Hematopathology Practice Questions

Q: A 10-year-old child presents with pallor and a history of blood transfusion 2 months prior. Investigations reveal Hb 4.5 gm/dL, total leukocyte count 60,000/mm³, platelet count 2 lakhs/mm³, CD10 positive, CD19 positive, CD117 positive, VEPO positive, and CD33 negative. What is the most likely diagnosis?

Mixed phenotypic acute leukemia. ### Explanation The diagnosis of **Mixed Phenotypic Acute Leukemia (MPAL)** is based on the presence of markers from two different lineages on the same blast population (biphenotypic) or two distinct blast populations (bilineal). **1. Why Option D is Correct:** The child presents with hyperleukocytosis (TLC 60,000/mm³) and blasts expressing both lymphoid and myeloid markers: * **Lymphoid markers:** CD10 and CD19 are classic B-cell lineage markers. * **Myeloid markers:** CD117 (c-kit) and MPO (Myeloperoxidase, indicated here as VEPO positive) are definitive for myeloid lineage. According to the **WHO criteria**, the expression of strong B-cell markers (CD19) alongside definitive myeloid markers (MPO) confirms MPAL. **2. Why Other Options are Incorrect:** * **Option A (ALL):** While CD10 and CD19 are positive, the presence of MPO (VEPO) and CD117 strictly excludes a pure diagnosis of ALL. * **Option B (AML):** Although CD117 and MPO are present, the strong expression of B-cell markers (CD10, CD19) is not typical for pure AML. * **Option C (Undifferentiated Leukemia):** This diagnosis is reserved for cases where blasts lack any lineage-specific markers (negative for MPO, T-cell, and B-cell markers). **3. NEET-PG High-Yield Pearls:** * **MPO (Myeloperoxidase):** The most specific marker for Myeloid lineage. * **CD19:** The most specific marker for B-cell lineage. * **Cytoplasmic CD3:** The most specific marker for T-cell lineage. * **Prognosis:** MPAL generally carries a poorer prognosis compared to lineage-restricted ALL or AML and often requires intensive "hybrid" chemotherapy protocols followed by Stem Cell Transplant.

Q: Thrombocytopenia occurs in all of the following conditions except?

Henoch-Schönlein purpura. **Explanation:** The core concept tested here is the distinction between **quantitative** and **qualitative** platelet disorders. **1. Why Henoch-Schönlein Purpura (HSP) is the correct answer:** HSP is a small-vessel **leukocytoclastic vasculitis** mediated by IgA immune complex deposition. The "purpura" seen in HSP is **non-thrombocytopenic**; in fact, the platelet count in HSP is typically **normal or even elevated** (as an acute-phase reactant). The skin lesions occur due to inflammation and leakage of the blood vessels, not due to a deficiency in platelets. **2. Analysis of Incorrect Options:** * **Thrombotic Thrombocytopenic Purpura (TTP):** Characterized by the classic pentad, which includes **consumptive thrombocytopenia** due to the formation of extensive microthrombi in small vessels (ADAMTS13 deficiency). * **Disseminated Intravascular Coagulation (DIC):** Involves widespread activation of the coagulation cascade, leading to the **massive consumption** of both clotting factors and platelets, resulting in profound thrombocytopenia. * **Wiskott-Aldrich Syndrome:** A X-linked recessive immunodeficiency characterized by the triad of eczema, immunodeficiency, and **micro-thrombocytopenia** (small-sized platelets and low counts due to impaired production and increased destruction). **Clinical Pearls for NEET-PG:** * **HSP Tetrad:** Palpable purpura (without thrombocytopenia), arthritis/arthralgia, abdominal pain (intussusception risk), and renal involvement (IgA nephropathy). * **Platelet Size:** Wiskott-Aldrich is unique for **small** platelets, whereas ITP and Bernard-Soulier Syndrome feature **large** (giant) platelets. * **Rule of Thumb:** If a question mentions "Palpable Purpura," always think of Vasculitis (like HSP) rather than simple thrombocytopenia.

Q: Macrocytosis is seen in all of the following disorders, except?

Thalassemia major. **Explanation:** The correct answer is **Thalassemia major** because it is a disorder characterized by **microcytic hypochromic anemia**, not macrocytosis. [1] **1. Why Thalassemia Major is the correct answer:** In Thalassemia, there is a genetic defect in the synthesis of globin chains (alpha or beta). This leads to deficient hemoglobin production. To compensate for the lack of hemoglobin, the red blood cell undergoes extra divisions in the bone marrow, resulting in smaller cells (**Microcytosis**, MCV < 80 fL). [1] **2. Why the other options are incorrect (Causes of Macrocytosis):** * **Vitamin B12 and Folic Acid Deficiency:** These are the classic causes of **Megaloblastic Anemia**. Both are essential for DNA synthesis (specifically thymidine production). Deficiency leads to "nuclear-cytoplasmic asynchrony," where the nucleus matures slowly while the cytoplasm grows normally, resulting in large, oval red cells (**Macro-ovalocytes**, MCV > 100 fL). * **Hypothyroidism:** This is a common cause of **Non-megaloblastic macrocytosis**. The exact mechanism is multifactorial, involving altered lipid metabolism in the RBC membrane and a direct effect of thyroid hormone deficiency on erythropoiesis. **High-Yield NEET-PG Pearls:** * **MCV Ranges:** Microcytic ( 100 fL). * **Megaloblastic vs. Non-megaloblastic:** Megaloblastic macrocytosis features **hypersegmented neutrophils** (5+ lobes), whereas non-megaloblastic (Alcoholism, Liver disease, Hypothyroidism) does not. * **Thalassemia Hallmark:** Look for **Target cells** and a Mentzer Index (MCV/RBC count) **< 13**, which helps differentiate it from Iron Deficiency Anemia. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591, 600-601.

Question 1

A 10-year-old child presents with pallor and a history of blood transfusion 2 months prior. Investigations reveal Hb 4.5 gm/dL, total leukocyte count 60,000/mm³, platelet count 2 lakhs/mm³, CD10 positive, CD19 positive, CD117 positive, VEPO positive, and CD33 negative. What is the most likely diagnosis?

Accepted Answer

Mixed phenotypic acute leukemia

Answer

Acute lymphoblastic leukemia (ALL)

Answer

Acute myeloid leukemia (AML)

Answer

Undifferentiated leukemia

Question 2

What is the most common tumor of the spleen?

Accepted Answer

Lymphoma

Answer

Sarcoma

Answer

Hemangioma

Answer

Metastasis

Question 3

Acid phosphatase cytochemical staining is used in the diagnosis of which of the following hematological neoplasms?

Accepted Answer

T-cell acute lymphoblastic leukemia

Answer

Chronic myeloid leukemia

Answer

Acute myeloid leukemia

Answer

B-cell acute lymphoblastic leukemia

Question 4

Which of the following is pathognomonic of thalassemia major?

Accepted Answer

Raised HbF

Answer

Conjugated hyperbilirubinemia

Answer

Raised HbA2

Answer

Severe anemia

Question 5

Thrombocytopenia occurs in all of the following conditions except?

Accepted Answer

Henoch-Schönlein purpura

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Disseminated intravascular coagulation (DIC)

Answer

Wiskott-Aldrich syndrome

Question 6

All of the following statements regarding Sickle Cell Anemia are true EXCEPT:

Accepted Answer

Adults, but not children, with sickle cell disease are functionally asplenic, making them susceptible to infections caused by encapsulated bacteria, such as pneumococci.

Answer

The shift from HbF to HbS occurs due to point mutation.

Answer

Microvascular occlusion is a severe complication of Sickle Cell Anemia.

Answer

Deoxygenation of RBCs can lead to extensive membrane damage.

Question 7

Which of the following conditions is associated with Coombs-positive hemolysis?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Immune thrombocytopenic purpura (ITP)

Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Question 8

NESTROFT test is used for screening of:

Accepted Answer

Thalassemia

Answer

Sickle cell anemia

Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Answer

Paroxysmal cold hemoglobinuria (PCH)

Question 9

Macrocytosis is seen in all of the following disorders, except?

Accepted Answer

Thalassemia major

Answer

Hypothyroidism

Answer

Folic acid deficiency

Answer

Vitamin B12 deficiency

Question 10

A man living in southern Japan contracts HTLV-1 infection through sexual contact. Twenty-five years later he develops generalized lymphadenopathy with hepatosplenomegaly, a skin rash, hypercalcemia, and an elevated white blood count. This man has most likely developed which of the following?

Accepted Answer

Leukemia

Answer

AIDS

Answer

Autoimmunity

Answer

Delayed hypersensitivity reaction

Hematopathology — MCQs

Hematopathology — MCQs

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