Haematology

Topics in this chapter

• Anemia And Hemolysis
• Thalassemia And Microcytosis
• Bleeding And Clotting Disorders
• Leukemias And Lymphoid Malignancies
• Plasma Cell And Myeloproliferative Disorders
• Hematologic Cancer Treatment

Practice questions

1. Question 1Q-D2B60B

   A 32-year-old female presents with recurrent episodes of unprovoked deep venous thrombosis and pulmonary embolism. Laboratory workup reveals a prolonged activated partial thromboplastin time (aPTT) that does not correct upon 1:1 mixing with normal pooled plasma. Further testing shows a positive dilute Russell viper venom time (dRVVT) and anticardiolipin antibodies. Which of the following is the most appropriate long-term management for this patient?

   1. A.Indefinite anticoagulation with low-molecular-weight heparin or warfarin targeting an INR of 2.0-3.0
   2. B.Direct oral anticoagulants (DOACs) as first-line therapy
   3. C.Lifelong aspirin monotherapy
   4. D.Prophylactic placement of an inferior vena cava (IVC) filter without anticoagulation
   See the answer and solution
2. Question 2Q-87B024

   A 68-year-old man is evaluated for progressive fatigue, early satiety, and massive splenomegaly. Complete blood count shows hemoglobin of 9.2 g/dL, leukocyte count of 38,000/uL with a leukoerythroblastic blood picture (teardrop cells, nucleated red blood cells, myelocytes, and metamyelocytes), and platelet count of 450,000/uL. Bone marrow aspiration results in a dry tap. Trephine biopsy reveals marked fibrosis and atypical megakaryocytic hyperplasia. Cytogenetic analysis reveals a JAK2 V617F mutation. What is the definitive curative treatment for this condition?

   1. A.Allogeneic hematopoietic stem cell transplantation
   2. B.Ruxolitinib monotherapy
   3. C.Hydroxyurea combined with splenectomy
   4. D.Serial therapeutic phlebotomies
   See the answer and solution
3. Question 3Q-553E5F

   A 24-year-old pregnant female at 12 weeks of gestation is found to have asymptomatic microcytic hypochromic anemia. Her hemoglobin is 10.2 g/dL, mean corpuscular volume (MCV) is 68 fL, and red cell distribution width (RDW) is normal (13%). Serum ferritin is normal (150 ng/mL), and hemoglobin electrophoresis reveals HbA2 of 4.2% and HbF of 1.5%, with the remainder being HbA. Which of the following is the most likely diagnosis?

   1. A.Iron deficiency anemia
   2. B.Beta-thalassemia trait
   3. C.Alpha-thalassemia trait
   4. D.Anemia of chronic disease
   See the answer and solution
4. Question 4Q-FF8ABE

   A 55-year-old man presents with bone pain, progressive renal insufficiency, and fatigue. Laboratory investigations reveal normocytic anemia, hypercalcemia (serum calcium 12.5 mg/dL), and an elevated serum creatinine of 2.4 mg/dL. Serum protein electrophoresis (SPEP) shows an M-spike, and serum free light chain assay shows a markedly abnormal kappa/lambda ratio. Bone marrow biopsy demonstrates 30% clonal plasma cells. Which of the following additional findings is required to confirm the diagnosis of symptomatic multiple myeloma versus smoldering multiple myeloma?

   1. A.Presence of bone marrow plasma cells greater than 60%
   2. B.Presence of renal failure attributable to the plasma cell dyscrasia (CRAB criteria)
   3. C.Presence of serum free light chain ratio greater than 100
   4. D.Presence of more than one focal lesion on MRI
   See the answer and solution
5. Question 5Q-B4D0AB

   A 7-year-old boy is brought to the emergency department with prolonged bleeding following a minor dental extraction. Laboratory evaluation reveals a normal platelet count, prolonged aPTT, normal prothrombin time (PT), and normal thrombin time. Mixing studies demonstrate correction of the aPTT. Factor VIII activity is severely reduced at 2% of normal, while Factor IX activity is normal. The patient is treated with recombinant factor VIII concentrates. Which of the following pattern of inheritance characterizes this disorder?

   1. A.Autosomal dominant
   2. B.Autosomal recessive
   3. C.X-linked recessive
   4. D.Mitochondrial
   See the answer and solution