Hematopathology Practice Questions

Q: A 15-year-old boy presented with a 1-day history of bleeding gums, subconjunctival bleed, and purpuric rash. Investigations revealed: Hb 6.4 gm/dL; TLC-26,500/mm3; prothrombin time-20 sec with a control of 13 sec; partial thromboplastin time-50 sec; and fibrinogen 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Promyelocytic leukemia. ### Explanation The clinical presentation of a young patient with sudden onset bleeding (gums, subconjunctival, purpura) combined with a high white cell count and profound coagulopathy is classic for **Acute Promyelocytic Leukemia (APL)**, formerly classified as FAB M3 [1], [3]. **1. Why Promyelocytic Leukemia is Correct:** The hallmark of APL is the association with **Disseminated Intravascular Coagulation (DIC)** [2]. This is evidenced in the question by the prolonged PT (20s), prolonged PTT (50s), and critically low fibrinogen (10 mg/dL; normal: 200–400 mg/dL). In APL, the primary granules of the malignant promyelocytes contain procoagulants and fibrinolytic enzymes (like annexin II) that trigger a massive consumptive coagulopathy upon release. **2. Why Other Options are Incorrect:** * **A & B (M1 & M2):** While these are common subtypes of AML, they typically present with signs of bone marrow failure (anemia, infections) [1]. While they can cause thrombocytopenia, they rarely present with such severe, primary DIC at the time of diagnosis. * **D (M4):** Myelomonocytic leukemia is often associated with extramedullary involvement (e.g., gingival hyperplasia or CNS involvement) rather than the severe consumptive coagulopathy seen here [3]. **3. High-Yield Pearls for NEET-PG:** * **Cytogenetics:** Associated with **t(15;17)**, involving the **PML-RARA** fusion gene. * **Morphology:** Characterized by **Auer rods** (often in bundles called **Faggot cells**). * **Treatment:** Medical emergency! Start **ATRA (All-trans retinoic acid)** or Arsenic Trioxide immediately to differentiate the blasts and resolve DIC. * **Complication:** Watch for **Differentiation Syndrome** (fever, dyspnea, pulmonary infiltrates) during treatment.

Q: Small platelets on peripheral smear examination are seen in which of the following conditions?

Wiskott-Aldrich syndrome. **Explanation:** The size of platelets on a peripheral smear is a critical diagnostic clue in pediatric hematology. **1. Why Wiskott-Aldrich Syndrome (WAS) is correct:** WAS is an X-linked recessive disorder caused by mutations in the *WAS* gene, which encodes the Wiskott-Aldrich Syndrome Protein (WASP). This protein is essential for actin cytoskeleton remodeling. Defects lead to the formation of **microthrombocytes** (small platelets) due to abnormal megakaryocyte proplatelet formation and increased splenic clearance. It is classically characterized by the triad of **Thrombocytopenia (with small platelets), Eczema, and Recurrent infections** (due to combined B and T cell deficiency). **2. Why the other options are incorrect:** * **Bernard-Soulier Syndrome (BSS):** Characterized by a deficiency of GP Ib-IX-V complex. It is the classic cause of **Giant Platelets** (often as large as red blood cells) and failure of platelet aggregation with Ristocetin. * **Gray Platelet Syndrome:** A rare alpha-granule deficiency. Platelets appear large, pale, and "ghost-like" (gray) on Wright-Giemsa stain due to the lack of granules. * **Von Willebrand Disease (vWD) Type 2B:** This "gain-of-function" mutation leads to increased binding of vWF to GP Ib, causing spontaneous platelet aggregation and clearance. This typically results in mild **thrombocytopenia with large/giant platelets** (due to increased turnover). **High-Yield Clinical Pearls for NEET-PG:** * **Small Platelets:** Wiskott-Aldrich Syndrome, X-linked Thrombocytopenia (XLT). * **Giant Platelets:** Bernard-Soulier Syndrome, May-Hegglin Anomaly (look for Dohle-like bodies in neutrophils), and ITP (due to young, "stress" platelets). * **WAS Mnemonic:** **W**-**A**-**S** (Small platelets, Aldrich, Sex-linked/X-linked). * **Treatment of choice for WAS:** Hematopoietic stem cell transplant (HSCT).

Q: Dohle-like inclusions in neutrophils along with giant platelets is characteristic of which of the following conditions?

May-Hegglin anomaly. **Explanation:** **May-Hegglin Anomaly (MHA)** is an autosomal dominant disorder characterized by a triad of **thrombocytopenia, giant platelets, and large, basophilic leukocyte inclusions** (Döhle-like bodies). 1. **Why it is correct:** The condition is caused by a mutation in the **MYH9 gene**, which encodes the non-muscle myosin heavy chain IIA. This defect leads to disordered megakaryocyte maturation (resulting in giant platelets) and the precipitation of myosin heavy chains in the cytoplasm of neutrophils, forming **Döhle-like bodies**. Unlike true Döhle bodies (seen in infections/burns), these are larger, more prominent, and persist even in the absence of inflammation. 2. **Why other options are incorrect:** * **Bernard-Soulier Syndrome:** Features giant platelets and thrombocytopenia (due to GpIb-IX-V deficiency) [1], but **lacks** leukocyte inclusions. * **Chédiak-Higashi Syndrome:** Characterized by **massive lysosomal granules** in leukocytes and partial albinism, not Döhle-like bodies or giant platelets. * **Pelger-Huët Anomaly:** Involves a failure of nuclear segmentation (hyposegmented, "pince-nez" neutrophils) but does not affect platelets. **High-Yield Clinical Pearls for NEET-PG:** * **MYH9-Related Disorders:** MHA is part of a spectrum including Epstein and Fechtner syndromes (which add nephritis and sensorineural hearing loss to the hematologic findings). * **Döhle-like vs. Döhle bodies:** True Döhle bodies are composed of **rough endoplasmic reticulum**; Döhle-like bodies in MHA are composed of **myosin heavy chains**. * **Peripheral Smear:** Always look for the combination of "Big Platelets + Blue Inclusions" to diagnose May-Hegglin. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669.

Q: Which of the following is a common bleeding manifestation of hemophilia?

Hemarthrosis. **Explanation:** Hemophilia (A and B) is a disorder of **secondary hemostasis** caused by deficiencies in clotting factors VIII and IX, respectively [1]. Unlike primary hemostatic defects (platelet disorders), which present with superficial mucosal bleeding, secondary hemostatic defects manifest as deep-seated bleeding. **Why Hemarthrosis is Correct:** **Hemarthrosis** (bleeding into joint spaces) is the hallmark clinical manifestation of hemophilia [1]. It occurs most commonly in the weight-bearing joints (knees, elbows, and ankles). Repeated episodes lead to synovial hypertrophy and chronic joint destruction, known as hemophilic arthropathy [1]. **Analysis of Incorrect Options:** * **Hemoptysis (A):** Bleeding from the respiratory tract is rare in hemophilia and usually suggests underlying pulmonary pathology (e.g., TB or malignancy) rather than a primary coagulation defect. * **Hematomas (C):** While large, deep muscle hematomas are common in hemophilia, they are generally considered less specific or "classic" than hemarthrosis [1]. In the context of a "most common/hallmark" question, hemarthrosis is the preferred answer. * **Hematuria (D):** Though it can occur in hemophiliacs, it is not the primary or most characteristic presenting feature [1]. **NEET-PG High-Yield Pearls:** * **Inheritance:** Both Hemophilia A and B are **X-linked recessive**. * **Lab Findings:** Characterized by a **prolonged aPTT** with a **normal PT and Bleeding Time (BT)**. * **Mixing Study:** The prolonged aPTT will **correct** upon mixing with normal plasma (differentiating it from factor inhibitors). * **Treatment:** Factor replacement is the mainstay. For mild Hemophilia A, **Desmopressin (DDAVP)** can be used as it releases stored Factor VIII and vWF from Weibel-Palade bodies. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 623-624.

Q: Popcorn cells are characteristic of which type of Hodgkin's lymphoma?

Lymphocyte predominance. **Explanation:** **Popcorn cells** (also known as **L&H cells** – Lymphocytic and Histiocytic variants) are the hallmark diagnostic feature of **Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)** [1]. Unlike the classic Reed-Sternberg (RS) cells, these cells have a multi-lobed, folded nucleus resembling a kernel of popped corn, with inconspicuous nucleoli and a B-cell phenotype (CD20+, CD45+, but CD15- and CD30-) [1][2]. **Analysis of Options:** * **Nodular Sclerosis (Option A):** Characterized by **Lacunar cells** (RS cells sitting in empty spaces due to formalin fixation) and broad bands of collagen fibrosis [2]. It is the most common subtype. * **Mixed Cellularity (Option B):** Characterized by **classic "Owl-eye" RS cells** (CD15+, CD30+) in a polymorphic background of eosinophils, plasma cells, and histiocytes [2]. Often associated with EBV. * **Lymphocyte Depletion (Option D):** The rarest and most aggressive form, showing numerous **pleomorphic/anaplastic RS cells** with very few background lymphocytes. **High-Yield Pearls for NEET-PG:** 1. **Immunophenotype:** Popcorn cells are **CD20+ and CD45+**, whereas Classic Hodgkin Lymphoma (CHL) cells are **CD15+ and CD30+**. 2. **Clinical Course:** NLPHL usually presents as localized peripheral lymphadenopathy (cervical/axillary) and has an excellent prognosis, though it can transform into Diffuse Large B-Cell Lymphoma (DLBCL) [1]. 3. **Background:** In NLPHL, the background consists predominantly of small B-lymphocytes arranged in a nodular pattern, unlike the T-cell rich background of CHL [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 618. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 616.

Q: Increased Prothrombin time results from a deficiency of which factor?

Fibrinogen. **Explanation:** Prothrombin Time (PT) measures the integrity of the **Extrinsic** and **Common pathways** of the coagulation cascade [1]. The factors involved in these pathways are Factors **I (Fibrinogen)**, **II (Prothrombin)**, **V**, **VII**, and **X** [1]. 1. **Why Fibrinogen is correct:** Fibrinogen (Factor I) is the final substrate in the common pathway [2]. It is converted into fibrin to form a stable clot. Since PT monitors the common pathway, a significant deficiency in Fibrinogen (<100 mg/dL) will lead to a prolonged PT [1]. 2. **Why the others are incorrect:** * **Factor IX and Factor XI:** These are components of the **Intrinsic pathway** [1]. Deficiencies in these factors (e.g., Hemophilia B for Factor IX) will prolong the **Activated Partial Thromboplastin Time (aPTT)**, but the PT will remain normal. * **Factor VI:** This is a historical term. There is no "Factor VI" in the modern coagulation cascade (it was originally thought to be activated Factor V). **High-Yield Clinical Pearls for NEET-PG:** * **PT** is the most sensitive marker for **Vitamin K deficiency** and **Warfarin therapy** because it measures Factor VII, which has the shortest half-life (approx. 6 hours) [3]. * **Isolated prolonged PT:** Suggests Factor VII deficiency or early liver disease. * **Prolonged PT and aPTT:** Suggests a deficiency in the **Common Pathway** (Factors I, II, V, X) or severe Vitamin K deficiency/liver failure [1]. * **Mixing Study:** If PT/aPTT corrects with normal plasma, it indicates a **factor deficiency**; if it does not correct, it indicates the presence of an **inhibitor** (e.g., Lupus anticoagulant) [1].

Question 1

A 15-year-old boy presented with a 1-day history of bleeding gums, subconjunctival bleed, and purpuric rash. Investigations revealed: Hb 6.4 gm/dL; TLC-26,500/mm3; prothrombin time-20 sec with a control of 13 sec; partial thromboplastin time-50 sec; and fibrinogen 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Accepted Answer

Promyelocytic leukemia

Answer

Myeloblastic leukemia without maturation

Answer

Myeloblastic leukemia with maturation

Answer

Myelomonocytic leukemia

Question 2

A 60-year-old man presented with fatigue, weight loss, and heaviness in the left hypochondrium for 6 months. The hemogram showed Hb 10 gm/dL, TLC 5 lakhs/mm3, platelet count 4 lakhs/mm3, and DLC showed neutrophils 55%, lymphocytes 4%, monocytes 2%, basophils 6%, metamyelocytes 10%, myelocytes 18%, promyelocytes 2%, and blasts 3%. What is the most likely cytogenetic abnormality in this case?

Accepted Answer

t(9;22)

Answer

t(1;21)

Answer

t(15;17)

Answer

Trisomy 21

Question 3

Small platelets on peripheral smear examination are seen in which of the following conditions?

Accepted Answer

Wiskott-Aldrich syndrome

Answer

Von Willebrand disease 2B

Answer

Gray platelet syndrome

Answer

Bernard-Soulier syndrome

Question 4

Dohle-like inclusions in neutrophils along with giant platelets is characteristic of which of the following conditions?

Accepted Answer

May-Hegglin anomaly

Answer

Bernard-Soulier syndrome

Answer

Chédiak-Higashi syndrome

Answer

Pelger-Huët anomaly

Question 5

Which of the following is a common bleeding manifestation of hemophilia?

Accepted Answer

Hemarthrosis

Answer

Hemoptysis

Answer

Hematomas

Answer

Hematuria

Question 6

Ringed sideroblasts are seen in which of the following conditions?

Accepted Answer

Myelodysplastic syndrome

Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Anemia of chronic disease

Question 7

Popcorn cells are characteristic of which type of Hodgkin's lymphoma?

Accepted Answer

Lymphocyte predominance

Answer

Nodular sclerosis

Answer

Mixed cellularity

Answer

Lymphocyte depletion

Question 8

Which of the following conditions follows an autosomal dominant inheritance pattern?

Accepted Answer

Hereditary spherocytosis

Answer

Albinism

Answer

Sickle cell anemia

Answer

Thalassemia

Question 9

What is a cause of Vitamin B12 deficiency megaloblastic anemia?

Accepted Answer

Fish tapeworm infestation

Answer

Dilantin therapy

Answer

Gastrectomy

Answer

Ileal resection

Question 10

Increased Prothrombin time results from a deficiency of which factor?

Accepted Answer

Fibrinogen

Answer

Factor IX

Answer

Factor VI

Answer

Factor XI

Hematopathology — MCQs

Hematopathology — MCQs

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