Hematopathology Practice Questions

Q: Blast crises in Chronic Myeloid Leukemia (CML) are characterized by which of the following?

Blast cells in blood >= 20%. Chronic Myeloid Leukemia (CML) is a myeloproliferative neoplasm characterized by the Philadelphia chromosome $t(9;22)$. It typically progresses through three distinct phases: the Chronic Phase, the Accelerated Phase, and the **Blast Crisis (Blast Phase)**. **1. Why Option B is Correct:** According to the WHO classification, the **Blast Crisis** is defined by the presence of **$\ge$ 20% blasts** in the peripheral blood or bone marrow [1]. This stage represents the transformation of CML into an acute leukemia [1]. Interestingly, in 70% of cases, the blasts are myeloid (AML-like), while in 30%, they are lymphoid (ALL-like), reflecting the origin of CML in a pluripotent stem cell. **2. Why Other Options are Incorrect:** * **Option A:** A blast count of **10-19%** (along with other criteria like persistent thrombocytopenia or increasing splenomegaly) defines the **Accelerated Phase**, not the Blast Crisis. * **Option C:** Blasts are never absent in CML; even in the Chronic Phase, blasts are typically present but remain $<10\%$. **High-Yield Clinical Pearls for NEET-PG:** * **Defining Genetic Abnormality:** $t(9;22)$ resulting in the *BCR-ABL1* fusion gene, which encodes a constitutively active tyrosine kinase. * **Leukocyte Alkaline Phosphatase (LAP) Score:** Characteristically **decreased** in CML (helps differentiate it from a Leukemoid Reaction where LAP is elevated). * **Basophilia:** An increase in peripheral blood basophils is a hallmark of CML and often heralds progression to the accelerated phase. * **Treatment:** Imatinib (Tyrosine Kinase Inhibitor) is the first-line therapy. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 613-614.

Q: Comment on the type of anemia observed in a peripheral smear.

Pernicious anemia. ***Pernicious anemia*** - Characterized by **macro-ovalocytes** (large, oval-shaped RBCs) and **hypersegmented neutrophils** (>5 nuclear lobes) on peripheral smear. - Results from **vitamin B12 deficiency** due to lack of **intrinsic factor**, leading to megaloblastic anemia with distinctive morphological changes. *Iron deficiency anemia* - Shows **microcytic hypochromic** RBCs with **pencil cells** and **target cells** on peripheral smear. - Associated with **decreased serum ferritin** and **increased TIBC**, not the megaloblastic changes seen in this case. *Sickle cell anemia* - Peripheral smear reveals characteristic **sickle-shaped cells** (drepanocytes) and **target cells**. - Caused by **hemoglobin S mutation**, leading to sickling under hypoxic conditions, not megaloblastic morphology. *Hereditary spherocytosis* - Shows **spherocytes** (round RBCs lacking central pallor) and **increased osmotic fragility** on peripheral smear. - Results from **membrane protein defects** causing loss of membrane surface area, distinct from megaloblastic changes.

Q: A 20-year-old female presented with complaints of easy bruising, frequent episodes of nose bleed, menorrhagia, and few episodes of upper gastrointestinal bleeding. On examination, purpura and petechial rash were noted. Laboratory findings revealed thrombocytopenia, normal prothrombin time (PT), and normal activated partial thromboplastin time (aPTT). Platelets do not aggregate in response to ristocetin after adding normal plasma, but have normal aggregation in response to adenosine diphosphate, epinephrine, and collagen. Platelet granules are normal. What is the most likely diagnosis in the above patient?

Bernard-Soulier syndrome. **Explanation:** The clinical presentation of mucosal bleeding (epistaxis, menorrhagia, GI bleed) and petechiae in a young patient suggests a **disorder of primary hemostasis** [1]. **1. Why Bernard-Soulier Syndrome (BSS) is correct:** BSS is caused by a deficiency or dysfunction of the **GPIb-IX-V receptor** complex on the platelet surface [3]. This receptor is essential for platelet adhesion to the subendothelial von Willebrand factor (vWF). * **Key Diagnostic Feature:** Platelets fail to aggregate with **Ristocetin**. Crucially, unlike von Willebrand Disease (vWD), this defect **cannot be corrected** by adding normal plasma (which contains vWF) because the defect lies in the platelet receptor itself, not the plasma factor. * **Laboratory Findings:** Characterized by **thrombocytopenia** and **Giant Platelets** (though not explicitly mentioned here, it is a hallmark) [2]. PT and aPTT remain normal as secondary hemostasis is intact. **2. Why incorrect options are wrong:** * **Glanzmann Thrombasthenia:** Caused by a deficiency of **GPIIb/IIIa** [3]. While it presents with similar bleeding, aggregation is **absent** with ADP, epinephrine, and collagen, but **normal** with Ristocetin (the exact opposite of this case). * **Wiskott-Aldrich Syndrome:** An X-linked recessive disorder characterized by the triad of eczema, immunodeficiency, and thrombocytopenia with **micro-platelets** (small platelets) [2]. * **Platelet Function Disorder:** This is a broad category; the specific Ristocetin pattern provided points specifically to BSS. **Clinical Pearls for NEET-PG:** * **BSS:** Giant Platelets + Thrombocytopenia + No aggregation with Ristocetin (not corrected by plasma). * **vWD:** Normal Platelet count + No aggregation with Ristocetin (**Corrected** by adding normal plasma) [4]. * **Glanzmann:** Normal Platelet count + No aggregation with ADP/Collagen/Epinephrine.

Question 1

Blast crises in Chronic Myeloid Leukemia (CML) are characterized by which of the following?

Accepted Answer

Blast cells in blood >= 20%

Answer

Blast cells in blood or bone marrow > 10%

Answer

Blast cells absent

Answer

All of the above

Question 2

Comment on the type of anemia observed in a peripheral smear.

Accepted Answer

Pernicious anemia

Answer

Iron deficiency anemia

Answer

Sickle cell anemia

Answer

Hereditary spherocytosis

Question 3

Acanthocytosis on peripheral smear is a feature of which of the following conditions?

Accepted Answer

Abetalipoproteinemia

Answer

Uremia

Answer

Thalassemia trait

Answer

Paroxysmal nocturnal hemoglobinuria

Question 4

Bence Jones proteins are composed of?

Accepted Answer

Light chains

Answer

Heavy chains

Answer

Cryoglobulins

Answer

Constitutive enzyme

Question 5

A 20-year-old female presented with complaints of easy bruising, frequent episodes of nose bleed, menorrhagia, and few episodes of upper gastrointestinal bleeding. On examination, purpura and petechial rash were noted. Laboratory findings revealed thrombocytopenia, normal prothrombin time (PT), and normal activated partial thromboplastin time (aPTT). Platelets do not aggregate in response to ristocetin after adding normal plasma, but have normal aggregation in response to adenosine diphosphate, epinephrine, and collagen. Platelet granules are normal. What is the most likely diagnosis in the above patient?

Accepted Answer

Bernard-Soulier syndrome

Answer

Platelet function disorder

Answer

Wiskott-Aldrich syndrome

Answer

Glanzmann thrombasthenia

Question 6

Which virus is known to cause hemopoietic carcinoma?

Accepted Answer

Epstein-Barr virus (EBV)

Answer

Human T-lymphotropic virus type I (HTLV-I)

Answer

Human herpesvirus 8 (HHV-8)

Answer

Cytomegalovirus (CMV)

Question 7

Which of the following conditions does NOT cause a prolonged PTT?

Accepted Answer

Idiopathic Thrombocytopenic Purpura (ITP)

Answer

Hemophilia A

Answer

Von Willebrand disease

Answer

Christmas disease

Question 8

The direct globulin test is positive in which of the following conditions?

Accepted Answer

Paroxysmal cold hemoglobinuria

Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Answer

Sickle cell anemia

Answer

Thalassemia

Question 9

Which of the following statements is NOT true regarding idiopathic thrombocytopenic purpura?

Accepted Answer

Antibodies of IgM class

Answer

Autoantibodies to Gp IIb/IIIa or Ib/IX

Answer

Increased megakaryocytes in the bone marrow

Answer

Spleen is normal in size

Question 10

After an accident, a male patient presented for routine evaluation. His RBCs showed Cabot's rings. What is the likely condition responsible?

Accepted Answer

Post-splenectomy state

Answer

Acquired hemolytic anemia

Answer

Hemochromatosis

Answer

Thalassemia

Hematopathology — MCQs

Hematopathology — MCQs

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