Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 1361: What is the most important diagnostic feature for beta thalassemia trait?

A. Increased HbF
B. Decreased MCH
C. Decreased MCV
D. Increased HbA2 (Correct Answer)

Explanation: ***Raised HbA2*** - In beta thalassemia trait, the most significant diagnostic feature is an **increased level of HbA2** (>3.5%), which helps differentiate it from other types of anemias [1]. - This is due to a compensatory mechanism as the body attempts to produce more **alpha globin chains** in response to decreased beta globin production. *Reduced MCV* - While **reduced mean corpuscular volume (MCV)** can indicate microcytic anemia, it is not specific enough for beta thalassemia trait as it can appear in other conditions. - MCV can vary in individuals, making it less reliable as a **diagnostic feature** compared to HbA2 levels. *Raised HbF* - An increase in **hemoglobin F (HbF)** is more characteristic of beta thalassemia major rather than the trait; levels in the trait do not typically rise significantly. - This feature can also be elevated in other conditions, thus not serving as a definitive marker for the trait. *Reduced MCH* - A **decreased mean corpuscular hemoglobin (MCH)** is indicative of microcytic anemia but lacks specificity for beta thalassemia trait alone. - Similar to MCV, it can occur in various types of anemia and does not pinpoint the diagnosis effectively. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 649-650.

Question 1362: Which type(s) of hemophilia is/are X-linked?

A. Hemophilia A
B. Hemophilia B
C. Hemophilia C
D. Both A & B (Correct Answer)

Explanation: ***Both A & B*** - Both **Hemophilia A** and **Hemophilia B** are **X-linked recessive** disorders [1][2] caused by deficiency of specific clotting factors (Factor VIII for A and Factor IX for B). - They are inherited through **carrier females**, affecting predominantly males, showcasing their X-linked transmission [1]. *Hemophilia A* - It is indeed an **X-linked recessive** disorder [2] but does not encompass all types of hemophilia. - It results from a deficiency in **Factor VIII** [2], leading to bleeding disorders, yet is just one form of hemophilia. *Hemophilia C* - This form is **autosomal recessive** and not linked to the X chromosome, primarily affecting both genders equally. - It involves a deficiency of **Factor XI**, distinguishing it from the X-linked nature of A and B. *Hemophilia B* - Like Hemophilia A, it is X-linked but by itself does not account for all hemophilias. - It results from a deficiency in **Factor IX**, yet the question about X-linkage applies to both A and B together. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, p. 151. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 670-671.

Question 1363: With regards to hereditary spherocytosis, which of the following is false?

A. Caused by mutations in genes for proteins such as spectrin, ankyrin or band 3
B. Usually has autosomal dominant inheritance
C. Aplastic crises are common (Correct Answer)
D. Red blood cells are destroyed in the spleen

Explanation: ***Aplastic crises are common*** - Aplastic crises occur in cases of **hereditary spherocytosis** due to parvovirus B19 infections, leading to **acute cessation of erythropoiesis** [1][3]. - They are characterized by a **sudden drop in hemoglobin levels**, which is not a common feature of hereditary spherocytosis itself, hence the misconception [1]. *Usually has autosomal dominant inheritance* - Hereditary spherocytosis is primarily inherited in an **autosomal dominant pattern**, where one copy of the mutated gene is sufficient to cause the disease. - However, it can also present in an **autosomal recessive** form, making this statement misleading. *Red blood cells are destroyed in the spleen* - This statement is actually **true**; hereditary spherocytosis leads to the **destruction of abnormally shaped red blood cells** in the spleen due to their rigid membrane structure [2][3]. - The condition is characterized by **hemolytic anemia** resulting from splenic sequestration [3]. *Caused by mutations in genes for proteins such as spectrin, ankrin or band 3* - This is a **correct statement**, as hereditary spherocytosis results from mutations in **membrane proteins** that result in loss of elasticity and stability of the red blood cell membrane [2]. - The mutations commonly involve **spectrin, ankyrin, and band 3 proteins**, leading to the pathophysiology seen in this condition [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 641-642. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-641. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598.

Question 1364: In which subtype of Hodgkin's disease are popcorn cells predominantly found?

A. Nodular sclerosis subtype
B. Mixed cellularity subtype
C. Lymphocyte predominant subtype (Correct Answer)
D. Lymphocyte depletion subtype

Explanation: ***Lymphocyte predominant*** - Popcorn cells, or **"L&H" (Lymphocytic and Histiocytic) cells**, are characteristic of lymphocyte predominant Hodgkin's disease, indicating a **rich lymphocytic background** [1]. - This subtype is more commonly seen in younger patients and is associated with a better prognosis relative to other types of Hodgkin's lymphoma [1]. *Mixed cellularity* - This subtype is characterized by a **varied cellular composition** including numerous Reed-Sternberg cells but does not specifically feature popcorn cells [2]. - It tends to have a higher number of **eosinophils** and **plasma cells** rather than lymphocytes predominating [2]. *Lymphocyte depletion* - Lymphocyte depletion is marked by a **deficient lymphocytic component** and an abundance of Reed-Sternberg cells, lacking the characteristic popcorn cells. - This variant is often more aggressive and typically has a poorer prognosis. *Nodular sclerosis* - Nodular sclerosis is recognized by the presence of **collagen bands** separating lymphoid nodules and also does not include popcorn cells. - This form is the most common variant of Hodgkin's lymphoma and often presents with **mediastinal masses**. **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, pp. 616-618.

Question 1365: Blood is stored at what temperature in blood bank?

A. -2 to -4 degrees Celsius
B. -2 to 0 degrees Celsius
C. 1 to 6 degrees Celsius (Correct Answer)
D. 6 to 12 degrees Celsius

Explanation: ***1 to 6 degrees Celsius*** - This temperature range is critical for maintaining the **viability of red blood cells** and slowing down metabolic processes, ensuring the blood is safe for transfusion. - At this temperature, the growth of most **pathogenic bacteria** is inhibited, and the degradation of blood components is minimized. *-2 to -4 degrees Celsius* - Temperatures below 0°C would cause **ice crystal formation** within the red blood cells, leading to hemolysis and making the blood unsuitable for transfusion. - This range is typically used for the **long-term storage of frozen plasma** or cryoprecipitate after special processing, not whole blood or red blood cell units. *-2 to 0 degrees Celsius* - This temperature range is incorrect for storing whole blood or packed red blood cells as it is still likely to cause **freezing and cellular damage**. - While close to freezing, even slight freezing can result in **hemolysis** and render the blood product unusable. *6 to 12 degrees Celsius* - Temperatures above 6°C significantly increase the **metabolic activity of red blood cells** and the risk of **bacterial growth**. - This range would lead to a more rapid decline in the viability and safety of the stored blood, reducing its shelf life considerably.

Question 1366: What is the defect in Glanzmann's thrombasthenia?

A. GpIIb-IIIa (Correct Answer)
B. GpIIIa-Ib
C. GpIIa-IIIb
D. GpIIb-Ia

Explanation: ***Gp1Ib-IlIa*** - Glanzmann's thrombasthenia is characterized by a defect in the **Gp1b-IlIa complex**, which is essential for platelet aggregation. - Patients with this condition exhibit **prolonged bleeding time** and poor clot formation due to ineffective platelet-fibrinogen binding. *GpIIIa-I lb* - This option refers to a component of the GpIIb-IIIa complex, which is primarily involved in **platelet aggregation** and is not the defect in Glanzmann's thrombasthenia. - Defects in GpIIb-IIIa lead to conditions such as **Glanzmann's thrombasthenia** [1], but specifically, the Gp1b-IlIa complex is implicated. *Gpfilb-I la* - This designation does not correspond correctly to either a known receptor complex related to platelet function or to Glanzmann's thrombasthenia. - Mislabeling of the glanzmann's complex results in confusion as it does not specify the **correct interaction involved in platelet aggregation**. *GpIla-Illb* - This combination refers to components that are not involved in Glanzmann's thrombasthenia, which specifically involves the **Gp1b-IlIa interaction**. - GpIla-Illb complex is part of the **integrin family**, but its defect does not lead to the bleeding problems seen in Glanzmann's thrombasthenia [1]. **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.

Question 1367: In a blood typing test, no agglutination is observed with anti-A and anti-B antisera, but agglutination is observed with anti-D (Rh) antisera. What is the blood type?

A. O-ve
B. A+ve
C. B-ve
D. O+ve (Correct Answer)

Explanation: ***O+ve*** - No agglutination with **anti-A** and **anti-B antisera** indicates the absence of A and B antigens, characteristic of **blood group O**. - Agglutination with **anti-D (Rh) antisera** signifies the presence of the **Rh antigen**, thereby indicating a positive (+) blood type. *O-ve* - While no agglutination with anti-A and anti-B points to **blood group O**, no agglutination with **anti-D** would be expected for an Rh-negative type. - The observed agglutination with **anti-D** contradicts an Rh-negative classification. *A+ve* - Agglutination with **anti-A antisera** would be expected for an A blood type, which is not observed in this scenario. - While agglutination with **anti-D** is consistent with 'positive', the absence of agglutination with **anti-A** rules out 'A' blood group. *B-ve* - Agglutination with **anti-B antisera** would be expected for a B blood type, which is not observed in this scenario. - The agglutination with **anti-D** contradicts an Rh-negative classification, making **B-ve** incorrect.

Question 1368: Bernard–Soulier syndrome is caused by a deficiency of which glycoprotein complex?

A. TNF
B. von Willebrand factor (vWf)
C. Gp Ib-IX-V complex (Correct Answer)
D. Gp IIb/IIIa

Explanation: ***Gp 1b*** - Bernard–Soulier syndrome is primarily caused by a deficiency in **Gp1b**, which is crucial for platelet adhesion to the von Willebrand factor (vWF) [1]. - This results in **thrombocytopenia** and large platelets, which are characteristic features of the syndrome. *Gp 2b/3a* - Gp2b/3a is associated with **Glanzmann thrombasthenia**, not Bernard–Soulier syndrome [1]. - This receptor is essential for platelet aggregation and binds fibrinogen, contributing to a different bleeding disorder. *TNF* - Tumor Necrosis Factor (TNF) is a cytokine involved in systemic inflammation and does not directly relate to platelet function or deficiencies. - Deficiency of TNF is unrelated to bleeding disorders like Bernard–Soulier syndrome. *vWf* - von Willebrand factor (vWf) deficiency is associated with **von Willebrand disease**, which presents differently than Bernard–Soulier syndrome. - vWf is essential for the aggregation of platelets but is not the deficient factor in this syndrome. **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.

Question 1369: Which disease is associated with the CD59 marker?

A. PNH (Correct Answer)
B. Cowden syndrome
C. Bannayan-Riley-Ruvalcaba syndrome
D. PTEN hamartoma tumor syndrome

Explanation: ***PNH*** - The **CD59 marker** is associated with **Paroxysmal Nocturnal Hemoglobinuria (PNH)**, a condition characterized by the loss of glycosylphosphatidylinositol (GPI) anchored proteins [1]. - It protects red blood cells from **complement-mediated lysis**, and its absence leads to hemolysis and thrombosis in patients with PNH [1,4]. *PTEN* - The **PTEN gene** is a tumor suppressor associated with various cancers and is not related to CD59. - It is primarily involved in the **regulation of the Akt signaling pathway**, not in complement regulation. *BRR* - **BRR (Birt-Hogg-Dubé syndrome)** is linked to folliculin and does not involve CD59. - This genetic condition is characterized by **skin tumors** and renal tumors, unrelated to the complement system. *Cowden syndrome* - **Cowden syndrome** is associated with mutations in the PTEN gene, relating to **hamartomas** and breast cancer risk, not CD59. - It affects multiple systems but does not involve **complement regulatory proteins** like CD59. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 650-651. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 601-602.

Question 1370: In which type of Hodgkin's lymphoma are classical Reed-Sternberg cells most characteristically observed?

A. Lymphocyte depleted
B. Nodular sclerosis
C. Lymphocyte predominance
D. Mixed cellularity Hodgkin (Correct Answer)

Explanation: ***Lymphocyte predominance*** - The **Hodgkin's lymphoma (HL) lymphocyte predominance** variant characteristically displays a predominance of lymphocytes in the cellular makeup [1]. - This subtype is often associated with a better prognosis and fewer symptoms than other types of HL [1]. *Lymphocyte depleted* - This subtype features a significant decrease in lymphocytes, leading to a **higher proportion of Reed-Sternberg cells** [3]. - It typically presents with a more aggressive clinical course, which contrasts with lymphocyte predominance [3]. *Mixed cellularity hodgkin* - Mixed cellularity shows a variety of cell types, including a significant number of **Reed-Sternberg cells**, but does not demonstrate **lymphocyte predominance** [2]. - This subtype is generally found in older patients and associated with advanced disease, unlike lymphocyte predominance [2]. *Nodular sclerosis* - Nodular sclerosis subtype is characterized by **collagen bands** and a particular architecture that is distinct from lymphocyte predominance [2]. - It primarily affects younger patients and can often involve mediastinal lymph nodes; however, it does not have the features of lymphocyte predominance [2]. **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, pp. 616-618. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 559-560.

Practice by Chapter

Anemias: Classification and Approach

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Hemolytic Anemias

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Myeloproliferative Neoplasms

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Myelodysplastic Syndromes

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Acute Leukemias

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Chronic Leukemias

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Lymphomas and Lymphoid Neoplasms

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Plasma Cell Disorders

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Bleeding Disorders

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Thrombotic Disorders

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