Hematology Practice Questions

Q: Which of the following conditions is associated with thrombocytopenia?

Bernard-Soulier syndrome. The correct answer is **Bernard-Soulier Syndrome (BSS)**. **1. Why Bernard-Soulier Syndrome is correct:** BSS is a rare autosomal recessive bleeding disorder caused by a deficiency or dysfunction of the **GPIb-IX-V complex**, which serves as the receptor for von Willebrand factor (vWF) [1]. This defect impairs platelet adhesion to the subendothelium. A hallmark diagnostic feature of BSS is **thrombocytopenia** (usually mild to moderate) accompanied by **Giant Platelets** (often as large as red blood cells) [2]. On peripheral smear, these are often referred to as "macrothrombocytopenia." **2. Why the other options are incorrect:** * **Glanzmann Thrombasthenia:** This is caused by a deficiency of **GPIIb/IIIa**, leading to defective platelet aggregation [1]. Crucially, the **platelet count and morphology are normal** in these patients. * **Gray Platelet Syndrome:** This is an alpha-granule deficiency. While it can occasionally present with mild thrombocytopenia, its primary characteristic is the "ghost-like" or gray appearance of platelets due to the lack of granules, rather than a primary quantitative defect. * **Storage Pool Disease:** This involves a deficiency of dense granules (delta-SPD). Like Glanzmann’s, the **platelet count is typically normal**, but the platelets fail to release ADP/serotonin, leading to impaired secondary aggregation. **NEET-PG High-Yield Pearls:** * **BSS vs. Glanzmann:** In BSS, platelets **do not** aggregate with Ristocetin, and the addition of normal plasma does **not** correct it (unlike vWD). In Glanzmann, Ristocetin aggregation is **normal**, but aggregation with ADP, collagen, and epinephrine is defective. * **Giant Platelets Differential:** Think of BSS, May-Hegglin anomaly, and Bernard-Soulier Syndrome. * **Mnemonic:** **B**ernard **S**oulier = **B**ig **S**ize (Giant platelets) and **B**elow count (Thrombocytopenia).

Q: Fresh Frozen Plasma (FFP) is not indicated in which of the following conditions?

Factor XII deficiency. The correct answer is **Factor XII deficiency (Hageman factor deficiency)**. **1. Why Factor XII deficiency is the correct answer:** Factor XII deficiency is a unique hematological condition characterized by a **markedly prolonged Activated Partial Thromboplastin Time (aPTT)** in vitro, but **no clinical bleeding tendency** in vivo [2]. Since patients do not suffer from hemorrhagic complications (and may actually be at a slightly increased risk for thrombosis), there is no clinical indication for the administration of FFP or replacement therapy, even before major surgery. **2. Analysis of Incorrect Options:** * **Thrombotic Thrombocytopenic Purpura (TTP):** FFP is a mainstay of treatment. It is used during **plasmapheresis** (plasma exchange) to replace the deficient **ADAMTS13** enzyme, which is essential for cleaving large von Willebrand factor multimers. * **Vitamin K Deficiency:** FFP is indicated for the **urgent reversal** of coagulopathy (deficiency of Factors II, VII, IX, and X) when there is active bleeding or a need for emergency surgery, as Vitamin K administration takes 6–24 hours to be effective [1]. * **Antithrombin III (AT-III) Deficiency:** FFP contains AT-III. It is indicated in patients with hereditary AT-III deficiency who are undergoing high-risk procedures or are resistant to heparin therapy (since heparin requires AT-III to function). **Clinical Pearls for NEET-PG:** * **FFP Dosage:** Usually 10–15 mL/kg; it raises clotting factor levels by approximately 20-30%. * **Factor XII Paradox:** Prolonged aPTT + No bleeding = Factor XII deficiency [2]. * **Storage:** FFP is stored at -18°C or colder and must be used within 24 hours of thawing to ensure adequate levels of labile factors (V and VIII) [1].

Q: A patient has normal prothrombin time (PT) and platelet count with increased activated partial thromboplastin time (aPTT), factor 8 levels given as 60 IU/ml, and no history of bleeding. What is the diagnosis?

Factor IX deficiency. The clinical presentation of a **normal PT**, **normal platelet count**, and **isolated prolonged aPTT** indicates a defect in the **intrinsic pathway** of the coagulation cascade (Factors XII, XI, IX, or VIII) [1]. 1. **Why Factor IX Deficiency is correct:** Factor IX is a key component of the intrinsic pathway. Its deficiency (Hemophilia B) leads to a prolonged aPTT while PT and bleeding time remain normal [2]. The question specifies **Factor VIII levels are normal (60 IU/ml)**, which effectively rules out Hemophilia A or von Willebrand Disease [3], making Factor IX deficiency the most logical diagnosis among the options. *Note: While severe Hemophilia B usually presents with bleeding, mild cases may be asymptomatic until surgical challenge.* 2. **Why other options are incorrect:** * **Thalassemia:** This is a quantitative hemoglobinopathy leading to microcytic hypochromic anemia; it does not affect the coagulation cascade or aPTT. * **Factor VIII Inhibitors:** These are acquired antibodies against Factor VIII. While they prolong aPTT, the **Factor VIII levels would be significantly low**, not normal (60 IU/ml). * **Lupus Anticoagulant (LA):** While LA prolongs aPTT in vitro and typically presents without bleeding, it is an antiphospholipid antibody associated with **thrombosis**, not a factor deficiency. Given the specific mention of Factor VIII levels, the question directs focus toward the intrinsic factor hierarchy. **High-Yield Clinical Pearls for NEET-PG:** * **Isolated prolonged aPTT:** Think Factors VIII, IX, XI, XII, or Lupus Anticoagulant [1]. * **Isolated prolonged PT:** Think Factor VII deficiency (Extrinsic pathway) [1]. * **Mixing Study:** If aPTT corrects with normal plasma, it’s a **factor deficiency**; if it does not correct, it’s a **factor inhibitor** (like Lupus Anticoagulant) [1]. * **Factor XII Deficiency:** Characterized by a very high aPTT but **no clinical bleeding** tendency.

Q: What is the best prognostic indicator of multiple myeloma at the time of diagnosis?

Beta 2 microglobulin. The prognosis of Multiple Myeloma (MM) is currently determined by the **International Staging System (ISS)**, which identifies **Serum Beta-2 Microglobulin (β2M)** as the single most important prognostic marker [1]. 1. **Why Beta-2 Microglobulin is correct:** β2M is a component of the MHC Class I molecule found on the surface of nucleated cells. In MM, its levels reflect the **total tumor burden** and the **severity of renal impairment**. Higher levels correlate with a larger mass of plasma cells and poorer survival outcomes [1]. It is the cornerstone of both the ISS and the Revised-ISS (R-ISS) staging systems. [1] 2. **Why other options are incorrect:** * **Number of myeloma cells (A):** While the percentage of plasma cells in the bone marrow is used for *diagnosis* (e.g., >10% for MM), it does not correlate as accurately with survival or prognosis as biochemical markers like β2M. [1] * **Alkaline Phosphatase (C):** In MM, bone lesions are purely **osteolytic** (mediated by osteoclasts). Because there is no osteoblastic activity, the ALP level is typically **normal**. An elevated ALP in a suspected MM patient should prompt a search for an alternative diagnosis or a fracture. * **Hypercalcemia (D):** While hypercalcemia is a "CRAB" feature indicating end-organ damage, it is a reversible complication and not as reliable a predictor of long-term survival as β2M. **High-Yield Clinical Pearls for NEET-PG:** * **ISS Staging:** Stage I (β2M <3.5 mg/L + Albumin ≥3.5 g/dL); Stage III (β2M ≥5.5 mg/L). [1] * **R-ISS:** Adds **LDH levels** and **High-risk Cytogenetics** [t(4;14), t(14;16), or del(17p)] to the standard ISS for even more precise prognosis. * **Serum Albumin:** Low albumin is also a poor prognostic sign in MM (reflecting IL-6 mediated suppression). [1]

Q: Autoimmune destruction of platelets is seen in which of the following conditions?

Systemic Lupus Erythematosus (SLE). **Explanation:** The correct answer is **Systemic Lupus Erythematosus (SLE)**. **1. Why SLE is correct:** SLE is a multisystem autoimmune disorder characterized by the production of various autoantibodies [1]. Hematological involvement is a hallmark of the disease. Thrombocytopenia in SLE occurs primarily due to **immune-mediated destruction of platelets**, where anti-platelet antibodies (IgG) coat the platelets, leading to their premature clearance by the splenic macrophages [2]. This mechanism is identical to **Immune Thrombocytopenic Purpura (ITP)**. In fact, ITP can often be the presenting feature of SLE. **2. Why other options are incorrect:** * **Polyarteritis Nodosa (PAN):** This is a necrotizing vasculitis of medium-sized arteries. While it causes multisystem involvement (renal, GI, skin), it does not typically involve autoantibody-mediated destruction of platelets. * **Rheumatoid Arthritis (RA):** While RA is autoimmune, its primary hematological association is **Felty’s Syndrome** (RA, Splenomegaly, and Neutropenia). Thrombocytopenia is not a classic feature unless secondary to drug toxicity (e.g., methotrexate) or hypersplenism. * **Sarcoidosis:** This is a granulomatous disease. Hematological issues usually involve lymphopenia or anemia of chronic disease. Thrombocytopenia, if present, is usually due to splenic sequestration (splenomegaly) rather than direct autoimmune destruction. **Clinical Pearls for NEET-PG:** * **Evans Syndrome:** The coexistence of Autoimmune Hemolytic Anemia (AIHA) and Immune Thrombocytopenia (ITP); frequently associated with SLE. * **Most common hematological abnormality in SLE:** Anemia of Chronic Disease (however, Lymphopenia is the most *specific* hematologic criteria in the ACR classification) [2]. * **Mechanism:** Type II Hypersensitivity reaction (Antibody-mediated) [3].

Q: All of the following can lead to the development of overt anemia in patients with long-standing compensated hemolytic anemia, EXCEPT:

Hypothyroidism. In patients with **compensated hemolytic anemia**, the bone marrow increases erythropoiesis (up to 6–8 times the normal rate) to match the shortened lifespan of red blood cells. **Overt anemia** occurs when this delicate balance is disrupted, either by an increased demand for RBCs or a decrease in marrow production. ### Why Hypothyroidism is the Correct Answer **Hypothyroidism** typically causes a mild, normocytic, hypoproliferative anemia due to a generalized decrease in metabolic rate and tissue oxygen demand, which leads to reduced erythropoietin (EPO) production. However, it does **not** cause an acute "decompensation" or a sudden crisis in hemolytic states. Unlike the other options, it does not acutely interfere with the high-turnover requirements of a hemolytic marrow. ### Explanation of Incorrect Options * **Pregnancy:** Increases plasma volume (dilutional effect) and significantly raises the demand for RBC production and nutrients. This can tip a compensated state into overt anemia. * **Renal Failure:** Leads to a deficiency in **Erythropoietin (EPO)**. In hemolysis, the marrow requires high levels of EPO to maintain compensation; loss of EPO leads to a rapid drop in hemoglobin. * **Folate Deficiency:** Hemolysis creates a high-turnover state with a massive demand for folate for DNA synthesis. If stores are depleted, a **megaloblastic/aplastic crisis** occurs, causing profound anemia. ### High-Yield Clinical Pearls for NEET-PG * **Aplastic Crisis:** Most commonly caused by **Parvovirus B19**, which infects erythrocyte precursors. * **Megaloblastic Crisis:** Caused by folate deficiency due to chronic hyperactive erythropoiesis. * **Hemolytic Crisis:** An acute acceleration of hemolysis (e.g., G6PD deficiency after oxidant stress). * **Splenic Sequestration:** Common in Sickle Cell Anemia; causes sudden pooling of blood in the spleen and acute anemia.

Question 1

Which of the following conditions is associated with thrombocytopenia?

Accepted Answer

Bernard-Soulier syndrome

Answer

Gray platelet syndrome

Answer

Glanzmann thrombasthenia

Answer

Storage pool disease

Question 2

A 60-year-old man is referred for evaluation of marked erythrocytosis and splenomegaly. Laboratory studies confirm an elevated red blood cell count and additionally demonstrate a moderate increase in circulating granulocytes and platelets. Oxygen saturation studies are normal, and isotopic studies reveal an increase in total red cell mass. Which of the following is characteristic of this disorder?

Accepted Answer

Frequent association with thrombosis or hemorrhagic phenomena

Answer

Increased erythropoietin concentration

Answer

Manifestation of Cushing syndrome

Answer

Most often secondary to hypoxia

Question 3

Fresh Frozen Plasma (FFP) is not indicated in which of the following conditions?

Accepted Answer

Factor XII deficiency

Answer

Thrombotic Thrombocytopenic Purpura (TTP)

Answer

Vitamin K deficiency

Answer

Antithrombin III deficiency

Question 4

A patient has normal prothrombin time (PT) and platelet count with increased activated partial thromboplastin time (aPTT), factor 8 levels given as 60 IU/ml, and no history of bleeding. What is the diagnosis?

Accepted Answer

Factor IX deficiency

Answer

Thalassemia

Answer

Factor VIII inhibitors

Answer

Lupus anticoagulant

Question 5

What is the best prognostic indicator of multiple myeloma at the time of diagnosis?

Accepted Answer

Beta 2 microglobulin

Answer

Number of myeloma cells in the marrow

Answer

Alkaline phosphatase level

Answer

Hypercalcemia

Question 6

A 27-year-old female presents with easy fatigue and light-headedness. She also has a dry cough and fever for the past few days. On examination, she is pale, her lungs are clear, and the rest is normal. A chest x-ray shows patchy bilateral infiltrates; the hemoglobin is 8.4 g/dL, reticulocyte count of 6%, and many spherocytes on the peripheral blood film. Which of the following is the most likely significance of the spherocytosis on the blood film?

Accepted Answer

Coombs'-positive hemolytic anemia

Answer

Multiple long bone fractures

Answer

Hereditary elliptocytosis

Answer

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Question 7

A 42-year-old male presents with fatigue, pale skin, and exertional dyspnea. Laboratory findings reveal macrocytic, hyperchromic anemia with hyper-segmented neutrophils. Folate levels are normal. The patient reports long-term omeprazole use for gastric reflux disease. Which of the following interventions is indicated for this patient?

Accepted Answer

Vitamin B12 injections

Answer

Oral folic acid supplementation

Answer

Vitamin B6 injections

Answer

Intrinsic factor injections

Question 8

Which of the following statements is NOT true about heparin-induced thrombocytopenia?

Accepted Answer

It is more common with unfractionated heparin than low molecular weight heparin.

Answer

Low molecular weight heparins should not be used for treatment.

Answer

It causes both arterial and venous thrombosis.

Answer

It typically occurs after about a week of heparin therapy.

Question 9

Autoimmune destruction of platelets is seen in which of the following conditions?

Accepted Answer

Systemic Lupus Erythematosus (SLE)

Answer

Polyarteritis Nodosa (PAN)

Answer

Rheumatoid Arthritis (RA)

Answer

Sarcoidosis

Question 10

All of the following can lead to the development of overt anemia in patients with long-standing compensated hemolytic anemia, EXCEPT:

Accepted Answer

Hypothyroidism

Answer

Pregnancy

Answer

Renal failure

Answer

Folate deficiency

Hematology — MCQs

Hematology — MCQs

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