 9.Q1 .

Which of the following is the most likely explanation for these findings?

Acute myeloid leukemia (AML)

Vitamin B 12 deficiency

Hemolytic anemia

Myelodysplastic syndrome (MDS)

Primary myelofibrosis

2. A 45-year-old woman is admitted to the surgical service with severe arterial insufficiency of the right second toe. She has no prior medical history and takes no medications. Physical examination findings are normal except for mild splenomegaly and signs of early gangrene in the right second toe. All pulses are full and equal throughout. Diagnostic testing results are shown in Table 9.Q2 .

Which of the following is the most likely diagnosis?

Essential thrombocythemia

Philadelphia chromosome–negative chronic myeloid leukemia (CML)

Primary myelofibrosis (PMF)

3. A 22-year-old man is admitted to the hospital for an elective cholecystectomy. You are asked to see him because he had anemia on preoperative testing. He tells you that he has always been told by his physicians that he has mild anemia; his medical history is otherwise unremarkable. His vital signs are normal. His conjunctivae are mildly icteric, and the spleen is palpable in the left upper quadrant. Findings on the remainder of the physical examination are normal. Diagnostic testing results are shown in Table 9.Q3 .

Which of the following tests would most likely help confirm the diagnosis?

Hemoglobin electrophoresis

Osmotic fragility test

Direct and indirect antiglobulin (Coombs) tests

Bone marrow aspiration and biopsy

4. A 28-year-old black man with sickle cell disease presents to the emergency department with abdominal pain, chest pain, and shortness of breath. His dyspnea evolved over 36 hours after a visit with his niece and nephew. His history is significant for approximately 2 emergency department visits or hospital admissions per year for painful crises. Three years ago, he spent 4 weeks in the hospital after an episode of acute chest syndrome. He has been taking hydroxyurea but only intermittently because of financial concerns. His pulse is 116 beats per minute and regular, his blood pressure is 138/76 mm Hg, his respiratory rate is 18 breaths per minute, and his temperature is 38.3°C. Pulse oximetry shows 91% oxygen saturation with room air and 93% with 4 L of oxygen by nasal cannula. His lungs have scattered inspiratory crackles in the right midlung field. His spleen is not palpable. The remainder of the physical examination findings are normal. Diagnostic testing results are shown in Table 9.Q4 .

A chest radiograph shows a right middle and upper lobe air space infiltrate. The patient is given supplemental oxygen, adequate pain control, and intravenous antibiotics. Which of the following should you now order?

Hydroxyurea

Erythrocyte exchange transfusion

Plasma exchange

Anticoagulation with unfractionated heparin

Aggressive intravenous fluid hydration

5. A 70-year-old man presents with weakness of his right arm and leg. His symptoms began yesterday and are now resolved. He also reports a 6-month history of recurrent headaches and fatigue. He is a nonsmoker. His medical history is significant for high blood pressure. His blood pressure is 167/88 mm Hg, his oxygen saturation is 93% on room air, his face is plethoric, and a right carotid bruit is heard. Other findings on physical examination are normal. Diagnostic testing results are shown in Table 9.Q5 .

Carotid ultrasonography shows a 30% stenotic lesion in the right carotid. The patient is hospitalized and begins antiplatelet therapy. Which of the following should you order next?

JAK2 V617F mutation testing

Fluorescence in situ hybridization (FISH) for BCR-ABL testing

Arterial blood gas analysis

6. A 42-year-old woman with a history of systemic lupus erythematosus (SLE) presents with fatigue. She has been receiving anti–tumor necrosis factor therapy and has been managing the SLE well. However, she has recently experienced worsening fatigue. Her vital signs are normal. Her face and conjunctivae are jaundiced, and she has a fading butterfly rash on her face. The spleen is palpable on deep inspiration. Diagnostic testing results are shown in Table 9.Q6 , and the peripheral blood film is shown in Figure 9.Q6 .

Which of the following is the best interpretation of these data?

The hemolysis is predominantly intravascular.

The bone marrow is not responding to the anemia.

Direct Coombs testing results should be positive.

Urine hemoglobin testing results should be positive.

7. A 58-year-old woman with active rheumatoid arthritis presents with fatigue and joint pain. She received the diagnosis of rheumatoid arthritis 5 years earlier and has been taking prednisone 10 mg daily and methotrexate with folate weekly. She has had chronic fatigue and anemia. Her vital signs are normal. Her conjunctivae are pale, and she has active synovitis affecting both knees, her wrists, and elbows, with rheumatoid nodules on the extensor surface of her right forearm. The remainder of the physical examination findings are normal. Diagnostic testing results are shown in Table 9.Q7 .

Which of the following laboratory findings are consistent with this condition?

Elevated hepcidin, elevated ferritin, elevated total iron-binding capacity (TIBC), elevated serum iron

Elevated hepcidin, elevated ferritin, decreased TIBC, elevated serum iron

Decreased hepcidin, elevated ferritin, decreased TIBC, elevated serum iron

Elevated hepcidin, elevated ferritin, decreased TIBC, normal serum iron

Decreased hepcidin, elevated ferritin, elevated TIBC, normal serum iron

Coagulation

8. A 62-year-old man underwent right total knee replacement 8 days ago. Swelling has developed in his right lower extremity, and Doppler ultrasonography confirms the presence of a right superficial femoral vein thrombosis. His current medications include oxycodone and subcutaneous unfractionated heparin. Results of preoperative tests, including a complete blood cell count and liver and kidney function, were normal. Other laboratory data include the following: hemoglobin 12.2 g/dL, leukocyte count 8.5×10 9 /L, and platelet count 60×10 9 /L. In addition to stopping the use of subcutaneous heparin, what is the next most appropriate step in management of this patient?

Start low-molecular-weight heparin therapy.

Start intravenous therapeutic doses of heparin.

Start direct thrombin inhibitor therapy.

Start aspirin therapy.

9. A 45-year-old man presents with deep vein thrombosis of the right femoral vein. Three months ago, he received a diagnosis of systemic lupus erythematosus (SLE). In addition to confirming SLE, laboratory testing also documented the presence of a lupus anticoagulant (LAC). There is no family history of venous thrombosis. Current medications include hydroxychloroquine. Laboratory testing shows normal results for a complete blood cell count and for tests of liver and kidney function. Special coagulation testing confirms the persistence of an LAC. What is the most reasonable duration of warfarin anticoagulation for this patient?

10. A 20-year-old white woman has been admitted to the hospital with pulmonary embolism. She has no chronic illnesses and is receiving no medications except for combination estrogen-progesterone birth control pills that she started using approximately 1 year earlier. Results were normal for a complete blood cell count, baseline prothrombin time, activated partial thromboplastin time (aPTT), and tests of kidney and liver function. The patient is currently receiving therapeutic doses of intravenous unfractionated heparin, and her aPTT is therapeutic at 72 seconds. A panel of thrombophilia tests has been performed. Which of the following statements about her thrombophilia test results is correct?

DNA-based testing for factor V Leiden and prothrombin G20210A mutations are reliable.

Low antithrombin confirms a hereditary deficiency state.

A positive result on lupus anticoagulant (LAC) testing confirms antiphospholipid antibody syndrome.

Low protein S confirms the presence of a hereditary deficiency state.

11. A 62-year-old man with chronic atrial fibrillation has been treated with warfarin. He has no other chronic illnesses and is receiving no other medications long-term except for lipid-lowering agents. Results of his complete blood cell count and tests of renal and kidney function are normal. He checks his prothrombin time monthly and has kept the international normalized ratio (INR) within the therapeutic range (2–3) for the duration of his therapy with warfarin. He has heard about recent US Food and Drug Administration (FDA) approval of dabigatran, which requires no monitoring, and he would like a prescription for this new drug. Which of the following statements is true about the use of dabigatran in atrial fibrillation compared with the well-managed use of warfarin?

Switching to dabigatran would result in superior outcomes.

Switching to dabigatran would result in inferior outcomes.

Switching to dabigatran would provide no significant benefit.

Dabigatran is FDA approved for postoperative thromboprophylaxis for knee and hip replacement surgery.

Dabigatran is FDA approved as an anticoagulant for patients who have received a mechanical heart valve.

12. A 22-year-old woman is brought to the emergency department after having 1 witnessed tonic-clonic seizure. She had appeared confused for the preceding few hours. On examination, she is febrile and appears slightly confused; otherwise, neurologic and physical examination findings are normal. Laboratory testing results are shown in Table 9.Q12 , and the peripheral blood smear is shown in Figure 9.Q12 .

What is the most appropriate next step in management?

Red blood cell transfusion

Platelet transfusion

Gamma globulin administration

13. A 72-year-old man with chronic atrial fibrillation has been receiving dabigatran 75 mg twice daily for the past 6 months. He has not had any thrombotic or hemorrhagic complications. He has a history of colon polyps, for which he needs to undergo a colonoscopy with possible polypectomy. Apart from an irregular pulse, his physical examination findings are normal. Results were normal for a complete blood cell count and tests of renal and liver function. The calculated creatinine clearance is 28 mL/min. For how long should dabigatran use be discontinued before the colonoscopy?

No need to discontinue

Lymphoid Malignancies

14. At her annual physical examination, an asymptomatic 68-year-old woman has lymphocytosis (32×10 9 /L) with a normal hemoglobin level and platelet count. On examination, she has 1-cm lymphadenopathy in the cervical region and no palpable liver or spleen enlargement. A peripheral blood smear shows identically appearing mature lymphocytes with smudge cells. Flow cytometry of the peripheral blood lymphocytes shows a monoclonal B population with dim expression of λ light chain and CD20 that is positive for expression of CD5, CD19, and CD23. Which of the following is the best next step in her management?

Combination chemoimmunotherapy

Chlorambucil therapy

Allogeneic peripheral blood stem cell transplant

Combination monoclonal antibody therapy

Active monitoring for disease progression and complications

15. Ten years ago, a previously healthy 20-year-old woman presented to her physician with a 2-month history of pruritis, drenching night sweats, unintentional weight loss, and nonproductive cough. On examination, she had 2-cm cervical lymphadenopathy. A computed tomographic scan showed a 12-cm-diameter anterior mediastinal mass. An excisional biopsy of a cervical lymph node showed nodular sclerosing Hodgkin lymphoma. After she was treated with ABVD (doxorubicin [ A driamycin], b leomycin, v inblastine, and d acarbazine) combination chemotherapy followed by involved field radiotherapy, the disease was in complete remission. Now you see her for the first time for an annual physical examination. The disease remains in complete remission. Compared to her peers, this patient is at increased risk of which of the following conditions?

Breast cancer

Coronary artery disease

Hypothyroidism

Skin cancer

All of the above

16. An 80-year-old man is admitted to the hospital after falling on an icy sidewalk and fracturing his hip. He undergoes open reduction and internal fixation of the fracture. At surgery, there does not appear to be any bone disease at the fracture site. The patient was previously asymptomatic. Physical examination findings are otherwise unremarkable. Serum protein electrophoresis and immunofixation show an IgM κ monoclonal protein (0.3 g/dL). The complete blood cell count and serum creatinine levels are normal. Skeletal survey shows no additional bone defects. Which of the following statements is true for this patient?

He has multiple myeloma and requires treatment.

He has a lower risk of a clinically significant lymphocytic or plasma cell malignancy than patients with an IgG monoclonal protein.

He requires a radioisotope bone scan to evaluate his bone integrity.

He requires regular follow-up and serial measurements of his monoclonal protein level.

He has a 10% annual risk of multiple myeloma.

17. A 75-year-old African American man was seen last week by his primary care physician for mild dyspnea. He has also noted intermittent peripheral edema. During the evaluation, an electrocardiogram showed low-voltage QRS complexes in the limb leads. The troponin T level was elevated (0.07 ng/mL). This finding suggested the need for a coronary angiogram, which showed no significant coronary artery disease. An echocardiogram showed diffuse left ventricular thickening with a granular texture to the myocardium and a septal thickness of 2.5 cm (normal <1.1 cm). The complete blood cell count results were normal. Serum and urine protein electrophoresis and immunofixation were unremarkable. Serum free light chain levels were not increased. What is the most likely diagnosis?

AA amyloidosis

Light chain–related amyloidosis

Hypertrophic obstructive cardiomyopathy

Amyloidosis due to transthyretin deposition

Amyloidosis due to β 2 -microglobulin deposition

18. A 55-year-old man presented to his primary care physician for evaluation of fatigue. He was previously healthy with the exception of chronic musculoskeletal low back pain, for which he occasionally takes nonsteroidal anti-inflammatory drugs. On examination, he is pale. Complete blood cell count results are as follows: hemoglobin 8.3 g/dL, mean corpuscular volume 73 fL, leukocyte count 6.9×10 9 /L, and platelet count 398×10 9 /L. Results of the fecal occult blood test are positive. During upper and lower endoscopy, a 1.2×2.5-cm ulcerative lesion is noted in the lesser curvature of the stomach. The lesion is biopsied and identified as a MALT lymphoma. Which of the following is characteristic of MALT lymphoma?

Most cases are treated with anthracycline-based chemotherapy.

It is caused by chronic stimulation with Chlamydophila psittaci .

Radiotherapy is necessary in most cases.

It frequently undergoes transformation to a large-cell lymphoma.

The combination of amoxicillin, omeprazole, and clarithromycin is the most appropriate first-line treatment.

19. A 73-year-old woman presented to the emergency department with new-onset back pain, confusion, and constipation over the past week. Her past medical history is significant only for hypertension. On examination, she is slightly pale with slow cognition and point tenderness over the lumbar spine. Plain films of the lumbar spine show osteolytic lesions in L2, L3, and L5. Laboratory values are as follows: hemoglobin 9.3 g/dL, leukocyte count 4.6×10 9 /L with a normal differential count, platelet count 230×10 9 /L, creatinine 1.6 mg/dL, total calcium 13.1 mg/dL, albumin 3.6 g/dL, and total protein 9.1 g/dL. What is the most likely diagnosis?

Metastatic breast cancer

Hydrochlorothiazide use

Multiple myeloma

Primary hyperparathyroidism

Milk alkali syndrome

20. A 48-year-old man presents to the emergency department with a 6-week history of progressively worsening abdominal pain and night sweats. Physical examination findings were significant for palpable bilateral 2-cm axillary lymph nodes and diffuse abdominal tenderness with no rebound or guarding. Computed tomography of the abdomen and pelvis showed retroperitoneal and mesenteric lymphadenopathy. Excisional biopsy of an axillary node was positive for diffuse, large B-cell lymphoma. Positron emission tomography showed fluorodeoxyglucose-avidity in the axillary, mesenteric, and retroperitoneal lymph nodes. Results of the bone marrow examination were normal. Which of the following is the best next step?

Combination therapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP)

Observation

Combination therapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)

Autologous stem cell transplant

Involved field radiotherapy

1. Answer d.

MDS most commonly manifests as isolated macrocytic anemia. MDS can evolve to include pancytopenia over several years; the typical peripheral smear findings include a dimorphic erythrocyte population (microcytes and oval macrocytes) with an overall prominent macrocytosis and an MCV around 110 fL. The chronicity of MDS—in particular, anemia preceding the diagnosis of pancytopenia by several years—is in contrast to the typically acute manifestation of AML, which is therefore an unlikely possibility in this patient. Primary myelofibrosis, a myeloproliferative neoplasm, causes fibrosis in the bone marrow, resulting in extramedullary hematopoiesis and significant splenomegaly, and typically does not cause a macrocytic anemia. Vitamin B 12 deficiency can cause a megaloblastic anemia and manifest with slowly evolving macrocytic anemia and eventually pancytopenia, but the peripheral smear would not show a dimorphic erythrocyte population. (See Tefferi and Vardiman in the “Suggested Reading” list.)

2. Answer a.

Extreme thrombocytosis may be reactive and occur with severe iron deficiency or inflammatory states (with elevated erythrocyte sedimentation rates) or after splenectomy; patients are typically asymptomatic. Clonal thrombocytosis is related to a myeloproliferative neoplasm, which usually causes splenomegaly. Typical bone marrow findings include a hypercellular bone marrow with increased atypical megakaryocytes in clusters. Essential thrombocythemia may cause extreme thrombocytosis (platelet count >1,000×10 9 /L); however, it can also occur less commonly with polycythemia rubra vera (typically with erythrocytosis), the cellular phase of PMF, or rarely CML. The normal karyotype makes CML much less likely since it typically manifests with the Philadelphia chromosome t(9;22). Increased reticulin fibrosis would have been seen on the bone marrow biopsy if the patient had PMF. (See Tefferi in the “Suggested Reading” list.)

3. Answer b.

When a patient presents with premature gallstones, one should consider whether they may be due to pigment gallstones from chronic hemolysis causing indirect hyperbilirubinemia. The presence of microspherocytes is consistent with hereditary spherocytosis, and the diagnostic test is an osmotic fragility test, which identifies a congenital membrane defect. Typically, acquired warm autoimmune hemolytic anemia, which produces positive Coombs test results, can cause spherocytes as well; however, the history of lifelong anemia makes this diagnosis unlikely. A hemoglobin electrophoresis would help in diagnosing thalassemia or a hemoglobinopathy; however, these conditions do not manifest with microspherocytes on the peripheral blood film. There is no indication for a bone marrow biopsy since the reticulocyte response is appropriate and no other cytopenias are apparent. (See Gallagher in the “Suggested Reading” list.)

4. Answer b.

The patient has acute chest syndrome, a sickle cell anemia complication that is an indication for urgent red cell (not plasma) exchange transfusion to decrease the hemoglobin S level to less than 30% to 35%. Gentle fluid resuscitation is appropriate (along with oxygen support and antibiotics, since about one-third of acute chest syndrome events are initiated by or associated with bacterial pneumonia). Aggressive fluid resuscitation, leading to overhydration, might cause pulmonary edema and worsen the oxygenation. Pulmonary embolism is possible, but full anticoagulation is not warranted until embolism is documented. Use of hydroxyurea might have prevented this crisis, but it is of no value for the acute condition. (See Vij and Machado in the “Suggested Reading” list.)

5. Answer a.

Polycythemia may be secondary, as with erythropoietin- mediated causes such as chronic hypoxemia, living at high altitude, and high oxygen affinity hemoglobinopathies. Polycythemia vera is a myeloproliferative neoplasm that can manifest with arterial thrombosis secondary to hyperviscosity from the increased concentration of erythrocytes. The low erythropoietin rules out erythropoietin-mediated causes, leaving the presumptive diagnosis of polycythemia vera. With JAK2 V617F mutation testing of peripheral blood, results are positive for approximately 90% of patients who have polycythemia vera. FISH for BCR-ABL testing would screen for chronic myeloid leukemia, which does not manifest with polycythemia. Although bone marrow aspiration and biopsy would be helpful, it is not immediately necessary and could be considered later. (See Patnaik and Tefferi in the “Suggested Reading” list.)

6. Answer c.

Hematologic complications of SLE include anemia of chronic disease, pure red cell aplasia, and warm autoimmune hemolytic anemia (WAIHA). The presentation and laboratory data suggest hemolysis, and the blood smear shows spherocytes. These findings are consistent with WAIHA, which causes extravascular hemolysis. The reticulocytosis suggests that the bone marrow response is adequate. In intravascular hemolysis, the urine is positive for hemoglobin. (See Packman in the “Suggested Reading” list.)

7. Answer d.

Rheumatoid arthritis is a chronic inflammatory disorder that may lead to anemia of chronic disease. Anemia of chronic disease results from the effect of elevated cytokines on hematopoiesis, including upregulation of hepcidin, leading to increased ferritin from iron malutilization and downregulation of ferroportin, the main iron exporting system. Transferrin is also downregulated, leading to decreased TIBC and normal to decreased serum iron levels. (See Weiss and Goodnough in the “Suggested Reading” list.)

8. Answer c.

The timing and degree of thrombocytopenia are consistent with immune-mediated heparin-induced thrombocytopenia type II. Unfractioned heparin and low-molecular-weight heparin are contraindicated. Aspirin would not be the sole management agent for established thrombosis. The most appropriate step is to start a direct thrombin inhibitor.

9. Answer d.

Presentation with a vascular thrombosis and persistence of a LAC for 12 weeks or more satisfies the criteria for an antiphospholipid syndrome. This patient has a high risk for recurrent venous thrombosis on discontinuing anticoagulation; thus, long-term warfarin is recommended with periodic reassessment for safety.

10. Answer a.

DNA-based testing is reliable for patients receiving heparin or warfarin and for patients who have acute thrombosis. However, acute thrombosis and heparin can cause lower antithrombin activity results, which should be verified at another time, when heparin and acute thrombosis are not factors. A single positive test result for LAC does not confirm antiphospholipid syndrome; follow-up testing at 12-week intervals is required to demonstrate persistence of LAC. Acute thrombosis and estrogen use can lower protein S levels; thus, abnormally low results require follow-up confirmation.

11. Answer c.

Among patients randomly assigned to receive dabigatran, overall outcomes were noninferior when compared with the well-managed use of warfarin, thus providing no significant advantages. The group of patients that derived the most benefit from dabigatran was the group with INRs outside the recommended therapeutic range. Dabigatran is FDA approved only to reduce the risk of stroke and systemic embolism in patients with nonvalvular atrial fibrillation.

12. Answer d.

Plasma exchange is the treatment of choice for thrombotic thrombocytopenic purpura (TTP). Although red blood cell transfusion may be indicated, it does not address the underlying pathogenesis of TTP. Platelets are thought to be contraindicated in TTP because of the theoretical possibility of worsening the TTP. Gamma globulin is ineffective in increasing the platelet count in TTP.

13. Answer d.

Dabigatran is cleared through the kidneys. It has a prolonged half-life in patients who have a creatinine clearance less than 30 mL/min compared with patients who have a creatinine clearance greater than 30 mL/min.

14. Answer e.

Chronic lymphocytic leukemia (CLL) is a clonal lymphoproliferative disorder of mature lymphocytes. The clinical diagnosis requires a B-lymphocyte count of more than 5×10 9 /L. Peripheral blood smears typically show smudge cells, which are lymphocytes that have broken during processing of the slide. The clinical course of CLL is chronic in most patients. For those with early-stage disease, standard practice is to withhold treatment until the disease is active or progressive. However, patients need to be monitored for disease progression, autoimmune complications, infections, and second cancers.

15. Answer e.

Hodgkin lymphoma therapy is curative in about 80% of cases. However, there are late complications of therapy, particularly in those treated before modern chemotherapy and radiotherapy. At 15 years, the risk of death from other causes surpasses that of risk of death from Hodgkin lymphoma. Patients are at higher risk of secondary malignancies, cardiovascular disease, thyroid disorders, and infertility than the general population. Many of these conditions can be attributed to chemotherapy and radiotherapy.

16. Answer d.

This patient has monoclonal gammopathy of undetermined significance (MGUS), the most common dysproteinemia. In MGUS, the M protein level is typically less than 3 g/dL, the bone marrow has less than 10% plasma cells, and the hemoglobin, creatinine, calcium, and bone radiographs are normal. The risk of progression to a lymphocytic or plasma cell malignancy is about 1% per year. Patients with an IgM or IgA monoclonal protein are at higher risk of progression than those with an IgG protein. Patients with MGUS need to be observed.

17. Answer d.

The patient has senile cardiac amyloidosis. This syndrome is usually isolated to the heart with few clinically significant deposits elsewhere, and the echocardiographic findings are often out of proportion to the degree of symptoms. Transthyretin is the protein causing the amyloid deposits; most patients have wild-type transthyretin.

18. Answer e.

With combination antibiotic therapy, 70% of gastric MALT lymphomas are cured. In cases refractory to antibiotics, tumors may carry the t(11;18) translocation, and involved field radiotherapy is effective. Combination chemotherapy is reserved for advanced disease. The majority of cases are associated with Helicobacter pylori infection.

19. Answer c.

This patient has multiple myeloma with evidence of end-organ damage from the plasma cell proliferative disorder (hypercalcemia, renal failure, anemia, and osteolytic bone lesions). The other answer choices are possible causes of hypercalcemia, but only multiple myeloma accounts for all the presenting symptoms, including the elevated level of total protein.

20. Answer a.

This patient has advanced-stage, diffuse, large B-cell lymphoma, and R-CHOP chemotherapy is the standard of care. Rituximab is an anti-CD20 monoclonal antibody that improves overall survival when added to CHOP chemotherapy for aggressive B-cell lymphomas. For patients whose disease relapses or is refractory, autologous stem cell transplant is the standard therapy. Radiotherapy can be used in combination with chemotherapy in early-stage (I-IIA) nonbulky disease but is not standard therapy for advanced disease.

Suggested Reading

Gallagher PG . Red cell membrane disorders. Hematology Am Soc Hematol Educ Program.   2005 :13–8.

Packman CH . Hemolytic anemia due to warm autoantibodies.   Blood Rev.   2008 Jan;22(1):17–31. Epub 2007 Sep 27.

Google Scholar

Patnaik MM , Tefferi A . The complete evaluation of erythrocytosis: congenital and acquired.   Leukemia.   2009 May;23(5):834–44. Epub 2009 Mar 19.

Tefferi A . Annual clinical updates in hematological malignancies : a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management. Am J Hematol. 2011 Mar;86(3):292–301.

Tefferi A , Vardiman JW . Myelodysplastic syndromes.   N Engl J Med.   2009 Nov 5;361(19):1872–85.

Vij R , Machado RF . Pulmonary complications of hemoglobinopathies.   Chest.   2010 Oct;138(4):973–83.

Weiss G , Goodnough LT . Anemia of chronic disease.   N Engl J Med.   2005 Mar 10;352(10):1011–23.

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Case Studies in Hematology and Coagulation

• Product Description

This compendium of 200 case studies is the result of a unique collaboration of leading hematologists, hematopathologists, and oncologists. It serves as both a case-based guide to the diagnosis and management of patients suffering from hematologic conditions and a valuable teaching tool.

The editors have compiled an invaluable collection of cases covering common and rare entities—from anemias and acute leukemias to plasma cell, platelet and coagulation disorders. Cases are presented in an easy-to-follow format, grouped by related conditions. The final two sections present 27 self-study challenge cases that include answers (with images) provided by the authors of each case.

A broad range of topics in hematology and coagulation are covered in this CaseSet, including:

• Myeloproliferative Disorders
• Myelodysplastic Syndromes
• Lymphoproliferative Disorders
• Lymphomas and Their Mimicks
• Plasma Cell Disorders
• Platelet Disorders
• Hematologic Infectious Diseases
• Other Hematologic Disorders
• Bleeding Disorders
• Thrombophilias
• Other Hemostasis Disorders

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Chapter 5 Answers to case study questions

5.1 Consequences of iron deficiency

We find a 50-year-old woman with long-term Crohn’s disease, on various treatments and with an abdominal surgical history. Her blood tests were requested following a routine GP visit, when she complained of some lethargy, fever and diarrhoea.

• The results outside the reference range are haemoglobin, MCV and ESR. This result, along with the history, is sufficient to confer the diagnosis of anaemia. With the MCV below the bottom of the reference range, we can extend the diagnosis to microcytic anaemia. The abnormal ESR adds little to the diagnosis as it is the likely consequence of the anaemia.
• Having given the patient a diagnosis, a treatment must be initiated. However, this is not yet possible as the basis of the microcytic anaemia must be defined. As the two major causes of microcytic anaemia are iron deficiency and haemoglobinopathy, the blood is tested for iron. A level below the bottom of reference range extends the diagnosis to iron-deficient microcytic anaemia. The reason for the vitamin B12 request is unclear, but as the result is within the reference range, then malnutrition as a cause seems unlikely.
• The diagnosis is not entirely unexpected given the history—Crohn’s disease being an inflammatory disorder of the intestines known to lead to malabsorption. Indeed, the inflammation may well contribute to the abnormal ESR. The referral to surgery may well have been to remove a section of diseased intestine.
• The next step is to initiate treatment. This is likely to be subcutaneous, intra-muscular or intravenous iron. Perhaps a month or six weeks later a follow-up FBC and ESR should show some response. However, Crohn’s is a chronic inflammatory disease, so that there may also be a suppression of erythropoiesis that may need immunosuppression to rectify.

5.2 Consequences of vitamin B 12 deficiency

This case study describes a 75-year-old woman complaining of slowly developing fatigue causing her difficulty in getting out of her house. On examination, there is a swollen and painful tongue, and she complains of becoming forgetful, with occasional ‘tingling’ of her fingertips and numbness in her toes.

• Results outside the reference range are haemoglobin, MCV and ESR. This result, along with the history, is sufficient to confer the diagnosis of anaemia. With the MCV above the top of the reference range, we can extend the diagnosis to macrocytic anaemia. The abnormal ESR adds little to the diagnosis as it is the likely consequence of the anaemia.
• The key result is the low serum vitamin B12, which provides the most likely cause of the disease and symptoms. Indeed, the symptoms are typical. Normal iron levels seem likely to count against malnutrition. One could proceed to determine the precise cause of the deficiency, which, if found to be autoantibodies to gastric parietal cells or IF, will confer the diagnosis of pernicious anaemia. In most clear cases (such as this), examination of the bone marrow (to search for megaloblasts) seems unnecessary.
• The next step will be treatment with parenteral vitamin B12, with monitoring after several weeks to confirm its effect on the full blood count.

5.3 Anaemia after renal transplantation

• Azathioprine may be responsible for the anaemia as it suppresses the bone marrow
• With an MCV within the reference range, the anaemia is normocytic
• With an MCV below the reference range, the anaemia is microcytic
• The leading cause of microcytic anaemia is iron deficiency
• The patient probably had good iron stores in the liver and elsewhere that may have been slowly consumed by the disease process
• Yes. Azathioprine causes macrocytosis, so it is possible that it counteracted the effect of a lack of iron causing microcytosis. Once azathioprine was stopped, the red cells reverted to microcytes.
• As the patient is post-transplant, the practitioner will either be experienced with a case of this nature or will have sought expert opinion.
• Iron needs to be continued in order to build up the patient’s iron stores which were presumably depleted by the chemotherapy.

5.4 An anaemia of chronic disease

An 82-year-old man complains of being tired and lethargic. On examination, he is pale and there is a distended and painful abdomen. He also complains of periodic diarrhoea and constipation and recalls that he has lost perhaps a stone in the past year.

• The abnormalities are low haemoglobin and raised ESR. This, coupled with the history (being pale and complaining of tiredness and lethargy) is sufficient to give a diagnosis of anaemia. With an MCV within the normal range, we describe a normocytic anaemia.
• If the anaemia is normocytic then we are likely, at this stage, to eliminate failure to provide micronutrients to the bone marrow. It is also unlikely that there is a haemoproliferative disease within the bone marrow (such as leukaemia) as the white blood cell count and platelet count are both within the reference range. This, however, still leaves numerous possible reasons, such as haemolysis, and other tests could be used to exclude some diagnoses, such as a direct or indirect antiglobulin test for autoimmune or other anti-body-mediated haemolytic anaemia.
• In many cases, non-laboratory information can be useful, and the history can provide clues. There is loss of weight over a year, and gastrointestinal symptoms. This is indicative of several possible diagnoses, bowel malignancy among them. If this is the cause of the anaemia, one mechanism may be loss of blood into the faeces, which would normally be undetectable. This can be tested with the test ‘faecal occult blood’.

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Hematology-Oncology Board Review and Cases

• The MD Anderson Manual of Medical Oncology Cases
• Williams Hematology Cases

Hematology-Oncology Board Review and Cases, a unique case-based content with multiple-choice questions, detailed explanations, and high yield pearls. This resource can also be used by the clinician daily to work through dosing and treatment options in preparation for administering care. All topics are covered on the ABIM's medical oncology board examination.

Author(s): Moshe C. Ornstein; Sudipto Murkhurjee

Genitourinary Malignancies

• Cancer Case 1 Bladder Cancer Case 1
• Cancer Case 2 Bladder Cancer Case 2
• Cancer Case 1 Testicular Cancer Case 1
• Cancer Case 2 Testicular Cancer Case 2
• Cancer Case 3 Testicular Cancer Case 3
• Cancer Case 4 Testicular Cancer Case 4

Coagulation

• Basis Of Natural Anticoagulants, Fibrinolytic Pathway, And anticoagulant Therapy (Non-Disease-Specific) Case 1 Molecular Basis Of Natural Anticoagulants, Fibrinolytic Pathway, And anticoagulant Therapy (Non-Disease-Specific) Case 1
• Basis Of Natural Anticoagulants, Fibrinolytic Pathway, And anticoagulant Therapy (Non-Disease-Specific) Case 2 Molecular Basis Of Natural Anticoagulants, Fibrinolytic Pathway, And anticoagulant Therapy (Non-Disease-Specific) Case 2
• Basis Of Natural Anticoagulants, Fibrinolytic Pathway, And anticoagulant Therapy (Non-Disease-Specific) Case 3 Molecular Basis Of Natural Anticoagulants, Fibrinolytic Pathway, And anticoagulant Therapy (Non-Disease-Specific) Case 3

Gastrointestinal Cancers

• Cancer Case 1 Esophageal Cancer Case 1

Gynecologic Cancers

• Neoplasms Case 1 Uterine Neoplasms Case 1
• Neoplasms Case 2 Uterine Neoplasms Case 2
• Neoplasms Case 3 Uterine Neoplasms Case 3

Head and Neck

• Gland Malignancies Case 1 Salivary Gland Malignancies Case 1
• Gland Malignancies Case 2 Salivary Gland Malignancies Case 2
• Cancer Case 1 Thyroid Cancer Case 1
• Cancer Case 2 Thyroid Cancer Case 2

Hematologic Neoplastic Disorders

• Lymphocytic Leukemia (CLL) Case 1 Chronic Lymphocytic Leukemia (CLL) Case 1
• Lymphocytic Leukemia (CLL) Case 2 Chronic Lymphocytic Leukemia (CLL) Case 2
• Lymphocytic Leukemia (CLL) Case 3 Chronic Lymphocytic Leukemia (CLL) Case 3
• Lymphocytic Leukemia (CLL) Case 4 Chronic Lymphocytic Leukemia (CLL) Case 4
• Lymphocytic Leukemia (CLL) Case 5 Chronic Lymphocytic Leukemia (CLL) Case 5
• Myeloma Case 1 Multiple Myeloma Case 1
• Myeloma Case 2 Multiple Myeloma Case 2
• Neoplasms Case 1 Myeloproliferative Neoplasms Case 1
• Neoplasms Case 2 Myeloproliferative Neoplasms Case 2
• Neoplasms Case 3 Myeloproliferative Neoplasms Case 3
• Neoplasms Case 4 Myeloproliferative Neoplasms Case 4
• Neoplasms Case 5 Myeloproliferative Neoplasms Case 5
• Lymphoma Case 1 Non-Hodgkin Lymphoma Case 1
• Lymphoma Case 2 Non-Hodgkin Lymphoma Case 2
• Lymphoma Case 3 Non-Hodgkin Lymphoma Case 3
• Lymphoma Case 4 Non-Hodgkin Lymphoma Case 4
• Lymphoma Case 5 Non-Hodgkin Lymphoma Case 5
• Lymphoma Case 6 Non-Hodgkin Lymphoma Case 6
• Lymphoma Case 7 Non-Hodgkin Lymphoma Case 7

Hematopoietic System

• Marrow Failure Case 1 Bone Marrow Failure Case 1
• Marrow Failure Case 2 Bone Marrow Failure Case 2
• Marrow Failure Case 3 Bone Marrow Failure Case 3
• and acquired bone marrow failure syndromes Case 1 Inherited and acquired bone marrow failure syndromes Case 1
• and acquired bone marrow failure syndromes Case 2 Inherited and acquired bone marrow failure syndromes Case 2
• and acquired bone marrow failure syndromes Case 3 Inherited and acquired bone marrow failure syndromes Case 3
• Cell Disease Case 1 Sickle Cell Disease Case 1
• Cell Disease Case 2 Sickle Cell Disease Case 2
• Cell Disease Case 3 Sickle Cell Disease Case 3
• Cell Disease Case 4 Sickle Cell Disease Case 4
• Cell Disease Case 5 Sickle Cell Disease Case 5

Thoracic Oncology

• Pleural Mesothelioma Case 1 Malignant Pleural Mesothelioma Case 1
• Pleural Mesothelioma Case 2 Malignant Pleural Mesothelioma Case 2

The Biology Corner

Biology Teaching Resources

Blood Case Studies for Anatomy (Hematology)

I found this assignment online and re-created it to work for remote-learning, so that it is now in slide format. This format will also work when students are back to in-person learning. I plant to print the slides in color and then place them into dry-erase sleeves so that students can write on them with expo markers and then erase for the next class. This saves on color printing costs and paper.

Another thing that is preferable with in-person learning is the ability for students to work together and communicate what they know. Each group can get a single case (slide) and then we can share the images on the projector. They can point out to the class where the data is highlighted to indicate an abnormal values.

If using this as a remote assignment, I don’t think it would be too much to ask students to complete all of the slides. (Note: if you assign this on LMS, you will need to make a copy and remove the note on the slide that has the answer.)

Ideally, students will have already learned about various conditions, like sickle cell disease and anemia. I have Google slides and guided notes for these units. The remote version, Interactive Slides: Blood also explains the different types of blood cells and their functions.

Each slide shows a CBC (complete blood count) with values for each type of blood cell in the sample. The normal range is shown next to the patient values, so students can easily compare the two. The image of the blood slide does not reveal as many clues as the CBC panel, and is mainly there as a reference.

There are five cases, each showing a particular condition with the CBC counts. Students highlight any abnormalities in the patient’s blood. For example, Teddy has swollen lymph nodes and a high lymphocyte count. From the list of possibilities, the best fit for his disorder is mononucleosis. Each slide has the answer listed under speaker notes. If you are assigning the slides over LMS, make a copy that does not include the answers.

Shannan Muskopf

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Hematology Case Studies

Hematology is a branch of internal medicine that deals with the physiology, pathology, etiology, diagnosis, treatment, prognosis, and prevention of blood-related disorders. Four major areas of study within hematology include hemoglobinopathy, hematological malignancies, anemia, and coagulopathy . 1

Common hematological diseases

• Iron deficiency anemia
• Sickle cell anemia
• Thalassemia
• Bleeding disorders

Signs and symptoms

• Recurrent infection
• Spontaneous bleeding
• Easy bruising

Hematology tests 1

One of the most common hematology tests is the complete blood count, or CBC. This test is often conducted during a routine exam and can detect anemia, clotting problems, blood cancers, immune system disorders and infections. Other hematology tests include:

• Blood chemistry test;

• Blood enzyme test; and

• Blood tests to assess heart disease risk.

• What is hematology?. (2022). Retrieved 24 January 2022, from https://www.healio.com/news/hematology-oncology/20120331/what-is-hematology#:~:text=Hematology%20is%20the%20science%20or,lymphoma%20and%20sickle%2Dcell%20anemia.

Creative Commons License

This post is adapted for the Health & Medical Case Studies created by the Master of Medical Biotechnology program of the University of Windsor. This work licensed under a Creative Commons Attribution-NonCommercial (CC BY-NC-ND) 4.0 International License .

Coagulopathy (also called a bleeding disorder) is a condition in which the blood's ability to coagulate (form clots) is impaired.

Hunt, Beverley J. (2014). "Bleeding and Coagulopathies in Critical Care". New England Journal of Medicine. 370 (9): 847–859. doi:10.1056/NEJMra1208626. ISSN 0028-4793. PMID 24571757.

Thalassemias are inherited blood disorders characterized by decreased hemoglobin production.

HEALTH & MEDICAL CASE STUDIES (V1.01) Copyright © by Dr. Tranum Kaur is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License , except where otherwise noted.

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Case study: hematology, pediatric case of plasmodia falciparum (malaria).

A 13 year-old previously healthy girl who is one of Jehovah’s Witnesses, with a history of malaria 4 years ago, presented with fever, headache, nausea, vomiting and abdominal pain after a recent trip to Liberia to visit family. During her visit, she was taking prophylactic chloroquine but she missed a few doses. Of note, she was recently admitted to a local hospital a week prior to this admission where she was treated with supportive care and discharged home on a bland diet.

Upon admission her core temperature was 103°F and she was hypotensive (BP 70/40). Her hemoglobin level was 10.0 g/dL, white blood cell count 3,000, with a platelet count of 22,000. She was given 3 liters of IV normal saline and started on a dopamine infusion to treat hypotension.  Her hemoglobin level after hydration was 6.6 g/dL.  She was admitted to the pediatric ICU with concern for impending circulatory collapse. The Pediatric service ordered a peripheral blood smear which was concerning for Plasmodia Falciparum (Malaria). The slide was read as 7% parasitemia and the diagnosis of severe malaria was entertained – image below shows parasites inside red blood cells.

After consulting with Pediatric Infectious Disease, treatment was begun with IV quinidine gluconate and Clindamycin. Blood draws were limited to essential lab tests using pediatric phlebotomy tubes to minimize iatrogenic blood loss.  Serial ECGs were obtained to monitor for quinidine toxicity. Despite her low hemoglobin, her and her family’s wishes to avoid blood transfusion were honored.  By day 4 she was afebrile, and her platelet count was up to 111,000. Given her gastrointestinal symptoms, a stool culture was done to rule out parasitic infections such as Giardia.  On day 5 she was discharged on oral quinine 600 mg (10 mg/kg/dose) three times per day, and clindamycin 20 mg/kg/day, both of which were to be continued for 7 days.  After ruling out G6PD enzyme deficiency, Primaquine 53 mg, given orally every 24 hours was started and continued for 14 days for eradication of possible Plasmodium ovale hypnozoites. She was also sent home on oral iron supplements, B12, and folate for her anemia. For future travel to Liberia, Infectious Disease recommended that she take prophylactic doxycycline, atovaquone-proguanil, or mefloquine for malaria prophylaxis, but not cholorquine.  

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Case Study: Sickle Cell Disease A 25-Year-Old in Transition

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A 25-year-old woman with a history of sickle cell disease (SCD) presents to the clinic for follow-up after a hospitalization for a vaso-occlusive pain crisis complicated by influenza A. She has a history of an acute ischemic stroke at age 5 years and has received monthly, simple red cell transfusions since the stroke. Her last transfusion was approximately four months prior. She is taking deferasirox 20 mg/kg daily but occasionally misses doses.

Laboratory results show the following:

Which of the following is the next best step in diagnosis

• Restart scheduled red blood cell transfusions
• Start prophylactic penicillin
• Discontinue transfusions and start hydroxyurea
• Order transcranial doppler ultrasonography (TCD) to assess risk of stroke
• Increase dose of deferasirox to 25 mg/kg/day

Explanation

The incidence of primary stroke in children with SCD is 0.6 to 0.8 events per 100 patient-years, with a cumulative incidence of 7.8 percent by age 14 years in the Jamaican cohort and 11 percent by age 20 years in the U.S. Cooperative Study of Sickle Cell Disease. Once stroke has occurred, the incidence of recurrent (secondary) stroke ranges from 47 to 93 percent in patients not started on regular transfusions. The Stroke Prevention Trial in SCD (STOP) randomized 130 high-risk children with SCD to either transfusion therapy (to maintain HbS 30%) or observation. These high-risk children had an increased blood flow in the internal carotid or middle cerebral artery by TCD. This study showed a 92 percent reduction in incidence of first stroke in transfused high-risk patients. A follow-up study, STOP2, randomly assigned 72 children whose TCD had normalized after 30 months of transfusion therapy to either ongoing or discontinued transfusions. The study was closed early due to a significant increase in abnormal TCD velocity and stroke risk for those who halted transfusion therapy.

The multicenter phase III TWiTCH trial evaluated children with SCA and abnormal TCD velocities without a history of stroke on chronic transfusions. Data showed that hydroxyurea at maximal tolerated dose was noninferior to chronic transfusions for maintaining TCD velocities as primary stroke prophylaxis (choice C). This patient has a history of ischemic stroke, so the results of TWiTCH do not apply to her.

The Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) study was designed as a phase III multicenter trial to determine the efficacy of hydroxyurea/phlebotomy, compared with transfusions/chelation for children with SCA, stroke, and iron overload in secondary stroke prophylaxis. The primary endpoint was a composite of noninferiority for stroke prevention and superiority for reduction of liver iron content. The trial was terminated at the first scheduled interim analysis for futility for the composite endpoint, which required superiority of phlebotomy over iron chelation for reducing excess iron stores. The incidence of stroke on the hydroxyurea plus phlebotomy arm was higher (7 of 67 patients; 10.4%) than in the transfusion plus chelation arm (1 of 66 patients; 1.5%). These results, though not powered for inferiority, showed a trend towars increased stroke risk with transition to hydroxyurea. In patients with prior stroke, cessation of transfusion therapy is currently not recommended.

Whether chronic transfusion therapy can be stopped after a longer period of transfusions in a patient with a prior stroke remains unclear even though risk of recurrent stroke remains high in adolescence and young adulthood. In patients older than 16 years, TCD velocity criteria to determine stroke risk is not reliable (choice D).

In the Prophylaxis with Oral Penicillin in Children with Sickle Cell Anemia trial, children with SCA were randomly assigned to receive oral prophylactic penicillin or placebo PROPS 1986 ). The trial ended eight months early after the occurrence of 15 cases of pneumococcal sepsis, 13 in the placebo group and two in the penicillin group, showing an 84 percent reduction in pneumococcal sepsis with penicillin prophylaxis. The follow-up study, PROPS II, did not show an increased risk in pneumococcal infections with discontinuation of prophylactic penicillin after age 5 years. Therefore, prophylactic penicillin is not recommended in adults with SCA (choice B).

The trajectory of ferritin in this patient has not been established and an increase in oral iron chelation is not indicated at this time.

Case Study submitted by Marquita Nelson, MD, of University of Chicago, Chicago, IL.

• Hirst C, Owusu-Ofori S Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease . Cochrane Database Syst Rev. 2014 6:CD003427.
• Valadi N, Silva GS, Bowman LS, et al Transcranial Doppler ultrasonography in adults with sickle cell disease . Neurology. 2006 22:572-574.
• Ware RE, Davis BR, Schultz WH, et al Stroke with transfusions changing to hydroxyurea (SWiTCH) . Blood. 2012 119:3925-3932.
• Kumar N, Gross JB Jr, Ahlskog JE TCD with transfusions changing to hydroxyurea (TWiTCH): hydroxyurea therapy as an alternative to transfusions for primary stroke prevention in children with sickle cell anemia . Blood. 2015 126:3.

American Society of Hematology. (1). Case Study: Sickle Cell Disease A 25-Year-Old in Transition. Retrieved from https://www.hematology.org/education/trainees/fellows/case-studies/sickle-cell-disease-a-25-year-old-in-transition .

American Society of Hematology. "Case Study: Sickle Cell Disease A 25-Year-Old in Transition." Hematology.org. https://www.hematology.org/education/trainees/fellows/case-studies/sickle-cell-disease-a-25-year-old-in-transition (label-accessed August 10, 2024).

"American Society of Hematology." Case Study: Sickle Cell Disease A 25-Year-Old in Transition, 10 Aug. 2024 , https://www.hematology.org/education/trainees/fellows/case-studies/sickle-cell-disease-a-25-year-old-in-transition .

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