Hematopathology Practice Questions

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: 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 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: Which type of anemia is most commonly characterized by marked poikilocytosis and anisocytosis on peripheral blood smear?

Iron deficiency anemia. ***Iron deficiency anaemia*** - Characterized by **poikilocytosis** (abnormal shapes) and **anisocytosis** (variation in red blood cell sizes), which are common findings in iron deficiency [1]. - Typically results in **microcytic hypochromic anemia** [1], distinguishing it from other types of anemia. *Nutritional deficiency anaemia* - May present with various blood cell morphology but does not specifically exhibit **poikilocytosis** and **anisocytosis** characteristic of iron deficiency anemia. - Usually includes deficiencies like **vitamin B12** or **folate**, which result in **macrocytic anemia** instead. *Megaloblastic anaemia* - Primarily caused by deficiency of **vitamin B12** or **folate**, leading to large, immature red blood cells (megaloblasts) rather than varied shapes and sizes. - Associated with **hypersegmented neutrophils** in the blood smear, which differentiates it from iron deficiency anemia. *Thalassemia* - Characterized by **microcytic hypochromic red blood cells** and often involves **target cells** rather than generalized poikilocytosis and anisocytosis. - Typically presents with **hemolytic anemia** but does not show the same variability in cell shapes and sizes as seen in iron deficiency anemia. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Q: Which of the following is typically expected in a case of Microcytic Hypochromic anemia?

Reduced serum Iron. ***Reduced total RBC distribution width*** - Microcytic hypochromic anemia typically shows **increased RBC distribution width (RDW)** due to varied sizes of red blood cells, indicating a diverse population in response to microcytic anemia [1]. - A **reduced RDW** would suggest a more uniform red blood cell population, which is not characteristic of this type of anemia. *Normal Ferritin levels* - Ferritin levels are usually **low** in microcytic hypochromic anemia, indicating **iron deficiency** [1]. - Normal ferritin might suggest other types of anemia rather than the iron deficiency expected in microcytic anemia. *Reduced serum Iron* - Serum iron is generally **low** in microcytic hypochromic anemia due to **iron deficiency**, making this an expected finding [1]. - The presence of reduced serum iron supports the diagnosis and is not inconsistent with this type of anemia. *Increased TIBC* - TIBC (Total Iron Binding Capacity) is typically **increased** in microcytic anemia due to low iron stores, reflecting the body's attempt to maximize iron uptake. - An increased TIBC aligns with iron deficiency anemia, which is a common cause of microcytic hypochromic anemia [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Q: Which tumor is characterized by the presence of cells that resemble tennis rackets?

Langerhans cell histiocytosis. ***Langerhans cell histiocytosis*** - This condition is characterized by **Birbeck granules** within the Langerhans cells, which have a distinctive **tennis racket-like appearance** on electron microscopy. - The cells are also positive for **CD1a** and **S-100** immunohistochemical markers. *Sarcoma botyroides* - This is a subtype of **embryonal rhabdomyosarcoma** typically found in mucosal sites, presenting as a polypoid, grape-like mass. - Its histology shows small, undifferentiated cells with primitive skeletal muscle differentiation, but **not tennis racket-shaped cells**. *Vaginal adenocarcinoma* - This is a rare malignancy of the vagina, often associated with **diethylstilbestrol (DES) exposure** in utero, presenting as glands. - The cells show glandular differentiation and produce mucin, but **lack Birbeck granules**. *Seminoma* - This is a germ cell tumor of the testis characterized by large, uniform cells with clear cytoplasm and prominent nucleoli, divided into lobules by fibrous septa containing lymphocytes. - There are **no tennis racket-shaped cells** or Birbeck granules associated with seminoma.

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.

Q: Shelf life of platelets in a blood bank is

5 days. **5 days** - Platelets stored at **room temperature (20-24°C)** have a limited shelf life due to the risk of bacterial contamination and metabolic changes. - This short storage period ensures the **viability and function** of platelets for transfusion. *7 days* - A 7-day shelf life was initially proposed but was not widely adopted due to concerns about increased **bacterial growth** and the practical challenges of extended storage at room temperature. - The risk of **bacterial sepsis** significantly increases with longer room temperature storage. *10 days* - This duration is beyond the currently accepted shelf life for platelets, leading to an unacceptably high risk of **bacterial contamination** and reduced therapeutic efficacy. - Storing platelets for 10 days would likely result in an increased incidence of **transfusion-associated sepsis**. *21 days* - A shelf life of 21 days is typical for **red blood cells** when stored at 1-6°C with specific anticoagulants, but it is far too long for platelets. - Platelets stored for this duration at room temperature would be significantly **non-viable** and pose a severe risk of bacterial infection.

Q: What is a distinguishing feature of reticulocytes?

Presence of residual RNA and ribosomes. ***Presence of residual RNA and ribosomes*** - This is the **defining and most distinguishing feature** of reticulocytes that differentiates them from mature red blood cells. - Reticulocytes contain residual **ribosomal RNA** and other organelles that are lost when they mature into erythrocytes. - This residual RNA forms a **reticular (network-like) pattern** when stained with supravital stains like **new methylene blue** or **brilliant cresyl blue**, which is the basis for their name and identification. - The presence of RNA allows for **reticulocyte counting**, an important marker of bone marrow erythropoietic activity. *Slightly larger in size than RBCs* - While reticulocytes may be slightly larger (polychromatophilic appearance), size variation is **not specific** and overlaps significantly with mature RBCs. - Size is not a reliable distinguishing feature and is not used for identification or counting. *Mature in bone marrow* - Reticulocytes are **released from the bone marrow** as immature red cells and complete their maturation in the **peripheral circulation** over 24-48 hours. - They do not fully mature in the bone marrow; their presence in peripheral blood is normal. *Constitute approximately 1% of the red cells* - Normal reticulocyte count is **0.5-2%** (or approximately 1%) of total red blood cells in healthy adults. - This is a **population characteristic** indicating normal erythropoietic activity, not a distinguishing cellular feature.

Question 1

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 2

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 3

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 4

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 5

Which type of anemia is most commonly characterized by marked poikilocytosis and anisocytosis on peripheral blood smear?

Accepted Answer

Iron deficiency anemia

Answer

Megaloblastic anemia

Answer

Nutritional anemia

Answer

Thalassemia

Question 6

Which of the following is typically expected in a case of Microcytic Hypochromic anemia?

Accepted Answer

Reduced serum Iron

Answer

Normal Ferritin levels

Answer

Increased TIBC

Answer

Increased total RBC distribution width

Question 7

Which tumor is characterized by the presence of cells that resemble tennis rackets?

Accepted Answer

Langerhans cell histiocytosis

Answer

Seminoma

Answer

Vaginal adenocarcinoma

Answer

Sarcoma botyroides

Question 8

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

Question 9

Shelf life of platelets in a blood bank is

Accepted Answer

5 days

Answer

7 days

Answer

10 days

Answer

21 days

Question 10

What is a distinguishing feature of reticulocytes?

Accepted Answer

Presence of residual RNA and ribosomes

Answer

Slightly larger in size than RBCs

Answer

Mature in bone marrow

Answer

Constitute approximately 1% of the red cells

Hematopathology — MCQs

Hematopathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?