Hematology Practice Questions

Q: A 60-year-old female presents with weakness, back pain and repeated infections. Work up shows M spike on serum electrophoresis. Prognosis is determined by which of the following factors?

Albumin and $\beta_{2}$-microglobulin. ***Albumin and $\beta_{2}$-microglobulin*** - These two serum markers are the cornerstones of the **International Staging System (ISS)**, the most widely used system for determining prognosis in Multiple Myeloma. - High **$\beta_{2}$-microglobulin** levels reflect a high tumor burden and/or renal dysfunction, while low **serum albumin** indicates a more advanced disease state and poorer prognosis [1]. *IgG and albumin* - While **albumin** is a key prognostic factor, the specific type of immunoglobulin, such as **IgG**, is not a primary component of the main prognostic staging systems like the ISS. - The *quantity* of the monoclonal protein is used to monitor disease response, but the *type* is less critical for staging than **$\beta_{2}$-microglobulin**. *IgM and albumin* - A monoclonal gammopathy involving **IgM** is the hallmark of **Waldenström macroglobulinemia**, not Multiple Myeloma [1]. - Multiple Myeloma is characterized by the monoclonal proliferation of plasma cells that typically produce **IgG** or **IgA**, not **IgM** [1]. *Albumin and LDH* - While elevated **Lactate Dehydrogenase (LDH)** is an adverse prognostic factor, it is used in the **Revised-ISS (R-ISS)**, which builds upon the original ISS. - The foundational ISS, which is universally applied, is based on the combination of **serum albumin** and **$\beta_{2}$-microglobulin** levels.

Q: A 62-year-old male presents with left leg swelling and pain for 5 days and has a positive D-dimer test. Duplex ultrasonography confirms an extensive DVT involving the left popliteal and femoral veins. Which of the following is the most appropriate initial management strategy?

LMWH followed by oral anticoagulant. LMWH followed by oral anticoagulant - Low Molecular Weight Heparin (LMWH) is the preferred initial treatment for stable patients with extensive DVT due to its predictable response [1], [2], subcutaneous administration, and lower risk of heparin-induced thrombocytopenia (HIT) compared to UFH. - Following the initial parenteral therapy (LMWH or UFH), long-term treatment with an oral anticoagulant (such as a DOAC or warfarin) is necessary for at least 3 to 6 months to prevent thrombus extension and recurrent Pulmonary Embolism (PE) [1]. Unfractionated heparin followed by warfarin - Unfractionated Heparin (UFH) often requires hospitalization for IV administration and intense monitoring of aPTT levels [2], making LMWH the outpatient standard for stable patients. - UFH is typically reserved for patients with severe renal impairment (Creatinine Clearance < 30 mL/min) or those who are hemodynamically unstable and may require urgent reversal. Catheter-directed thrombolysis - This invasive therapy is reserved for patients with DVT accompanied by limb ischemia (phlegmasia cerulea dolens) [1] or for selected young patients with extensive proximal DVT and a low risk of bleeding. - It is associated with a significantly higher risk of major bleeding and is not the standard initial strategy for an uncomplicated extensive DVT [1]. IVC filter placement - An IVC filter is indicated only for patients with acute DVT who have an absolute contraindication to anticoagulation (e.g., active hemorrhage) or who experience recurrent PE despite adequate anticoagulation [1]. - Filter placement does not treat the existing deep vein clot and increases the long-term risk of recurrent DVT.

Q: A young woman presents with recurrent headaches, easy bruising, and episodes of visual blurring. Her platelet count is elevated, while her red blood cell (RBC) and white blood cell (WBC) counts are normal. What is the most likely diagnosis?

Essential Thrombocythemia. ***Essential Thrombocythemia*** - This **myeloproliferative neoplasm (MPN)** is characterized by isolated, persistent elevation of the **platelet count** (thrombocytosis) with relatively normal RBC and WBC counts, fitting the clinical picture. - Symptoms like headaches, visual blurring, and bruising are common because the functionally abnormal platelets can cause both **microvascular thrombosis** and bleeding tendencies. *Polycythemia Vera* - PV is primarily defined by **erythrocytosis** (elevated RBC count or hematocrit), which is inconsistent with this patient's normal red blood cell count. - Although PV often causes thrombocytosis, the core diagnostic feature involves a panmyelosis dominated by excessive **RBC mass** leading to hyperviscosity symptoms. *Chronic Myeloid Leukemia (CML)* - CML is hallmarked by significant **leukocytosis** (elevated WBC count, especially neutrophils and their precursors) due to the **BCR-ABL fusion gene**. - The patient's normal WBC count makes CML an unlikely diagnosis. *Acute Myeloid Leukemia (AML)* - AML typically presents with signs of **bone marrow failure**, such as anemia, neutropenia, and thrombocytopenia (low platelets), indicating acute lack of mature blood cells. - The lab findings in AML usually show cytopenia or the presence of immature **myeloblasts**, not isolated thrombocytosis.

Q: A 46-year-old male presents to the outpatient department with a history of lower back pain and painful urination. Upon investigation, he is found to have normocytic normochromic anemia and hypercalcemia. Serum protein electrophoresis reveals an M-spike in the gamma region. Which of the following tests is most appropriate to confirm the diagnosis of multiple myeloma?

Bone marrow examination. ***Bone marrow examination*** - The **definitive confirmatory test** for multiple myeloma is bone marrow examination showing **≥10% clonal plasma cells** (or presence of plasmacytoma on biopsy) [1]. - According to **International Myeloma Working Group (IMWG) criteria**, diagnosis requires: (1) clonal bone marrow plasma cells ≥10% OR biopsy-proven plasmacytoma AND (2) evidence of end-organ damage (CRAB criteria: hypercalcemia, renal insufficiency, anemia, bone lesions) [1]. - This patient has M-spike (monoclonal protein), hypercalcemia, anemia, and bone pain—bone marrow biopsy **confirms the diagnosis** by demonstrating clonal plasma cell proliferation [2]. *Liver function test* - These tests primarily evaluate **hepatic function** (e.g., ALT, AST, bilirubin) and are not diagnostic for multiple myeloma. - While some secondary abnormalities may occur (e.g., elevated **LDH** or **ALP** if bone involvement is extensive), LFTs lack the required specificity for confirming the diagnosis. *Urine albumin-to-creatinine ratio (UACR) test* - UACR is primarily used to screen for **microalbuminuria** in conditions such as **diabetic nephropathy** or hypertensive kidney disease. - The proteinuria seen in multiple myeloma is due to excretion of **kappa or lambda light chains** (Bence Jones protein), not albumin, making UACR inappropriate for MM diagnosis. *Bence Jones protein in urine* - Detection of **monoclonal free light chains** (Bence Jones protein) in urine is a **supportive finding** present in 50-60% of MM cases [1]. - While clinically significant and part of the diagnostic workup, it is **not confirmatory by itself**—bone marrow examination remains the gold standard. - Modern detection uses **urine immunofixation electrophoresis** or serum free light chain assays.

Q: Which of the following is not included in the Gaisböck syndrome?

Hypotension. Hypotension- Gaisböck syndrome, often called stress polycythemia or relative polycythemia, is strongly associated with hypertension due to the hemoconcentration and reduced plasma volume [1].- Hypotension is not a feature of this syndrome, which typically presents in middle-aged men with cardiovascular risk factors.*Erythrocytosis*- Relative erythrocytosis (or pseudopolycythemia) is the defining characteristic of Gaisböck syndrome [1].- This increase in hematocrit is due to a decrease in plasma volume (hemoconcentration) rather than an absolute increase in red cell mass.*Normal leukocyte counts*- Maintaining normal white blood cell (WBC) counts is typical in Gaisböck syndrome [1].- This feature helps exclude Polycythemia Vera, where both leukocytes and platelets are often elevated along with the absolute erythrocytosis.*Obesity*- Obesity is a common and significant component of the syndrome, along with other metabolic risk factors like hyperlipidemia.- The patient group is often described as overweight, anxious, and highly prone to cardiovascular events.

Q: A 38-year-old female presents with fatigue, weakness, and pallor for 3 months. She has a history of menorrhagia. Laboratory findings show hemoglobin 8.5 g/dL, MCV 68 fL, MCH 22 pg, serum ferritin 8 ng/mL, serum iron 30 μg/dL, TIBC 450 μg/dL, and peripheral smear shows microcytic hypochromic RBCs with pencil cells. Bone marrow examination shows absent iron stores. What is the most appropriate next step in management?

Oral iron supplementation with vitamin C. The laboratory findings (low Hb, low MCV, low MCH, significantly low **serum ferritin**, high **TIBC**) are classic for severe **Iron Deficiency Anemia (IDA)** [1]. The most appropriate first-line treatment for uncomplicated IDA, especially in a patient with chronic blood loss (menorrhagia), is high-dose **oral ferrous salts** (e.g., ferrous sulfate) [1]. Menorrhagia is a common cause of anaemia in pre-menopausal females [2]. Elevated hepcidin in anemia of inflammation can make it refractory to iron supplementation, making the diagnosis of true IDA important [3]. Blood transfusion is indicated for patients with anemia causing **hemodynamic instability**, impending **cardiac decompensation**, or extremely low Hgb levels (often <7 g/dL) [4].

Q: A 32-year-old woman presents with easy bruising and petechiae. Her platelet count is 18,000 / mm³. Hemoglobin and total leukocyte count are normal. She recently recovered from a mild upper respiratory tract infection. Peripheral smear shows no abnormal cells. What is the most appropriate next step in management?

Oral corticosteroids. ***Oral corticosteroids*** - This is the **first-line treatment** for newly diagnosed Immune Thrombocytopenic Purpura (ITP), especially when the platelet count is severely low (typically <30,000/mm³) and the patient exhibits bleeding symptoms like **petechiae** and easy bruising [1]. - Corticosteroids reduce the clearance of antibody-coated platelets by the **reticuloendothelial system** (primarily splenic macrophages) and suppress autoantibody production. ***Platelet transfusion*** - Platelet transfusions are generally **contraindicated** in ITP because the transfused platelets are rapidly destroyed by the same autoantibodies that target the patient's native platelets [1]. - They are reserved only for **life-threatening hemorrhage** (e.g., intracranial bleeding) or for pre-operative stabilization, usually given concurrently with high-dose IVIG or corticosteroids. ***Bone marrow biopsy*** - A **bone marrow biopsy** is usually not required for a young patient with classic ITP presentation (isolated thrombocytopenia, normal Hb/TLC, recent infection, and normal peripheral smear). - It is typically mandatory only if the patient is older (e.g., >60), has unexplained cytopenias in other lineages, or is **refractory** to first-line treatment. ***Splenectomy*** - Splenectomy is a **second-line treatment** option, typically reserved for patients with chronic ITP who fail to respond adequately to medical therapy (corticosteroids and IVIG) after 6 to 12 months [2]. - Although the spleen is the primary site of autoantibody production and platelet destruction, it is not the **most appropriate immediate next step** in initial management [2].

Q: A 16-year-old boy presents with jaundice and splenomegaly. His father had a similar illness during adolescence. MCHC : high? What is the most likely diagnosis?

B. Hereditary spherocytosis. ***Hereditary spherocytosis*** - The presentation of **jaundice**, **splenomegaly**, strong **family history** (autosomal dominant pattern), and an elevated **MCHC** is classic for Hereditary Spherocytosis. [1] - Elevated MCHC is a key diagnostic feature due to mild cellular dehydration in the characteristic **spherocytes**. [1] *IDA* - **Iron Deficiency Anemia (IDA)** is typically microcytic and hypochromic, leading to a **low MCHC**, not high. - Symptoms of IDA include fatigue and pallor, generally without marked jaundice or splenomegaly unless severe. *Thalassemia major* - **Thalassemia major** presents with severe microcytic, hypochromic anemia (low MCHC) usually evident in early infancy, requiring regular transfusions. - While it causes jaundice and hepatosplenomegaly, it is characterized by severe bone changes and a very low MCHC. *AIHA* - **Autoimmune Hemolytic Anemia (AIHA)** typically presents acutely or subacutely and often responds to immunosuppression (e.g., steroids). - Unlike Hereditary Spherocytosis, AIHA generally does not present with a positive **family history** and the primary lab finding is a positive **Direct Antiglobulin Test (DAT)**, not specifically high MCHC.

Q: Which of the following is the most common inherited bleeding disorder?

Von Willebrand Disease. ***Von Willebrand Disease*** - VWD is the most common inherited bleeding disorder, with a prevalence estimated to be as high as 1% of the population, often showing an **autosomal dominant** inheritance pattern. - It results from a quantitative or qualitative defect in **von Willebrand factor (vWF)**, which impairs platelet adhesion and stabilizes **Factor VIII** [1][2]. *Disseminated Intravascular Coagulation* - DIC is an **acquired** life-threatening condition caused by systemic activation of coagulation, usually triggered by underlying severe diseases like **sepsis** or trauma, not inheritance. - It is characterized by simultaneous widespread thrombosis and severe bleeding due to the consumption of clotting factors and platelets. *Factor V Deficiency* - Factor V deficiency (Parahemophilia) is an extremely rare inherited bleeding disorder, transmitted in an **autosomal recessive** manner. - While inherited, its overall prevalence is substantially lower compared to VWD, making it not the most common. *Hemophilia* - Hemophilia (A and B) is the most common severe inherited bleeding disorder, known for causing deep tissue bleeding (e.g., **hemarthroses**). - It is an **X-linked recessive** condition, meaning it is less prevalent overall in the population compared to the high frequency of mild, often undiagnosed, VWD cases.

Q: A 40-year-old patient presents with complains of low back pain and development of pallor. During workup very high levels of urinary beta2 microglobulin was noted. X-ray skull was performed. Bone marrow aspiration shows >10% plasma cells and serum electrophoresis shows M protein >30 g/L. All are useful for management of this patient except?

Melphalan. ***Melphalan*** - While melphalan is used in **multiple myeloma**, its high-dose application is primarily a conditioning regimen for **autologous stem cell transplantation**, rather than a standalone maintenance or initial therapy, especially in younger patients who are transplant-eligible. - Furthermore, alkylating agents like melphalan are associated with an increased risk of **secondary malignancies**, making their long-term use less favorable in transplant-eligible patients when newer, less toxic agents are available. *Lenalidomide* - **Lenalidomide** is an immunomodulatory drug (IMiD) that is highly effective in the treatment of multiple myeloma, often used in maintenance therapy post-transplant or as part of initial induction regimens. - It works by modulating the immune system and has direct anti-myeloma activity, making it a valuable tool in the management of this patient [1]. *Bortezomib* - **Bortezomib** is a proteasome inhibitor that is a cornerstone of initial induction therapy for transplant-eligible and transplant-ineligible multiple myeloma patients [1]. - It effectively inhibits the break down of cellular proteins, leading to apoptosis of myeloma cells, and is crucial for achieving deep responses. *Autologous transplantation* - Given the patient's age (40 years old) and likely fitness, **autologous stem cell transplantation** is a standard and highly effective treatment option for multiple myeloma, aimed at achieving deep and durable remissions [1]. - It involves harvesting the patient's own stem cells, then administering high-dose chemotherapy (often melphalan) to eradicate myeloma cells, followed by reinfusion of the stored stem cells to rescue hematopoiesis [1].

Question 1

A 60-year-old female presents with weakness, back pain and repeated infections. Work up shows M spike on serum electrophoresis. Prognosis is determined by which of the following factors?

Accepted Answer

Albumin and $\beta_{2}$-microglobulin

Answer

Albumin and LDH

Answer

IgM and albumin

Answer

IgG and albumin

Question 2

A 62-year-old male presents with left leg swelling and pain for 5 days and has a positive D-dimer test. Duplex ultrasonography confirms an extensive DVT involving the left popliteal and femoral veins. Which of the following is the most appropriate initial management strategy?

Accepted Answer

LMWH followed by oral anticoagulant

Answer

Unfractionated heparin followed by warfarin

Answer

Catheter-directed thrombolysis

Answer

IVC filter placement

Question 3

A young woman presents with recurrent headaches, easy bruising, and episodes of visual blurring. Her platelet count is elevated, while her red blood cell (RBC) and white blood cell (WBC) counts are normal. What is the most likely diagnosis?

Accepted Answer

Essential Thrombocythemia

Answer

Polycythemia Vera

Answer

Chronic Myeloid Leukemia (CML)

Answer

Acute Myeloid Leukemia (AML)

Question 4

A 46-year-old male presents to the outpatient department with a history of lower back pain and painful urination. Upon investigation, he is found to have normocytic normochromic anemia and hypercalcemia. Serum protein electrophoresis reveals an M-spike in the gamma region. Which of the following tests is most appropriate to confirm the diagnosis of multiple myeloma?

Accepted Answer

Bone marrow examination

Answer

Liver function test

Answer

Urine albumin-to-creatinine ratio (UACR) test

Answer

Bence Jones protein in urine

Question 5

Which of the following is not included in the Gaisböck syndrome?

Accepted Answer

Hypotension

Answer

Normal leukocyte counts

Answer

Obesity

Answer

Erythrocytosis

Question 6

A 38-year-old female presents with fatigue, weakness, and pallor for 3 months. She has a history of menorrhagia. Laboratory findings show hemoglobin 8.5 g/dL, MCV 68 fL, MCH 22 pg, serum ferritin 8 ng/mL, serum iron 30 μg/dL, TIBC 450 μg/dL, and peripheral smear shows microcytic hypochromic RBCs with pencil cells. Bone marrow examination shows absent iron stores. What is the most appropriate next step in management?

Accepted Answer

Oral iron supplementation with vitamin C

Answer

Parenteral iron therapy immediately

Answer

Blood transfusion followed by iron therapy

Answer

Investigate for gastrointestinal malignancy first

Question 7

A 32-year-old woman presents with easy bruising and petechiae. Her platelet count is 18,000 / mm³. Hemoglobin and total leukocyte count are normal. She recently recovered from a mild upper respiratory tract infection. Peripheral smear shows no abnormal cells. What is the most appropriate next step in management?

Accepted Answer

Oral corticosteroids

Answer

Platelet transfusion

Answer

Splenectomy

Answer

Bone marrow biopsy

Question 8

A 16-year-old boy presents with jaundice and splenomegaly. His father had a similar illness during adolescence. MCHC : high? What is the most likely diagnosis?

Accepted Answer

B. Hereditary spherocytosis

Answer

D. AIHA

Answer

C. Thalassemia major

Answer

A. IDA

Question 9

Which of the following is the most common inherited bleeding disorder?

Accepted Answer

Von Willebrand Disease

Answer

Hemophilia

Answer

Factor V Deficiency

Answer

Disseminated Intravascular Coagulation

Question 10

A 40-year-old patient presents with complains of low back pain and development of pallor. During workup very high levels of urinary beta2 microglobulin was noted. X-ray skull was performed. Bone marrow aspiration shows >10% plasma cells and serum electrophoresis shows M protein >30 g/L. All are useful for management of this patient except?

Accepted Answer

Melphalan

Answer

Lenalidomide

Answer

Bortezomib

Answer

Autologous transplantation

Hematology — MCQs

Hematology — MCQs

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