Hematopathology Practice Questions

Q: Gandy-Gandy bodies are typically seen in chronic venous congestion of which of the following organs?

Spleen. **Explanation:** **Gandy-Gamna bodies** (also known as Gamna-Gandy bodies or Siderofibrotic nodules) are a classic histopathological finding in the **Spleen**. 1. **Mechanism (Why Spleen is correct):** In conditions causing chronic venous congestion of the spleen (most commonly **Portal Hypertension**), there is increased pressure leading to focal hemorrhages within the splenic parenchyma. As the blood breaks down, deposits of **hemosiderin** (iron) and **calcium** form on the fibrous connective tissue and elastic fibers [1]. Microscopically, these appear as yellow-brown or rust-colored nodules consisting of fibrous tissue, iron, and calcium salts. 2. **Analysis of Incorrect Options:** * **Lung:** Chronic venous congestion of the lung (often due to left heart failure) leads to the formation of **Heart Failure Cells** (hemosiderin-laden macrophages in the alveoli), not Gandy-Gamna bodies. * **Liver:** Chronic venous congestion here results in the **"Nutmeg Liver"** appearance due to centrilobular necrosis and congestion, but it does not typically form siderofibrotic nodules. * **Kidney:** Congestion in the kidney leads to dusky, enlarged organs but lacks the specific fibro-calcific and iron-laden reaction seen in the spleen. **High-Yield Clinical Pearls for NEET-PG:** * **Staining:** Gandy-Gamna bodies stain positive with **Prussian Blue** (for iron) and **Von Kossa** (for calcium). * **Common Associations:** Portal hypertension (due to Cirrhosis), Sickle Cell Anemia, and Splenic Lymphoma. * **Radiology:** On MRI, they appear as multiple low-signal intensity foci (due to the paramagnetic effect of iron) on T2-weighted sequences, often referred to as the "tobacco-fleck" appearance. **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, pp. 630-634.

Q: All are important laboratory features of hereditary spherocytosis, EXCEPT:

Reduced MCHC. In Hereditary Spherocytosis (HS), the primary defect lies in the red cell membrane proteins (most commonly **Ankyrin**, followed by Spectrin) [2], leading to a loss of membrane surface area. **Explanation of the Correct Answer:** * **Option A (Reduced MCHC):** This is the correct answer because it is **false**. In HS, the loss of membrane surface area relative to the volume results in the formation of spherical cells (spherocytes) [1]. These cells are dehydrated, leading to a relative increase in hemoglobin concentration within the cell. Therefore, **MCHC is typically increased (>36 g/dL)**, not reduced [1]. An elevated MCHC is a classic hallmark of HS. **Explanation of Incorrect Options:** * **Option B (Normocytic anemia):** While spherocytes appear small, their MCV is usually within the normal range or slightly low (80-100 fL) because the reticulocytosis (immature, larger RBCs) compensates for the smaller spherocytes. * **Option C (Positive Pink Test):** The "Pink Test" is a simplified version of the Glycerol Lysis Test. It measures the rate of hemolysis; spherocytes lyse more rapidly than normal cells in an acidic glycerol solution, yielding a positive result. * **Option D (Normal WBC count):** HS is a primary disorder of the RBC membrane. Unless there is a secondary infection or an acute crisis, the white blood cell and platelet counts remain normal. **High-Yield Clinical Pearls for NEET-PG:** 1. **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test (Flow cytometry). 2. **Classic Triad:** Anemia, Jaundice, and Splenomegaly. 3. **Peripheral Smear:** Spherocytes (lack central pallor) and Polychromasia (due to reticulocytosis). 4. **Complications:** Pigment gallstones (calcium bilirubinate) and Aplastic crisis (associated with Parvovirus B19). 5. **Treatment of Choice:** Splenectomy (usually deferred until after age 6 to reduce sepsis risk) [2].

Q: Which congenital syndromes are associated with lymphoproliferative malignancy?

Chediak Higashi syndrome. Explanation: The correct answer is Chediak-Higashi Syndrome (CHS). 1. Why Chediak-Higashi Syndrome is correct: CHS is an autosomal recessive disorder caused by a mutation in the LYST gene, leading to defective vesicle trafficking and the formation of giant lysosomal granules. The primary immunological defect involves impaired Natural Killer (NK) cell function and cytotoxic T-cell activity. A hallmark of CHS is the "Accelerated Phase," a hemophagocytic lymphohistiocytosis (HLH)-like syndrome characterized by a non-neoplastic but aggressive lymphoproliferative infiltration of organs (liver, spleen, lymph nodes), which often progresses to or mimics high-grade lymphoma. 2. Why the other options are incorrect: * Bloom Syndrome: While associated with a high risk of various cancers due to DNA helicase deficiency (sister chromatid exchanges), it is more classically linked to leukemias (AML/ALL) and solid tumors rather than primary lymphoproliferative syndromes. * Fanconi’s Anemia: This is a DNA repair defect primarily associated with Acute Myeloid Leukemia (AML) and squamous cell carcinomas. It is not typically categorized under lymphoproliferative malignancies. * Turner Syndrome (45, XO): This chromosomal anomaly is associated with an increased risk of gonadoblastoma (if Y chromatin is present) and germ cell tumors, but not lymphoproliferative disorders. 3. High-Yield Clinical Pearls for NEET-PG: * Chediak-Higashi Triad: Partial oculocutaneous albinism, recurrent pyogenic infections (Staph/Strep), and peripheral neuropathy. * Peripheral Smear: Look for pathognomonic giant azurophilic granules in neutrophils and precursors. * Other Immunodeficiencies with Lymphoma risk: Wiskott-Aldrich Syndrome, Ataxia-Telangiectasia, and Common Variable Immunodeficiency (CVID) are also high-yield associations for lymphoproliferative malignancy.

Q: Ring sideroblasts, characterized by iron-laden mitochondria, are found in which cellular organelle?

Mitochondria. **Explanation:** **1. Why Mitochondria is Correct:** Ring sideroblasts are hallmark cells found in **Sideroblastic Anemia**. The underlying pathology involves a defect in heme synthesis, specifically the inability to incorporate iron into protoporphyrin IX. When this process fails, iron continues to enter the erythroid precursor but cannot be utilized. This excess iron accumulates within the **mitochondria**, which are arranged in a "necklace-like" or perinuclear distribution around at least one-third of the nucleus [1]. On a **Prussian Blue (Perls') stain**, these iron-laden mitochondria appear as distinct blue granules, forming the characteristic "ring." **2. Why Other Options are Incorrect:** * **Endoplasmic Reticulum:** While involved in protein synthesis and calcium storage, the ER does not play a direct role in the terminal steps of heme synthesis or pathological iron sequestration. * **Nucleus:** Iron does not accumulate within the nucleus in sideroblastic anemia. The "ring" appearance is perinuclear (around the nucleus), not intranuclear. * **Nuclear Membrane:** Although the iron-laden mitochondria cluster closely around the nucleus, they are distinct organelles located in the cytoplasm and are not part of the nuclear membrane structure itself. **3. NEET-PG High-Yield Pearls:** * **Stain of Choice:** Prussian Blue (Perls') stain is essential to visualize ring sideroblasts. * **Definition:** A "ring sideroblast" must have $\geq$ 5 iron granules covering at least one-third of the nuclear circumference. * **Common Causes:** Hereditary (ALAS2 mutation), Lead poisoning, Alcoholism, Isoniazid (Vitamin B6 deficiency), and Myelodysplastic Syndrome (MDS-RS). * **Key Enzyme:** The most common hereditary defect is in **5-aminolevulinate synthase (ALAS2)**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. With Illustrations By, pp. 18-19.

Q: Which of the following is the investigation of choice for CML?

Karyotyping. ***Karyotyping (Conventional Cytogenetics)*** - **Gold standard** and investigation of choice for CML diagnosis - Detects the **Philadelphia chromosome t(9;22)** present in 95% of CML cases [2] - WHO diagnostic criterion for CML - Can identify additional chromosomal abnormalities with prognostic significance - Provides complete chromosomal analysis *FISH (Fluorescence In Situ Hybridization)* - Used as **complementary technique** when karyotyping fails or metaphases are inadequate - More sensitive than karyotyping but NOT the first-line investigation [2] - Useful for monitoring minimal residual disease - Cannot detect additional chromosomal abnormalities *Molecular testing (BCR-ABL PCR)* - Used for **monitoring treatment response** and detecting minimal residual disease [1] - Highly sensitive and quantitative [1] - Not the initial investigation of choice for diagnosis *LAP score (Leukocyte Alkaline Phosphatase)* - Used to differentiate CML from leukemoid reaction - Low in CML, high in leukemoid reaction - Older diagnostic tool, now largely replaced by molecular methods - Not the investigation of choice **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 185-187. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 225-226.

Question 1

Gandy-Gandy bodies are typically seen in chronic venous congestion of which of the following organs?

Accepted Answer

Spleen

Answer

Lung

Answer

Kidney

Answer

Liver

Question 2

All are important laboratory features of hereditary spherocytosis, EXCEPT:

Accepted Answer

Reduced MCHC

Answer

Normocytic anemia

Answer

Positive pink test

Answer

Normal WBC count

Question 3

Which of the following is true about Burkitt's lymphoma?

Accepted Answer

Shows a starry sky pattern on histology

Answer

Associated with t(14;18)

Answer

Expresses BCL-2

Answer

Slowly progressing

Question 4

Which congenital syndromes are associated with lymphoproliferative malignancy?

Accepted Answer

Chediak Higashi syndrome

Answer

Bloom syndrome

Answer

Fanconi's anemia

Answer

Turner syndrome

Question 5

The typical bone marrow finding in Idiopathic Thrombocytopenic Purpura (ITP) is:

Accepted Answer

Increased megakaryocytes

Answer

Absent megakaryocytes

Answer

Foam cells

Answer

Fragmented megakaryocytes

Question 6

Ring sideroblasts, characterized by iron-laden mitochondria, are found in which cellular organelle?

Accepted Answer

Mitochondria

Answer

Endoplasmic reticulum

Answer

Nucleus

Answer

Nuclear membrane

Question 7

A 40-year-old man presents with a 5-day history of productive cough and fever. *Pseudomonas aeruginosa* is isolated from a pulmonary abscess. A complete blood count (CBC) shows a marked leukocytosis (50,000/mL) with a left shift in granulocytes. Which of the following terms best describes these hematologic findings?

Accepted Answer

Leukemoid reaction

Answer

Leukopenia

Answer

Myeloid metaplasia

Answer

Neutrophilia

Question 8

Which of the following is not a B cell marker?

Accepted Answer

CD 134

Answer

CD 19

Answer

CD 20

Answer

CD 10

Question 9

A 28-year-old female presents with prolonged bleeding time and a normal platelet count. She reports a history of easy bruising, frequent gum bleeding, epistaxis, cutaneous bleeding, and menorrhagia. Further testing revealed a deficiency of Von Willebrand factor. Which of the following thrombogenic processes involving platelets is most directly impaired?

Accepted Answer

Adhesion

Answer

Aggregation

Answer

Formation of fibrinogen bridges

Answer

Conformational change with activation of phospholipid surface

Question 10

Which of the following is the investigation of choice for CML?

Accepted Answer

Karyotyping

Answer

LAP score

Answer

FISH

Answer

Molecular testing (BCR-ABL PCR)

Hematopathology — MCQs

Hematopathology — MCQs

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