Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 1361: 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 1362: What is the neutrophil count for moderate neutropenia?

A. <500/mm3
B. 500 - 1000/mm3 (Correct Answer)
C. >1000/mm3
D. 100/mm3

Explanation: ***500 - 1000/mm3*** - **Moderate neutropenia** is defined as an absolute neutrophil count (ANC) between **500 and 1000 cells/mm³**. - Patients in this range have an increased risk of infection, although generally less severe than those with very low counts. *<500/mm3* - An absolute neutrophil count of **less than 500 cells/mm³** indicates **severe neutropenia**, which is associated with a significantly higher risk of life-threatening infections. - Patients with counts below 500 cells/mm³ are often considered to be at risk for **febrile neutropenia**. *>1000/mm3* - An absolute neutrophil count of **greater than 1000 cells/mm³** is generally considered within the **mild neutropenia** or even **normal** range, depending on the exact cutoff used in specific contexts (normal range typically 1500-8000 cells/mm³). - This level of neutropenia is usually not clinically significant in terms of increased infection risk. *100/mm3* - An absolute neutrophil count of **100 cells/mm³** falls into the category of **severe neutropenia** (also known as agranulocytosis), which is a critical level. - This count indicates an extremely high risk of serious bacterial and fungal infections.

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: 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 1366: 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 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: Which of the following is an example of an intracorpuscular defect causing hemolysis?

A. Paroxysmal nocturnal hemoglobinuria (PNH) (Correct Answer)
B. Toxins in uremia
C. Paroxysmal Cold Hemoglobinuria
D. Mechanical trauma in portal hypertension

Explanation: ***PNH*** - **Paroxysmal Nocturnal Hemoglobinuria (PNH)** is caused by a deficiency of glycosylphosphatidylinositol (GPI) anchors, leading to **intracorpuscular defects** in red blood cells [1]. - It results in **hemolysis**, mainly due to increased complement-mediated lysis and associated symptoms like **hemoglobinuria** and dark urine upon waking [1]. *Portal hypertension* - Portal hypertension primarily affects the **portal venous system** and is not directly related to **intracorpuscular defects** in hemolysis. - It can lead to **splenomegaly** and subsequent sequestration of blood cells but does not cause intrinsic RBC defects. *PCH* - **Paroxysmal Cold Hemoglobinuria (PCH)** involves hemolysis due to exposure to cold and is associated with **IgG antibodies** against RBCs, but it's not an **intracorpuscular** defect. - PCH is characterized by hemolysis occurring in response to cold temperatures, rather than an intrinsic defect within the RBCs themselves. *Uremic syndrome* - Uremic syndrome arises due to **kidney failure**, leading to a buildup of waste products and a distinct form of **extracorpuscular hemolysis**, not intracorpuscular defects. - While it can cause anemia, it does not directly involve intrinsic RBC abnormalities like those seen in PNH. **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] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 642-643. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598.

Question 1369: 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.

Question 1370: 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.

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