Hematopathology Practice Questions

Q: Stored blood as compared to fresh blood has:

High extracellular K+. **Explanation:** The storage of blood leads to a series of biochemical and morphological changes collectively known as the **"Storage Lesion."** **1. Why Option B is Correct:** During storage at 1–6°C, the **Na+/K+ ATPase pump** on the red blood cell (RBC) membrane becomes sluggish due to the cold temperature and gradual depletion of ATP. This prevents the pump from maintaining the ionic gradient, causing potassium to leak out of the RBCs into the plasma. Consequently, **extracellular K+ levels increase** significantly over time. This is clinically vital because rapid transfusion of stored blood can lead to **hyperkalemia**, potentially causing cardiac arrhythmias. **2. Why Other Options are Incorrect:** * **Option A:** **2,3-DPG levels decrease** during storage. 2,3-DPG is essential for oxygen release to tissues; its depletion causes a "left shift" in the oxygen dissociation curve (increased oxygen affinity). * **Option C:** While some hemolysis occurs, **high extracellular hemoglobin** is not the primary characteristic change compared to the significant rise in potassium, unless the blood is expired or damaged. * **Option D:** Platelets are highly sensitive to cold. They lose their viability and function within 48–72 hours of storage at 1–6°C. Therefore, stored blood has **decreased/non-functional platelets.** **Clinical Pearls for NEET-PG:** * **pH:** Decreases (becomes acidic) due to lactic acid accumulation. * **Sodium:** Decreases (moves into the RBC). * **Factors V and VIII:** These are labile clotting factors and their levels decrease in stored blood. [1] * **Citrate Toxicity:** Massive transfusion of stored blood can lead to **hypocalcemia** because the citrate anticoagulant binds to the patient's ionized calcium. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 625-626.

Q: Presence of Birbeck granules in histopathological examination suggests which of the following?

Langerhan cells histiocytosis.. **Explanation:** **Langerhans Cell Histiocytosis (LCH)** is the correct answer. Birbeck granules are the pathognomonic ultrastructural hallmark of Langerhans cells [1]. On Electron Microscopy (EM), these are pentalaminar, rod-shaped cytoplasmic organelles with a central striated line and a bulbous end, giving them a characteristic **"Tennis Racket" appearance** [1]. These granules contain **Langerin (CD207)**, a protein involved in capturing and internalizing antigens [1]. **Why other options are incorrect:** * **Plasmacytoma:** This is a neoplastic proliferation of plasma cells. Histology shows "clock-face" nuclei and perinuclear halos (Golgi zone). Immunophenotype is positive for CD138 and CD38, not Birbeck granules. * **Burkitt Lymphoma:** This is a high-grade B-cell lymphoma characterized by a **"Starry Sky" appearance** (tingible body macrophages against a background of small malignant lymphocytes) [2]. It is associated with the t(8;14) translocation and c-MYC overexpression. **High-Yield Clinical Pearls for NEET-PG:** * **Immunohistochemistry (IHC) Markers for LCH:** CD1a (most specific), **S100** (sensitive), and **CD207 (Langerin)**. * **Clinical Presentation:** Can range from a solitary bone lesion (Eosinophilic Granuloma) to multisystem involvement (Letterer-Siwe disease). * **Hand-Schüller-Christian Disease:** A classic triad of LCH consisting of bone lesions (calvarium), exophthalmos, and diabetes insipidus. * **Radiology:** "Punched-out" lytic lesions in the skull are a frequent finding. **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. 629-630. [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. 606.

Q: Von Willebrand factor protects which coagulation factor from premature degradation?

Factor VIII. **Explanation:** **1. Why Factor VIII is correct:** Von Willebrand Factor (vWF) serves two primary roles in hemostasis: it facilitates platelet adhesion to subendothelial collagen and acts as a **carrier protein for Factor VIII (FVIII)** [1] in the circulation. In the absence of vWF, FVIII is highly unstable and undergoes rapid proteolytic degradation by activated Protein C and Factor Xa. By binding to FVIII, vWF increases its half-life from approximately 1–2 hours to 12 hours. This explains why patients with severe Von Willebrand Disease (vWD) often have secondary deficiencies of Factor VIII, leading to a prolonged Activated Partial Thromboplastin Time (aPTT). **2. Why other options are incorrect:** * **Factor II (Prothrombin):** This is a vitamin K-dependent factor synthesized in the liver. Its stability is not dependent on vWF. * **Factor V:** While Factor V shares structural homology with Factor VIII, it does not bind to vWF. It circulates freely or is stored within platelet alpha-granules. * **Factor X:** This factor is part of the common pathway and is activated by the tenase complex. It does not require a carrier protein for stabilization in the plasma. **3. Clinical Pearls for NEET-PG:** * **Synthesis:** vWF is synthesized in **endothelial cells** (stored in **Weibel-Palade bodies**) and **megakaryocytes** (stored in **alpha-granules** [1] of platelets). * **Ristocetin Cofactor Assay:** This is the gold standard for testing vWF function; ristocetin induces vWF to bind to platelet GP Ib/IX/V receptors. * **Treatment:** Desmopressin (DDAVP) is used in Type 1 vWD as it triggers the release of vWF and Factor VIII from endothelial stores. * **Inheritance:** Most forms of vWD are Autosomal Dominant, making it the most common inherited bleeding disorder. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 669-670.

Q: Leucocyte alkaline phosphatase (LAP) is raised in which of the following conditions?

Chronic myeloid leukemia. **Explanation:** The **Leucocyte Alkaline Phosphatase (LAP) score** (also known as the Neutrophil Alkaline Phosphatase/NAP score) measures the activity of the enzyme alkaline phosphatase within the secondary granules of mature neutrophils. **Correct Answer: D. Chronic Myeloid Leukemia (CML)** In CML, the LAP score is characteristically **decreased** (often near zero) [1]. This occurs because the malignant neutrophils produced in CML are functionally defective and lack the enzyme. Therefore, a low LAP score is a classic diagnostic marker used to differentiate CML from a Leukemoid reaction (where the score is high) [1]. **Analysis of Options:** * **A, B, and C (Myelofibrosis, Essential Thrombocythemia, Polycythemia Vera):** These are all Chronic Myeloproliferative Neoplasms (MPNs) [2]. Unlike CML, the LAP score in these conditions is typically **normal or elevated**. Specifically, in Polycythemia Vera, a high LAP score is a common finding [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Decreased LAP Score:** Seen in CML, Paroxysmal Nocturnal Hemoglobinuria (PNH), Hypophosphatasia, and Sideroblastic Anemia. * **Increased LAP Score:** Seen in **Leukemoid Reaction** (most common cause), Pregnancy, Polycythemia Vera, and during acute infections or treatment with G-CSF. * **The "CML vs. Leukemoid" Rule:** This is a favorite exam topic. If a patient has a massive shift to the left (high WBC count): * High LAP = Leukemoid Reaction. * Low LAP = CML. * **Note on CML:** The LAP score may normalize or increase if a patient with CML develops an infection or enters a **Blast Crisis**. **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. 624-629. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.

Question 1

Crumpled tissue paper appearance of cytoplasm in bone marrow examination is due to the intracellular accumulation of which of the following?

Accepted Answer

Glucocerebroside

Answer

Cerebroside

Answer

Ganglioside

Answer

Sphingomyelin

Question 2

Prolonged PT and normal PTT may be seen in which of the following conditions?

Accepted Answer

None of the above

Answer

Thrombocytopenia

Answer

Disseminated Intravascular Coagulation (DIC)

Answer

Vitamin K deficiency

Question 3

A 6-month-old child with sickle cell anemia has a chronically enlarged spleen. By 5 years of age, the child's spleen is no longer palpable. The decrease in size is most likely related to which of the following?

Accepted Answer

Infarctions

Answer

Chronic infection

Answer

Hodgkin's lymphoma

Answer

Non-Hodgkin's lymphoma

Question 4

A round cell having fine nuclear chromatin, prominent nucleoli, and fine azurophilic granules. What is this cell?

Accepted Answer

Myeloblast

Answer

Lymphoblast

Answer

Monoblast

Answer

None of the above

Question 5

Which of the following coagulation factors is deficient in classical hemophilia?

Accepted Answer

VIII

Answer

IX

Answer

X

Answer

XII

Question 6

Stored blood as compared to fresh blood has:

Accepted Answer

High extracellular K+

Answer

More 2,3 DPG

Answer

High extracellular hemoglobin

Answer

Increased platelets

Question 7

Presence of Birbeck granules in histopathological examination suggests which of the following?

Accepted Answer

Langerhan cells histiocytosis.

Answer

Plasmacytoma.

Answer

Burkett lymphoma.

Answer

All of the above.

Question 8

Polycythemia vera may transform into which of the following myeloid neoplasms?

Accepted Answer

Acute myeloid leukemia (AML)

Answer

Acute lymphoblastic leukemia (ALL)

Answer

Chronic myeloid leukemia (CML)

Answer

Renal cell carcinoma

Question 9

Von Willebrand factor protects which coagulation factor from premature degradation?

Accepted Answer

Factor VIII

Answer

Factor II

Answer

Factor V

Answer

Factor X

Question 10

Leucocyte alkaline phosphatase (LAP) is raised in which of the following conditions?

Accepted Answer

Chronic myeloid leukemia

Answer

Myelofibrosis

Answer

Essential thrombocythemia

Answer

Polycythemia

Hematopathology — MCQs

Hematopathology — MCQs

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