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: Which of the following statements about paroxysmal nocturnal hemoglobinuria (PNH) is false?

The gold standard test is the sucrose lysis test.. Paroxysmal Nocturnal Hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder caused by a mutation in the PIGA gene, leading to a deficiency of GPI-anchored proteins (CD55 and CD59) on the cell membrane [1]. This makes RBCs highly susceptible to complement-mediated lysis, representing a rare form of acquired intravascular hemolysis [1]. Why Option C is the correct (False) statement: The Sucrose Lysis Test and the Ham Test (Acidified Serum Test) are historical screening tools with low specificity and sensitivity. They have been replaced by Flow Cytometry as the current gold standard. Flow cytometry identifies the absence of GPI-anchored proteins (CD55/CD59) on RBCs and leukocytes (specifically using FLAER—Fluorescent Aerolysin—for white cells). Analysis of other options: * Option A: Thrombosis (often in unusual sites like hepatic veins—Budd-Chiari syndrome) is indeed the most common cause of mortality in PNH patients. * Option B: The bone marrow is typically hyperplastic as it attempts to compensate for chronic hemolysis. However, PNH can also be associated with aplastic anemia (hypoplastic marrow). * Option C: Hemolysis is triggered by factors that activate the complement system, such as infections, stress, and strenuous exercise. High-Yield Clinical Pearls for NEET-PG: * Triad of PNH: Hemolytic anemia, Pancytopenia, and Thrombosis. * Urine Findings: Hemosiderinuria is a classic finding in chronic intravascular hemolysis. * Treatment: Eculizumab (a monoclonal antibody against C5) is the drug of choice to prevent hemolysis. * Association: PNH has a strong link with Aplastic Anemia and may transform into Acute Myeloid Leukemia (AML).

Q: All are true regarding Anaemia of Chronic Diseases, except?

Decreased ferritin. **Explanation:** Anemia of Chronic Disease (ACD), also known as Anemia of Inflammation, is primarily mediated by **Hepcidin**, an acute-phase reactant produced by the liver in response to inflammatory cytokines (mainly IL-6) [1]. **Why Option B is the Correct Answer (The False Statement):** In ACD, serum **ferritin is increased or normal**, never decreased. Ferritin acts as an acute-phase reactant; inflammation triggers its synthesis and traps iron within the reticuloendothelial system (macrophages). A **decreased ferritin** is the hallmark and most sensitive indicator of **Iron Deficiency Anemia (IDA)**, not ACD [2]. **Analysis of Incorrect Options (True Statements for ACD):** * **A. Decreased serum iron:** Hepcidin degrades ferroportin channels, preventing iron release from macrophages and absorption from the gut [1]. This leads to low circulating iron (hypoferremia). * **C. Decreased TIBC:** In states of chronic inflammation, the body downregulates transferrin production (the protein measured by TIBC) to limit iron availability to potential pathogens. * **D. Increased bone marrow iron:** Because iron is "trapped" inside macrophages and cannot be utilized for erythropoiesis, bone marrow biopsy with Prussian blue staining shows abundant iron stores [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Transferrin Saturation:** Usually low or normal in ACD (but higher than in IDA). * **Soluble Transferrin Receptor (sTfR):** Normal in ACD, but **increased in IDA**. This is a key differentiator [2]. * **Morphology:** Usually Normocytic Normochromic, but can become Microcytic Hypochromic in long-standing cases [1]. * **Treatment:** Treat the underlying inflammatory condition; Erythropoietin (EPO) may be used in specific cases (e.g., CKD or malignancy) [1].

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: A 35-year-old female presents with cervical and axillary lymphadenopathy. She has a history of fever and drenching night sweats and is diagnosed with Hodgkin's lymphoma. What is the stage of the disease?

II-B. ### Explanation The staging of Hodgkin’s Lymphoma (HL) is determined using the **Ann Arbor Staging System** (modified by the Cotswolds criteria) [1]. **1. Why Option B (II-B) is correct:** * **Stage II:** The patient has involvement of two or more lymph node regions (cervical and axillary) on the **same side of the diaphragm** (both are above the diaphragm) [1]. * **Modifier "B":** The presence of systemic symptoms—specifically unexplained fever (>38°C), drenching night sweats, or weight loss (>10% body weight in 6 months)—designates the "B" category [1], [2]. Since this patient has fever and night sweats, she is classified as Stage II-B. **2. Why other options are incorrect:** * **Option A (II-A):** The modifier "A" denotes the **absence** of constitutional (B) symptoms [1]. This patient clearly exhibits B symptoms. * **Options C & D (IIE):** The modifier "E" is used for **extranodal** involvement (e.g., localized involvement of an extralymphatic organ like the lung or liver) adjacent to a known nodal site. This patient’s involvement is limited to lymph nodes only. **3. High-Yield Clinical Pearls for NEET-PG:** * **Stage I:** Single lymph node region or single extralymphatic site. * **Stage III:** Lymph node involvement on **both sides** of the diaphragm. * **Stage IV:** Diffuse or disseminated involvement of one or more extralymphatic organs (e.g., bone marrow, liver). * **Bulky Disease:** Defined as a nodal mass >10 cm or >1/3rd of the transthoracic diameter on CXR [1]. * **Most Common Subtype:** Nodular Sclerosis (often presents with mediastinal mass in young females). * **Best Prognosis:** Lymphocyte Predominant. * **Worst Prognosis:** Lymphocyte Depleted.

Q: Intracorpuscular hemolytic anemia is seen in which of the following conditions?

Thalassemia. Hemolytic anemias are broadly classified based on the site of the defect into **Intracorpuscular (Intrinsic)** and **Extracorpuscular (Extrinsic)** causes. **Why Thalassemia is Correct:** Thalassemia is an **intracorpuscular** hemolytic anemia [1]. The defect lies within the red blood cell (RBC) itself—specifically, a genetic mutation leading to the reduced synthesis of globin chains ($\alpha$ or $\beta$) [1]. This imbalance causes the precipitation of unpaired globin chains, leading to membrane damage, ineffective erythropoiesis, and premature destruction of RBCs by the spleen. Other examples of intracorpuscular defects include membrane defects (Hereditary Spherocytosis) [3], enzyme deficiencies (G6PD deficiency) [1], and hemoglobinopathies (Sickle Cell Anemia) [1]. **Analysis of Incorrect Options:** * **A. Autoimmune Hemolytic Anemia (AIHA):** This is an **extracorpuscular** cause where the RBC is structurally normal, but external factors (antibodies) tag the cell for destruction [2]. * **B. Thrombotic Thrombocytopenic Purpura (TTP):** This is an **extracorpuscular** microangiopathic hemolytic anemia (MAHA). RBCs are fragmented (schistocytes) as they pass through small vessels obstructed by fibrin/platelet thrombi. * **D. Infection:** Infections (e.g., Malaria, Clostridium welchii) cause **extracorpuscular** hemolysis through direct parasite invasion or toxin-mediated damage to the RBC membrane [2]. **NEET-PG High-Yield Pearls:** * **Rule of Thumb:** Almost all intracorpuscular defects are **hereditary** [1], with one notable exception: **Paroxysmal Nocturnal Hemoglobinuria (PNH)**, which is an acquired intracorpuscular defect [2]. * **Extracorpuscular** causes are typically **acquired** (Immune, Mechanical, or Infectious). * **Coombs Test:** Usually positive in immune-mediated extracorpuscular hemolysis (AIHA) and negative in intracorpuscular defects like Thalassemia.

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]

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

Which of the following statements about paroxysmal nocturnal hemoglobinuria (PNH) is false?

Accepted Answer

The gold standard test is the sucrose lysis test.

Answer

Thrombosis is the leading cause of death.

Answer

Bone marrow is hyperplastic.

Answer

Hemolysis is precipitated during exercise.

Question 3

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 4

All are true regarding Anaemia of Chronic Diseases, except?

Accepted Answer

Decreased ferritin

Answer

Decreased serum iron

Answer

Decreased total iron-binding capacity

Answer

Increased bone marrow iron

Question 5

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 6

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 7

All the following are true in Immune thrombocytopenic Purpura (ITP) EXCEPT?

Accepted Answer

Associated with prolonged bleeding time.

Answer

Chronic ITP commonly occurs in adult women.

Answer

Prothrombin Time (PT) and Partial Thromboplastin Time (PTT) are normal.

Answer

Increased megakaryocytes in bone marrow.

Question 8

A 35-year-old female presents with cervical and axillary lymphadenopathy. She has a history of fever and drenching night sweats and is diagnosed with Hodgkin's lymphoma. What is the stage of the disease?

Accepted Answer

II-B

Answer

II-A

Answer

IIE-A

Answer

IIE-B

Question 9

Intracorpuscular hemolytic anemia is seen in which of the following conditions?

Accepted Answer

Thalassemia

Answer

Autoimmune hemolytic anemia

Answer

Thrombotic thrombocytopenic purpura

Answer

Infection

Question 10

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

Hematology — MCQs

Hematology — MCQs

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