Hematopathology Practice Questions

Q: Donath-Landsteiner antibody is seen in?

Paroxysmal cold hemoglobinuria. ***Paroxysmal cold hemoglobinuria*** - **Donath-Landsteiner antibody** is a **biphasic IgG autoantibody** that binds to red blood cells in the cold and causes **hemolysis** upon warming, characteristic of paroxysmal cold hemoglobinuria. - This antibody has **anti-P specificity**, meaning it targets the P antigen on red blood cells, leading to complement activation and cell lysis. *PNH* - **Paroxysmal nocturnal hemoglobinuria** (PNH) is characterized by a deficiency in **GPI-anchored proteins** on red blood cells, notably **CD55** and **CD59**, making them susceptible to complement-mediated lysis. - It is not associated with the Donath-Landsteiner antibody; rather, it is identified by **flow cytometry** showing absence of CD55/CD59. *Waldenstrom's macroglobulinemia* - This is a **B-cell lymphoma** characterized by the overproduction of **monoclonal IgM antibodies**, leading to hyperviscosity syndrome and other symptoms. - It does not involve Donath-Landsteiner antibodies or cold-induced hemolysis in the same manner as paroxysmal cold hemoglobinuria. *Malaria* - **Malaria** is caused by **Plasmodium parasites** that infect and destroy red blood cells, leading to hemolytic anemia and fever. - While it causes **hemolysis**, it is not mediated by the Donath-Landsteiner antibody; the destruction is primarily due to parasitic replication and immune responses against infected cells.

Q: Intracorpuscular hemolytic anemia is seen in ?

Thalassemia. ***Thalassemia*** - Thalassemia is characterized by **intracorpuscular hemolysis** due to defective hemoglobin synthesis, leading to premature destruction of red blood cells [1][2]. - It manifests as **microcytic anemia** with associated **extramedullary erythropoiesis** in severe cases [1]. *Autoimmune hemolytic anemia* - This condition leads to **extravascular hemolysis**, primarily affecting red blood cells in the spleen, not within the plasma [2]. - It is often associated with **positive direct Coombs test**, indicating reactants on the RBC surface. *TIP* - TIP (Thrombotic Microangiopathy) primarily involves **microangiopathic hemolytic anemia** and is not classified as intracorpuscular [2]. - The hemolysis in TIP occurs due to **microthrombi**, causing damage to red blood cells as they pass through narrowed vessels. *Infection* - Infections can lead to **hemolysis**, but this is typically **extravascular** due to splenic clearance or due to other mechanisms like **malaria** [2]. - The hemolytic mechanism is not intracorpuscular, as seen in conditions like thalassemia. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 601-602. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 596-597.

Q: All of the following are features of juvenile CML except which of the following?

Philadelphia chromosome is positive. ***Philadelphia chromosome is positive*** - **Juvenile Chronic Myeloid Leukemia (JCML)**, now known as **Chronic Myelomonocytic Leukemia (CMML)** of childhood, is characterized by the **absence** of the **Philadelphia chromosome (Ph chromosome)**. - The Ph chromosome, a t(9;22)(q34;q11) translocation forming the **BCR-ABL1 fusion gene**, is the hallmark of adult Chronic Myeloid Leukemia (CML), but not JCML. *Thrombocytopenia* - **Thrombocytopenia** (low platelet count) is a common feature in JCML due to ineffective hematopoiesis and bone marrow infiltration. - This contrasts with adult CML, where **thrombocytosis** (high platelet count) is more characteristic of the chronic phase. *Fetal Hb is increased* - An **increased level of fetal hemoglobin (HbF)** is a characteristic laboratory finding in children with JCML. - This elevation is related to the dysregulated hematopoiesis and is a useful diagnostic marker. *Lymphadenopathy* - **Lymphadenopathy** (enlarged lymph nodes) is a frequent clinical manifestation in JCML, reflecting the widespread infiltration of monocytic cells. - This is part of the systemic involvement seen in this aggressive myeloproliferative disorder.

Q: Which of the following is a characteristic feature of Hereditary Spherocytosis?

Increased Osmotic Fragility. ***Direct Coomb's Positive*** - In hereditary spherocytosis, the **Coomb's test is typically negative**, indicating that the hemolysis is non-immune. - The condition is primarily due to defects in **red blood cell membrane proteins** [2], not autoimmune mechanisms. *Increased Osmotic Fragility* - A hallmark of hereditary spherocytosis; these red blood cells are more **sensitive to osmotic changes** due to their spherical shape [2]. - This fragility is used in clinical tests to help diagnose the condition [1]. *Gall stones* - Patients with hereditary spherocytosis may develop **pigment gallstones** due to chronic hemolysis and excess bilirubin [1]. - This is a common complication resulting from increased breakdown of red blood cells. *Splenomegaly* - Often presents in hereditary spherocytosis as the spleen works harder to clear **damaged red blood cells** [2]. - Splenomegaly is a common clinical feature due to the increased workload on the spleen. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598. [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.

Q: Which of the following conditions does not provide protection against malaria?

PNH. ***PNH*** - **Paroxysmal Nocturnal Hemoglobinuria (PNH)** does not provide protection against malaria; it is a disorder of the blood that leads to red blood cell destruction, but malaria specifically targets red blood cells. - PNH is associated with increased risk of venous thrombosis and hemolytic anemia, rather than any protective mechanism against malaria. *Sickle cell anemia* - The sickle cell trait provides a **protective advantage** against malaria by offering some resistance due to the abnormal shape of the cells [1]. - Individuals with the trait have a lower risk of severe malaria, particularly caused by *Plasmodium falciparum* [1]. *Duffy blood group* - People with the **Duffy negative phenotype** are resistant to certain strains of malaria, as the Duffy antigen serves as a receptor for *Plasmodium vivax*. - Therefore, those lacking the Duffy antigen are less susceptible to infections by this malaria strain. *Thalassemia* - Thalassemia provides a protective effect against malaria by producing **abnormal hemoglobin**, which malaria parasites cannot easily thrive in [2]. - This leads to an altered red blood cell environment that is less favorable for *Plasmodium* infection [1].

Q: In which condition is Hemoglobin F typically elevated?

Beta-thalassemia. ***Beta-thalassemia*** - In **beta-thalassemia**, there is reduced or absent synthesis of beta-globin chains, leading to an excess of alpha-globin chains. - To compensate, the body markedly increases production of fetal hemoglobin (**HbF**, α2γ2), which can constitute **50-90% of total hemoglobin** in beta-thalassemia major. - This compensatory mechanism helps alleviate the severity of anemia and ineffective erythropoiesis, making elevated HbF a **defining laboratory feature** of beta-thalassemia. *Hereditary spherocytosis* - This condition is characterized by defects in **erythrocyte membrane proteins** (spectrin, ankyrin, band 3), leading to fragile, spherical red blood cells. - While it causes **hemolytic anemia**, it does not involve elevated HbF levels; the defect is structural, not related to globin chain synthesis. *Congenital red cell aplasia* - This condition (e.g., **Diamond-Blackfan anemia**) involves a primary defect in early **erythroid progenitor cells**, leading to severe anemia. - While some patients may have elevated HbF, it is not a consistent or defining feature; the main problem is the lack of adequate red blood cell production, not a compensatory shift in globin synthesis. *Sickle cell disease* - In **sickle cell disease**, there is an abnormal beta-globin chain leading to the production of **hemoglobin S (HbS)**. - While HbF is indeed elevated in most patients (typically **2-15%**, compared to <1% in normal adults), the elevation is **less marked and consistent** compared to beta-thalassemia. - The primary defect is the presence of HbS, and while HbF elevation provides some clinical benefit (it inhibits HbS polymerization), beta-thalassemia remains the condition most characteristically associated with high HbF levels.

Q: The immunoglobulin most commonly involved in Multiple Myeloma is:

IgG. ***IgG*** - In Multiple Myeloma, the most commonly involved immunoglobulin is **IgG**, which is often produced in excess by malignant plasma cells [1][2]. - The presence of **monoclonal IgG** in serum is a key indicator of this malignancy, evident in diagnostic tests like serum protein electrophoresis. *IgM* - While **elevated IgM** levels can occur in other conditions like Waldenström's macroglobulinemia, it is not typically associated with Multiple Myeloma [2]. - IgM is produced by a different type of plasma cell and does not reflect the classic presentation of Multiple Myeloma. *IgA* - Although **IgA** can be involved in some cases of Multiple Myeloma, it is much less common than IgG [1][2]. - Patients with predominately **IgA Multiple Myeloma** are relatively rare compared to those with IgG. *IgD* - **IgD** myeloma is a very rare type of Multiple Myeloma, accounting for less than 2% of cases [1][2]. - It is not typically associated with the classic symptoms and conditions that characterize the more common IgG or IgA forms. **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. 608-609. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617.

Q: Which of the following is not typically screened for in blood donations?

Epstein-Barr Virus (EBV). ***Epstein-Barr Virus (EBV)*** ✓ - EBV is **NOT routinely screened** for in blood donations in India and most countries - While EBV is a common virus (>90% adults are seropositive), it is **not considered a major transfusion-transmitted infection** - The virus is primarily transmitted through saliva; transfusion-associated EBV transmission is **extremely rare and usually not clinically significant** in immunocompetent recipients - Risk-benefit analysis does not support routine screening due to **high prevalence, low clinical impact, and cost considerations** - EBV screening may only be considered for specific recipients (e.g., severely immunocompromised patients) *HIV* - **Routinely screened** in all blood donations worldwide - Screening includes HIV-1 and HIV-2 antibodies and/or HIV antigen/RNA testing - Transfusion-transmitted HIV causes AIDS with severe consequences - Mandatory screening under the Drugs and Cosmetics Act in India *HBV* - **Routinely screened** in all blood donations - Screening includes HBsAg (Hepatitis B surface antigen) testing, and often anti-HBc or HBV DNA - Can cause acute and chronic hepatitis, cirrhosis, and hepatocellular carcinoma - Mandatory screening in India and globally *HCV* - **Routinely screened** in all blood donations - Screening includes anti-HCV antibodies and/or HCV RNA (nucleic acid testing) - Major cause of chronic hepatitis, cirrhosis, and liver cancer - Mandatory screening under blood safety regulations

Q: All of the following provide protection against malaria except which of the following?

PNH. ***PNH*** - Paroxysmal Nocturnal Hemoglobinuria (PNH) does not provide any **protection against malaria**; it is an acquired bone marrow disorder. - While it leads to increased hemolysis and thrombosis, it does not affect **malaria susceptibility** directly. *Duffy blood group* - The Duffy blood group has specific antigens that are a **receptor for Plasmodium vivax**, making individuals with a Duffy-negative phenotype resistant to this type of malaria. - Therefore, Duffy blood group status can indeed serve as a protective factor against certain malaria strains. *Hereditary spherocytosis* - This condition results in **spherical red blood cells** [1], causing hemolytic anemia, but it does not confer protection against malaria. - People with hereditary spherocytosis do not have a lower prevalence of malaria and may still be susceptible to infection. *Sickle cell anemia* - Individuals with sickle cell anemia often exhibit **increased resistance to malaria**, especially against Plasmodium falciparum [2]. - The sickling of red blood cells under low oxygen conditions creates an unfavorable environment for the malarial parasites to thrive [2, 3].

Question 1

Donath-Landsteiner antibody is seen in?

Accepted Answer

Paroxysmal cold hemoglobinuria

Answer

PNH

Answer

Waldenstrom's macroglobulinemia

Answer

Malaria

Question 2

Intracorpuscular hemolytic anemia is seen in ?

Accepted Answer

Thalassemia

Answer

Infection

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Autoimmune hemolytic anemia

Question 3

All of the following are features of juvenile CML except which of the following?

Accepted Answer

Philadelphia chromosome is positive

Answer

Fetal Hb is increased

Answer

Lymphadenopathy

Answer

Thrombocytopenia

Question 4

Which of the following is a characteristic feature of Hereditary Spherocytosis?

Accepted Answer

Increased Osmotic Fragility

Answer

Splenomegaly

Answer

Gallstones

Answer

Direct Coombs Test Negative

Question 5

Which of the following conditions does not provide protection against malaria?

Accepted Answer

PNH

Answer

Sickle cell anemia

Answer

Thalassemia

Answer

Duffy blood group

Question 6

In which condition is Hemoglobin F typically elevated?

Accepted Answer

Beta-thalassemia

Answer

Congenital red cell aplasia

Answer

Hereditary spherocytosis

Answer

Sickle cell disease

Question 7

All the following are true about multiple myeloma except for which of the following?

Accepted Answer

Chromosomal translocation t(8;14), commonly seen in Burkitt's lymphoma

Answer

Hypercalcemia

Answer

Presence of Bence-Jones proteins in urine

Answer

Osteolytic bone disease

Question 8

The immunoglobulin most commonly involved in Multiple Myeloma is:

Accepted Answer

IgG

Answer

IgM

Answer

IgA

Answer

IgD

Question 9

Which of the following is not typically screened for in blood donations?

Accepted Answer

Epstein-Barr Virus (EBV)

Answer

HIV

Answer

HBV

Answer

HCV

Question 10

All of the following provide protection against malaria except which of the following?

Accepted Answer

PNH

Answer

Sickle cell anemia

Answer

Hereditary spherocytosis

Answer

Duffy blood group

Hematopathology — MCQs

Hematopathology — MCQs

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