Hematopathology Practice Questions

Q: Which disease is associated with the CD59 marker?

PNH. ***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.

Q: TRALI occurs within how many hours of transfusion?

6 hours. ***6 hours*** - **Transfusion-related acute lung injury (TRALI)** is defined as new acute lung injury occurring during or within **6 hours** after the completion of a blood transfusion [1]. - It is a severe and potentially life-threatening transfusion reaction characterized by **acute respiratory distress**, **hypoxemia**, and **bilateral pulmonary infiltrates** on chest imaging [1]. *48 hours* - While other transfusion reactions or complications may manifest within 48 hours, TRALI has a more **acute onset**, typically within the first 6 hours. - A pulmonary event occurring between 6 and 48 hours post-transfusion might be considered **delayed TRALI** or another diagnosis like **transfusion-associated circulatory overload (TACO)**, but the classic definition refers to the 6-hour window. *72 hours* - Reactions occurring 72 hours after transfusion are generally considered **delayed transfusion reactions**, which include conditions like **delayed hemolytic transfusion reactions** or **post-transfusion purpura**. - This timeframe is too long for the typical presentation of TRALI, which is characterized by rapid onset. *12 hours* - Although 12 hours falls within an acute window, the most commonly accepted and diagnostically crucial timeframe for TRALI is **within 6 hours** of transfusion. - A reaction occurring between 6 and 12 hours would still be considered suspiciously TRALI, but the strict definition emphasizes the earlier onset.

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

Gp Ib-IX-V complex. ***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.

Q: Whole blood is used as a sample for which test?

Complete Blood Count (CBC). **Complete Blood Count (CBC)** - A **CBC** directly analyzes the cellular components of blood (red blood cells, white blood cells, and platelets) which are found suspended in **whole blood**. [1] - It provides information on cell counts, sizes, and hemoglobin concentration, requiring the complete, unaltered blood mixture for accurate results. [3] *Interferon Gamma Release Assay (IGRA)* - IGRAs typically use **whole blood** in specialized collection tubes, but they are not measuring components *within* the whole blood itself. - Instead, they detect **interferon-gamma release** from T-cells stimulated by specific antigens, a cellular immune response assessed in a stimulated blood sample. *HIV ELISA* - **ELISA** (Enzyme-Linked Immunosorbent Assay) for HIV primarily detects **antibodies** or **antigens** in the blood. - For this, **plasma** or **serum** (the liquid components of blood after cells have been removed) is typically preferred over whole blood. *Blood Culture for Bacterial Infections* - **Blood cultures** require whole blood to be inoculated directly into specific culture media. [2] - The goal is to grow and identify **live bacteria** circulating in the bloodstream, making whole blood essential, but it is incorrect when compared to the direct cellular analysis of a CBC.

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

Mixed cellularity Hodgkin. ***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.

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

Paroxysmal nocturnal hemoglobinuria (PNH). ***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.

Q: Which marker is commonly associated with positivity in follicular lymphoma?

Bcl-2. ***Bcl-2*** - **Follicular lymphoma** is characterized by the overexpression of the **Bcl-2 protein**, which inhibits apoptosis, leading to the survival of malignant B cells [1][3]. - The **Bcl-2 gene** is often involved in the **t(14;18)** chromosomal translocation, which is a hallmark of this lymphoma [1][3][4]. *Bcl-6* - Although **Bcl-6** can be expressed in some lymphomas, it is primarily associated with **diffuse large B-cell lymphoma**, not follicular lymphoma. - **Bcl-6** is involved in **germinal center formation** and its positivity does not indicate follicular lymphoma specifically. *Bcl-1* - **Bcl-1** (also known as **CCND1**) is primarily associated with **mantle cell lymphoma** and is not a characteristic marker for follicular lymphoma. - It is linked to the **t(11;14)** translocation, which is distinct from the genetic alterations seen in follicular lymphoma. *None of the above* - This option is incorrect as **Bcl-2 positivity** is definitive for follicular lymphoma [2]. - The presence of other markers like **Bcl-6** or **Bcl-1** does not negate the expression of Bcl-2 in this lymphoma type. **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. 602-604. [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. 604. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 561-562. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 310-311.

Q: Which of the following statements about cross-matching of blood is false?

Donor serum is tested against recipient packed cells. ***Donor serum is tested against recipient packed cells*** - This statement is **FALSE** and describes a **minor crossmatch**, which is rarely performed in modern transfusion practice. - The minor crossmatch tests donor antibodies against recipient cells, but this is not standard practice because donor plasma is significantly diluted during transfusion, making clinically significant reactions rare. - Modern blood banking focuses on the **major crossmatch** as the critical safety measure. *Recipient serum is tested against donor packed cells* - This statement is **TRUE** and accurately describes the **major crossmatch**, which is the standard and most critical pre-transfusion compatibility test. - The major crossmatch detects antibodies in the recipient's serum that could react with donor red blood cell antigens, preventing potentially fatal hemolytic transfusion reactions. *Mandatory in all cases except emergency* - This statement is **TRUE**. Crossmatching is mandatory for safe transfusion practice. - In life-threatening emergencies where delay could be fatal, uncrossmatched O-negative (universal donor) blood may be given, but this is a rare exception. *Involves visible agglutination* - This statement is **TRUE**. A positive crossmatch indicating incompatibility is identified by **visible agglutination** or **hemolysis**. - These visible reactions occur when recipient antibodies bind to donor red blood cell antigens, signaling that transfusion would cause a severe reaction.

Q: The Ham test is specifically used for diagnosing paroxysmal nocturnal hemoglobinuria (PNH) and is based upon:

Complement. ***Complement*** - The HAM test is based on the activation of the **complement system** which enhances the opsonization and clearance of immune complexes [1]. - It is used in the diagnosis of certain conditions, notably those associated with **hemolytic anemia** due to complement fixation. *GPI Anchor Proteins* - GPI anchor proteins are involved in anchoring proteins to cell membranes but are **not related to the HAM test**. - This oes not explain the **mechanism** or purpose of the HAM test. *Mannose binding proteins* - Mannose binding lectins play a role in **innate immunity** but are not the basis of the HAM test. - They function in the **opsonization of pathogens**, which is unrelated to the complement activation aspect of the HAM test. *Spectrin protein* - Spectrin is a cytoskeletal protein that contributes to the integrity of cell membranes, particularly in red blood cells. - It does not relate to the **mechanism of the HAM test**, which focuses on complement involvement. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Inflammation and Repair, pp. 99-100.

Q: Indirect Coombs test detects:

Antibodies in the serum. ***Antibodies in the serum*** - The **indirect Coomb's test** is designed to detect the presence of **antibodies** against red blood cells (RBCs in the serum) before transfusion or during pregnancy [1]. - It is crucial in identifying **hemolytic disease of the newborn** (HDN) and ensuring safe blood transfusions [1]. *Antibodies attached to RBC Surface* - This scenario describes the **direct Coomb's test**, which identifies antibodies that are already bound to **RBCs**. - Direct testing assesses conditions like **autoimmune hemolytic anemia**, not the serum. *Antigens attached to RBC Surface* - This option suggests evaluating **antigens** present on the surface of RBCs, which is not the purpose of the indirect Coomb's test. - Antigens are important in blood typing and compatibility assessments, but this test focuses on antibodies. *Antigens in the serum* - Indirect Coomb's does not detect **antigens** but rather the **antibodies** related to those antigens. - Additionally, serum testing for antigens is not a standard procedure for assessing transfusion compatibility. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 627-628.

Question 1

Which disease is associated with the CD59 marker?

Accepted Answer

PNH

Answer

Cowden syndrome

Answer

Bannayan-Riley-Ruvalcaba syndrome

Answer

PTEN hamartoma tumor syndrome

Question 2

TRALI occurs within how many hours of transfusion?

Accepted Answer

6 hours

Answer

48 hours

Answer

72 hours

Answer

12 hours

Question 3

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

Accepted Answer

Gp Ib-IX-V complex

Answer

TNF

Answer

von Willebrand factor (vWf)

Answer

Gp IIb/IIIa

Question 4

Whole blood is used as a sample for which test?

Accepted Answer

Complete Blood Count (CBC)

Answer

Interferon Gamma Release Assay (IGRA)

Answer

HIV ELISA

Answer

Blood Culture for Bacterial Infections

Question 5

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

Accepted Answer

Mixed cellularity Hodgkin

Answer

Lymphocyte depleted

Answer

Nodular sclerosis

Answer

Lymphocyte predominance

Question 6

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

Accepted Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Answer

Toxins in uremia

Answer

Paroxysmal Cold Hemoglobinuria

Answer

Mechanical trauma in portal hypertension

Question 7

Which marker is commonly associated with positivity in follicular lymphoma?

Accepted Answer

Bcl-2

Answer

Bcl-1

Answer

Bcl-6

Answer

None of the options

Question 8

Which of the following statements about cross-matching of blood is false?

Accepted Answer

Donor serum is tested against recipient packed cells

Answer

Mandatory in all cases except emergency

Answer

Involves visible agglutination

Answer

Recipient serum is tested against donor packed cells

Question 9

The Ham test is specifically used for diagnosing paroxysmal nocturnal hemoglobinuria (PNH) and is based upon:

Accepted Answer

Complement

Answer

GPI Anchor Proteins

Answer

Spectrin protein

Answer

Mannose binding proteins

Question 10

Indirect Coombs test detects:

Accepted Answer

Antibodies in the serum

Answer

Antibodies bound to RBC Surface

Answer

Antigens bound to RBC Surface

Answer

Antigens in the plasma

Hematopathology — MCQs

Hematopathology — MCQs

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