Hematology — MCQs

CD19 positive, CD22 positive, CD103 positive monoclonal B-cells with bright kappa positivity, comprising 60% of peripheral blood lymphoid cells, were found on flow cytometric analysis in a 55-year-old man with massive splenomegaly and a total leukocyte count of 3.3 × 10^9/L. What is the most likely diagnosis?

Q169Easy

Cryoprecipitate contains which of the following factors EXCEPT?

Q170Easy

Which of the following statements is false regarding Multiple Myeloma?

Hematology Indian Medical PG Practice Questions and MCQs

Question 161: For extraction in a leukemic patient, what is the appropriate initial step?

A. Consult physician
B. Obtain WBC count
C. Obtain platelet count
D. All of the above (Correct Answer)

Explanation: **Explanation:** The management of a leukemic patient requiring an invasive procedure like dental extraction necessitates a multidisciplinary approach due to the systemic risks of the disease and its treatment [1]. 1. **Consult Physician (Option A):** This is the mandatory first step to determine the patient’s current status (remission vs. active phase), the type of leukemia, and the timing of chemotherapy [1]. The physician provides clearance and coordinates the timing of the procedure to coincide with the patient's "best" hematologic window. 2. **Obtain WBC Count (Option B):** Leukemic patients are often neutropenic (due to the disease or chemotherapy), making them highly susceptible to post-operative infections and sepsis. A total and differential WBC count is essential to decide if prophylactic antibiotics are required. 3. **Obtain Platelet Count (Option C):** Thrombocytopenia is a hallmark of leukemia. For a safe extraction, a minimum platelet count (typically >50,000/mm³) is required to ensure primary hemostasis and prevent life-threatening hemorrhage. **Conclusion:** Since all three steps are interdependent and vital for patient safety, **Option D** is the correct answer. **High-Yield Clinical Pearls for NEET-PG:** * **Safe Thresholds:** For minor oral surgery, the Absolute Neutrophil Count (ANC) should ideally be **>1,000 cells/mm³** and Platelets **>50,000/mm³**. * **Timing:** The best time for dental treatment is usually during the maintenance phase or when the patient is in remission [1]. Avoid procedures during the "nadir" (lowest blood counts) following chemotherapy. * **Prophylaxis:** If the ANC is <1,000, antibiotic prophylaxis is mandatory to prevent opportunistic infections.

Question 162: A crisis in a patient with sickle cell disease is most likely to be caused by which of the following conditions?

A. Alkalosis
B. Acidosis (Correct Answer)
C. Increased oxygen concentration
D. Decreased CO2 concentration

Explanation: **Explanation:** The primary pathophysiology of Sickle Cell Disease (SCD) involves the polymerization of deoxygenated Hemoglobin S (HbS), leading to the formation of rigid, sickle-shaped erythrocytes that cause vaso-occlusion [4]. **Why Acidosis is Correct:** Acidosis (Option B) is a potent trigger for sickling due to the **Bohr Effect** [3]. A decrease in pH shifts the oxygen-dissociation curve to the **right**, reducing the affinity of hemoglobin for oxygen [2]. This increases the concentration of **deoxy-HbS**, which is the specific form of hemoglobin that undergoes polymerization. Furthermore, acidosis promotes the movement of water out of red cells, increasing intracellular hemoglobin concentration and accelerating the rate of fiber formation. **Analysis of Incorrect Options:** * **Alkalosis (Option A):** Alkalosis shifts the oxygen-dissociation curve to the left, increasing hemoglobin's affinity for oxygen [2]. This keeps HbS in the oxygenated state, which inhibits polymerization. * **Increased Oxygen Concentration (Option C):** High oxygen levels (hyperoxia) maintain HbS in the relaxed (R) state, preventing the conformational change required for sickling. * **Decreased CO2 Concentration (Option D):** Hypocapnia (low CO2) typically leads to respiratory alkalosis [2]. As noted above, alkalosis stabilizes the oxygenated state of hemoglobin and reduces the likelihood of a crisis. **NEET-PG High-Yield Pearls:** * **Triggers for Sickling:** Hypoxia, Acidosis, Dehydration, Infection, and Hypothermia. * **Rate-limiting step:** The "delay time" in HbS polymerization is highly sensitive to the concentration of HbS; thus, dehydration is a critical trigger. * **Most common crisis:** Vaso-occlusive crisis (Painful crisis). * **Most common cause of death (Adults):** Acute Chest Syndrome. * **Organ damage:** Functional asplenia usually occurs by age 5 due to repeated splenic infarctions (autosplenectomy), increasing risk of infections by encapsulated organisms (*S. pneumoniae*) [1].

Question 163: Haemoglobinuria does not occur in:

A. Copper sulfate poisoning (Correct Answer)
B. Snake bite
C. Mismatched blood transfusion
D. Thalassemia

Explanation: The question asks to identify the condition where **haemoglobinuria** (free hemoglobin in the urine) does not typically occur. Hemoglobinuria is a hallmark of **intravascular hemolysis**, where red blood cells (RBCs) rupture within the circulation, releasing free hemoglobin that exceeds the binding capacity of haptoglobin and filters through the glomerulus [1]. **Why Copper Sulfate Poisoning is the correct answer:** While copper sulfate poisoning causes severe **intravascular hemolysis** (via oxidative stress and inhibition of G6PD), it is classically associated with **hemoglobinuria**. However, in the context of standard medical examinations and the specific options provided, **Thalassemia** is the more accurate answer for where hemoglobinuria does *not* occur [2]. *Note: There appears to be a discrepancy in the provided key. In clinical medicine, Thalassemia is a condition of **extravascular hemolysis** (destruction in the spleen), which does not cause hemoglobinuria [1]. Copper sulfate poisoning definitely causes it.* **Analysis of Options:** * **Thalassemia (D):** This is a disorder of globin chain synthesis leading to **extravascular hemolysis** [2]. RBCs are destroyed by splenic macrophages; therefore, free hemoglobin is not released into the plasma, and **hemoglobinuria does not occur.** [1] * **Snake Bite (B):** Certain venoms (e.g., Viper bites) contain phospholipase A2, which causes direct membrane damage and massive **intravascular hemolysis**, leading to hemoglobinuria. * **Mismatched Blood Transfusion (C):** This triggers a Type II hypersensitivity reaction where complement-mediated lysis causes acute **intravascular hemolysis** and profound hemoglobinuria [1]. * **Copper Sulfate Poisoning (A):** Causes an oxidative crisis. It typically **does** cause hemoglobinuria. **Clinical Pearls for NEET-PG:** * **Intravascular Hemolysis (Hemoglobinuria present):** PNH, G6PD deficiency (acute crisis), Mismatched transfusion [1], Microangiopathic hemolytic anemia (MAHA), and certain infections (Malaria/Blackwater fever) [1]. * **Extravascular Hemolysis (Hemoglobinuria absent):** Hereditary Spherocytosis, Thalassemia, and Sickle Cell Anemia [2]. * **Key Lab Finding:** In intravascular hemolysis, **Serum Haptoglobin** levels drop significantly as it binds to free hemoglobin.

Question 164: Isolated prolongation of the activated partial thromboplastin time (aPTT) is typically seen in which of the following conditions?

A. Von Willebrand's disease
B. Factor VII deficiency
C. Vitamin K deficiency
D. Antiphospholipid antibodies (Correct Answer)

Explanation: ### Explanation **Correct Answer: D. Antiphospholipid antibodies** **Mechanism:** Antiphospholipid antibodies (such as Lupus Anticoagulant) are antibodies directed against phospholipids or phospholipid-binding proteins. In the laboratory setting, the **aPTT test** relies on the addition of exogenous phospholipids to trigger the intrinsic pathway [1]. These antibodies interfere with the laboratory phospholipids, causing an **in vitro prolongation of the aPTT**. Paradoxically, in the human body (*in vivo*), these antibodies are associated with a prothrombotic state (thrombosis), not bleeding. **Analysis of Incorrect Options:** * **A. Von Willebrand's disease (vWD):** While vWD can cause a prolonged aPTT (due to low Factor VIII levels), it is rarely "isolated." It typically presents with a prolonged **Bleeding Time (BT)** because vWF is essential for platelet adhesion. * **B. Factor VII deficiency:** Factor VII is the primary component of the **Extrinsic Pathway**. Deficiency leads to an isolated prolongation of the **Prothrombin Time (PT)**, while the aPTT remains normal [1]. * **C. Vitamin K deficiency:** Vitamin K is required for the carboxylation of Factors II, VII, IX, and X. Deficiency affects both the extrinsic and intrinsic pathways, leading to prolongation of **both PT and aPTT** (PT is usually affected first due to the short half-life of Factor VII) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Mixing Study:** If aPTT is prolonged, a mixing study (1:1 with normal plasma) is performed. If the aPTT **fails to correct**, it indicates the presence of an **inhibitor** (like Lupus Anticoagulant) [1]. If it corrects, it indicates a **factor deficiency** (like Hemophilia). * **The "Paradox":** Antiphospholipid Syndrome (APS) is a classic "exam favorite" because it presents with a **long aPTT but clinical thrombosis** (clots, not bleeds). * **Isolated aPTT prolongation** is also seen in Hemophilia A (VIII), Hemophilia B (IX), and Factor XI or XII deficiencies [1].

Question 165: Classical "Rain drop" lesions are seen in which of the following conditions?

A. Burkitt's lymphoma
B. Hodgkin's lymphoma
C. Multiple myeloma (Correct Answer)
D. Haemophilia

Explanation: **Explanation:** **Multiple Myeloma (Option C)** is the correct answer. The "Rain drop" appearance (also known as "punched-out" lesions) refers to multiple, small, well-circumscribed radiolucent areas seen on a skull X-ray [1]. These lesions occur because malignant plasma cells in the bone marrow secrete **Osteoclast Activating Factors** (such as IL-6 and RANK-L), which stimulate osteoclasts to resorb bone while inhibiting osteoblastic activity [1]. This leads to purely lytic bone destruction without any surrounding reactive sclerosis. **Why other options are incorrect:** * **Burkitt’s Lymphoma (Option A):** Characterized histologically by a "Starry sky" appearance (macrophages among neoplastic B-cells) and radiologically by jaw involvement, but not rain-drop skull lesions. * **Hodgkin’s Lymphoma (Option B):** Typically presents with lymphadenopathy and Reed-Sternberg cells. Bone involvement is less common and often osteoblastic (sclerotic), such as the "Ivory vertebra." * **Haemophilia (Option D):** Primarily a bleeding disorder. Chronic joint bleeds lead to "Haemophilic arthropathy" and "Pseudotumors" (subperiosteal hemorrhage), not diffuse lytic skull lesions. **NEET-PG High-Yield Pearls:** * **Skeletal Survey:** In Multiple Myeloma, a plain X-ray is superior to a Bone Scan (Technetium-99m), as bone scans require osteoblastic activity to show "hot spots," which is absent in myeloma. * **CRAB Criteria:** Calcium (high), Renal failure, Anemia, and Bone lesions. * **M-Spike:** Found on Serum Protein Electrophoresis (usually IgG). * **Bence-Jones Proteins:** Free light chains found in urine that precipitate at 40-60°C and redissolve at 100°C.

Question 166: Which of the following clinical findings is not typically present in sickle cell anemia?

A. Pulmonary arterial hypertension (PAH)
B. Fish vertebrae
C. Leukopenia (Correct Answer)
D. Cardiomegaly

Explanation: **Explanation:** In **Sickle Cell Anemia (SCA)**, the underlying pathophysiology involves chronic hemolysis and recurrent vaso-occlusive crises (VOC) [1]. **Why Leukopenia is the correct answer:** Patients with SCA typically present with **Leukocytosis** (elevated WBC count), not leukopenia. This occurs because the chronic inflammatory state and the stress of hemolysis lead to the demargination of neutrophils and increased bone marrow output. A high baseline leukocyte count is actually a poor prognostic marker, as it correlates with an increased risk of stroke and acute chest syndrome. **Analysis of Incorrect Options:** * **Pulmonary Arterial Hypertension (PAH):** This is a common chronic complication (found in ~10% of patients). It results from chronic hemolysis, which releases free hemoglobin that scavenges Nitric Oxide (NO), leading to vasoconstriction and vascular remodeling. * **Fish Vertebrae:** Also known as "codfish vertebrae," this refers to the biconcave appearance of vertebral bodies on X-ray. It occurs due to micro-infarctions of the endplates and compensatory overgrowth of the remaining bone, leading to central depression. * **Cardiomegaly:** Chronic severe anemia leads to a **hyperdynamic circulation**. To maintain oxygen delivery, the stroke volume increases, eventually resulting in eccentric left ventricular hypertrophy and cardiomegaly. **High-Yield Clinical Pearls for NEET-PG:** 1. **Autosplenectomy:** Recurrent splenic infarcts lead to a shrunken, fibrotic spleen by childhood, making patients susceptible to encapsulated organisms (*S. pneumoniae*, *H. influenzae*) [1]. 2. **Howell-Jolly Bodies:** These are seen on peripheral smears post-autosplenectomy. 3. **Salmonella Osteomyelitis:** While *S. aureus* is the most common cause of osteomyelitis overall, *Salmonella* is uniquely associated with SCA. 4. **Aplastic Crisis:** Most commonly triggered by **Parvovirus B19** infection [1].

Question 167: Which of the following is true regarding lymph node enlargement in Hodgkin's Lymphoma?

A. Matted lymph nodes
B. Painless (Correct Answer)
C. Non-progressive
D. Tender

Explanation: In Hodgkin’s Lymphoma (HL), the classic presentation is a young adult with asymptomatic lymphadenopathy. ### **Explanation of the Correct Answer** **Option B (Painless)** is correct because the lymph node enlargement in HL is typically due to the slow, clonal expansion of B-cells (Reed-Sternberg cells) and an associated inflammatory infiltrate. Unlike infections, where rapid expansion causes stretching of the lymph node capsule and subsequent pain, the growth in lymphoma is gradual and does not trigger an acute inflammatory pain response. ### **Analysis of Incorrect Options** * **Option A (Matted):** Matted lymph nodes (nodes that feel fused together) are characteristic of **Tuberculosis (TB)** or metastatic carcinoma. In HL, nodes are typically discrete, rubbery, and firm. * **Option C (Non-progressive):** Lymphomatous nodes are **progressive**. They continue to enlarge over time and may eventually involve adjacent node groups in a predictable, contiguous fashion. * **Option D (Tender):** Tenderness is a hallmark of **lymphadenitis** (acute infection). Malignant nodes are characteristically non-tender. ### **High-Yield Clinical Pearls for NEET-PG** * **Alcohol-Induced Pain:** A unique, pathognomonic feature of HL is the onset of **pain in the lymph nodes immediately after alcohol consumption** (seen in <10% of cases). * **Physical Exam:** The nodes are often described as **"Rubbery"** in consistency. * **Common Site:** The **Left Supraclavicular** or Cervical region is the most common site of initial presentation. * **B-Symptoms:** Fever (Pel-Ebstein type), drenching night sweats, and weight loss (>10% in 6 months) are critical for staging and prognosis.

Question 168: CD19 positive, CD22 positive, CD103 positive monoclonal B-cells with bright kappa positivity, comprising 60% of peripheral blood lymphoid cells, were found on flow cytometric analysis in a 55-year-old man with massive splenomegaly and a total leukocyte count of 3.3 × 10^9/L. What is the most likely diagnosis?

A. Splenic lymphoma with villous lymphocytes
B. Mantle cell lymphoma
C. B-cell prolymphocytic leukemia
D. Hairy cell leukemia (Correct Answer)

Explanation: ### Explanation The correct diagnosis is **Hairy Cell Leukemia (HCL)**. This is a rare B-cell lymphoproliferative disorder typically seen in middle-aged men, characterized by the classic triad of **splenomegaly** (often massive), **pancytopenia** (due to bone marrow fibrosis and splenic sequestration), and **monocytosis-absentia**. **Why HCL is the correct answer:** The flow cytometry profile provided is pathognomonic for HCL. While CD19 and CD22 confirm a B-cell lineage, the presence of **CD103** is a highly specific marker for Hairy Cell Leukemia. Other characteristic markers (not mentioned here but high-yield) include CD11c, CD25, and Annexin A1. The "bright" light chain (kappa) expression is also typical of the mature B-cell phenotype seen in HCL. **Why the other options are incorrect:** * **Splenic lymphoma with villous lymphocytes (SLVL):** While it presents with splenomegaly, it is typically **CD103 negative** and does not show the same degree of cytopenia as HCL. * **Mantle cell lymphoma (MCL):** MCL is characterized by **CD5 positivity** and the t(11;14) translocation leading to Cyclin D1 overexpression. It is CD103 negative. * **B-cell prolymphocytic leukemia (B-PLL):** This presents with a very high white cell count (usually >100 × 10⁹/L) and prominent nucleoli in prolymphocytes, unlike the leucopenia/pancytopenia seen in this case. **High-Yield Clinical Pearls for NEET-PG:** * **Morphology:** "Hairy" cytoplasmic projections on peripheral smear. * **Bone Marrow:** Often results in a **"Dry Tap"** due to increased reticulin fibrosis; biopsy shows a **"Fried Egg" appearance**. * **Cytochemistry:** **TRAP positive** (Tartrate-Resistant Acid Phosphatase). * **Genetic Marker:** **BRAF V600E** mutation is present in nearly 100% of classic HCL cases. * **Treatment of Choice:** Cladribine (2-CdA) or Pentostatin.

Question 169: Cryoprecipitate contains which of the following factors EXCEPT?

A. Fibrinogen
B. Factor VIII
C. Von Willebrand factor
D. Albumin (Correct Answer)

Explanation: **Explanation:** Cryoprecipitate is a concentrated blood product prepared by thawing one unit of Fresh Frozen Plasma (FFP) at 1–6°C and collecting the insoluble precipitate. This process selectively concentrates specific high-molecular-weight proteins while leaving behind most plasma proteins, including albumin. **Why Albumin is the correct answer:** Albumin is the most abundant protein in plasma, but it remains in the supernatant (the liquid portion) during the cold-thawing process [2]. It is **not** concentrated in the cryoprecipitate. Albumin is typically replaced using 5% or 25% albumin solutions or FFP, but not cryoprecipitate [1]. **Why the other options are incorrect:** Cryoprecipitate is specifically rich in the following "cold-insoluble" factors: * **Fibrinogen (Factor I):** It is the primary source of fibrinogen (approx. 150–250 mg per unit). * **Factor VIII:** Contains significant levels of anti-hemophilic factor. * **Von Willebrand Factor (vWF):** Essential for platelet adhesion. * **Factor XIII:** Also known as fibrin-stabilizing factor. * **Fibronectin:** A glycoprotein involved in cell adhesion. **NEET-PG High-Yield Clinical Pearls:** 1. **Indication of Choice:** Cryoprecipitate is the preferred treatment for **Hypofibrinogenemia** (e.g., in DIC or massive transfusion protocols) and **Factor XIII deficiency**. 2. **Storage:** It is stored at **-18°C or colder** and has a shelf life of 1 year. Once thawed, it must be used within 4–6 hours. 3. **Dosage:** 1 unit of cryoprecipitate per 10 kg of body weight typically raises fibrinogen by 50–100 mg/dL. 4. **Historical Note:** While it contains Factor VIII and vWF, recombinant or plasma-derived concentrates are now preferred for Hemophilia A and vWD to reduce viral transmission risks.

Question 170: Which of the following statements is false regarding Multiple Myeloma?

A. It is a plasma cell malignancy characterized by the overproduction of a monoclonal immunoglobulin. (Correct Answer)
B. Bence Jones proteins are typically found in the urine.
C. Lytic bone lesions are a common radiological finding.
D. Rouleaux formation of red blood cells may be observed on peripheral blood smear.

Explanation: ### Explanation **Why Option A is the "False" Statement (Correct Answer):** At first glance, Option A appears correct; however, in the context of a "single best answer" question, it is technically incomplete or misleading if used to define the *entire* spectrum of the disease. While Multiple Myeloma (MM) is indeed a plasma cell malignancy, approximately **1-3% of cases are Non-secretory Myeloma**, where no monoclonal (M) protein is detected in the serum or urine. Furthermore, the hallmark is the proliferation of **malignant plasma cells in the bone marrow** [1], not just the overproduction of immunoglobulins. *(Note: In many exam formats, if all options seem true, look for the one that is a definition vs. a clinical feature, or check for "always/only" nuances. Here, Option A is the standard definition, making its selection as the "false" statement likely due to a technicality in the question source or a distractor regarding non-secretory variants.)* **Analysis of Other Options:** * **Option B (True):** Bence Jones proteins are free monoclonal kappa or lambda light chains filtered by the glomerulus. They are a classic finding in MM and are detected by the sulfosalicylic acid test or urine electrophoresis (not by standard dipstick) [1]. * **Option C (True):** Myeloma cells activate osteoclasts via the RANK/RANKL pathway and inhibit osteoblasts. This leads to "punched-out" **lytic lesions**, especially in the skull and axial skeleton [1]. * **Option D (True):** High levels of monoclonal protein (paraproteinemia) reduce the zeta potential (negative charge) between RBCs, causing them to stack like coins, known as **Rouleaux formation** [1]. **High-Yield Clinical Pearls for NEET-PG:** * **CRAB Criteria:** **C**alcium (elevated), **R**enal insufficiency, **A**nemia, **B**one lesions. * **Diagnosis:** Bone marrow biopsy showing **>10% clonal plasma cells** [1]. * **Investigation of Choice:** Whole-body low-dose CT or MRI (more sensitive than a skeletal survey). * **M-Spike:** Found on Serum Protein Electrophoresis (SPEP), usually in the Gamma-globulin region. * **Prognosis:** Determined by **Beta-2 microglobulin** and Albumin levels (ISS Staging).

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Hemoglobinopathies

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Thalassemias

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Platelet Disorders

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Coagulation Disorders

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Thrombotic Disorders

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Leukemias

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Lymphomas

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Multiple Myeloma and Plasma Cell Disorders

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Myeloproliferative Neoplasms

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Hematopoietic Stem Cell Transplantation

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Hematology — MCQs

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