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: Which of the following statements about Polycythemia vera is false?

Decreased LAP score. ***Decrease LAP score*** - In polycythemia vera, the **LAP (leukocyte alkaline phosphatase) score** is typically increased, indicating more mature leukocytes. - A **decrease in LAP score** is not consistent with the disease, making this statement incorrect. *Increased platelets* - Polycythemia vera often results in **thrombocytosis**, characterized by increased platelet counts [1]. - This is a common feature of the disorder, reflecting overproduction of blood cells in the bone marrow. *Leucocytosis* - Patients with polycythemia vera frequently exhibit **leucocytosis**, or increased white blood cell counts, due to hypercellularity of the bone marrow [1]. - This is an important aspect of the disease, often seen alongside increases in red blood cells and platelets. *Increased vit B12* - An elevation in **vitamin B12** levels can occur in polycythemia vera, often due to increased binding proteins. - This is a well-recognized phenomenon associated with the increased cell turnover in this condition. **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. 626-627.

Q: MALT lymphoma is positive for which of the following markers?

CD20. ***CD20*** - MALT lymphoma is a type of **B-cell non-Hodgkin lymphoma**, and CD20 is a **pan B-cell marker consistently expressed** in MALT lymphomas. - CD20 positivity is **crucial for diagnosis** and is the **primary therapeutic target** for anti-CD20 monoclonal antibody therapy (Rituximab). - In diagnostic practice, **CD20 is the most important B-cell marker** for identifying MALT lymphoma and guiding treatment decisions. *CD19* - CD19 is also a **pan B-cell marker** and is **typically positive in MALT lymphoma** along with CD20. - However, in the context of this question, **CD20 is the preferred answer** because it is the **standard diagnostic marker emphasized in clinical practice** and the **primary therapeutic target**. - Both markers are positive, but CD20 has greater **clinical and therapeutic significance** in MALT lymphoma management. *CD43* - CD43 is primarily a **T-cell and myeloid marker**, but can show **aberrant expression in 40-50% of MALT lymphomas**. - While it may be positive in some cases, it is **not a defining B-cell lineage marker** and is not used as a primary diagnostic criterion for MALT lymphoma. - Its variable expression makes it **less reliable** than consistent B-cell markers like CD20. *CD5* - CD5 is typically associated with **T-cells** and certain B-cell lymphomas, particularly **chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)** and **mantle cell lymphoma**. - **MALT lymphoma is characteristically CD5-negative**, which is an important feature for **differentiating it from CD5+ B-cell lymphomas**.

Q: Thalassemia gives protection against ?

Protection against malaria. Protection against malaria - Individuals with thalassemia, particularly thalassemia trait, have some degree of protection against severe forms of malaria, specifically Plasmodium falciparum [1]. - The altered red blood cell structure and reduced hemoglobin content in thalassemia make the red blood cells less hospitable for the parasites, hindering their replication and survival [1]. Protection against filaria - Filaria is caused by parasitic worms (nematodes) transmitted by mosquitoes, leading to lymphatic filariasis (elephantiasis) or onchocerciasis (river blindness). - Thalassemia's primary impact is on red blood cell health and oxygen transport, offering no known protective effect against nematode infections or their associated pathology. Protection against kala-azar - Kala-azar (visceral leishmaniasis) is caused by Leishmania parasites transmitted by sandflies, primarily affecting the reticuloendothelial system (spleen, liver, bone marrow). - There is no established scientific evidence indicating that thalassemia provides protection against Leishmania infections or their clinical manifestations. Protection against leptospirosis - Leptospirosis is a bacterial infection caused by Leptospira bacteria, typically acquired through contact with contaminated water or animal urine. - Thalassemia is a genetic blood disorder; its physiological effects are unrelated to the mechanisms of infection or immunity against bacterial pathogens like Leptospira.

Q: Which of the following statements about sickle cell anemia is false?

Ringed sideroblasts are associated with sickle cell anemia.. ***Ringed sideroblast*** - **Ringed sideroblasts** are not typically associated with sickle cell anemia; they are indicative of disorders like **sideroblastic anemia**. - In sickle cell anemia, the primary findings include **hemolysis** and ineffective erythropoiesis, not ringed sideroblasts [3]. *Howell jolly bodies* - These bodies are remnants of nuclear material and can be found in individuals with **spleen dysfunction**, which can occur in sickle cell anemia [1]. - They are actually a common finding due to **hyposplenism** or **asplenia** in patients with sickle cell disease [2]. *Sickle cells* - The presence of **sickle-shaped red blood cells** is a hallmark of sickle cell anemia, caused by the mutation in the **beta-globin chain** [3]. - These sickle cells are responsible for the characteristic complications of the disease, such as **vaso-occlusive crises** [1][3]. *Target cells* - Target cells, or **codocytes**, are often seen in disorders like **thalassemia** and liver disease, and can also be present in sickle cell anemia. - They are formed due to an increase in the **surface area to volume ratio** of red blood cells, often secondary to **membrane abnormalities** seen in sickle cell changes [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. 644-646. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 570-571. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 598-599.

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: Localized Langerhans cell histiocytosis affecting head and neck is?

Eosinophilic granuloma. ***Eosinophilic granuloma*** - This is a localized form of **Langerhans cell histiocytosis** that typically presents in the head and neck region, often affecting areas like the skull and mandible [1]. - Characterized by **bone lesions** and may present with **pain or swelling** in the affected area, making it a prominent form in children and young adults. *Pulmonary langerhans cell histiocytosis* - Primarily affects the **lungs** and is associated with **cough, dyspnea**, and pulmonary nodules, not the head and neck region. - Occurs predominantly in **smokers** and involves interstitial lung disease patterns on imaging studies. *Hand-schuller-christian disease* - This condition is a systemic form of Langerhans cell histiocytosis that affects multiple systems rather than being localized, commonly presenting with **diabetes insipidus** and bone lesions. - It is often associated with **exophthalmos** and may involve lymphadenopathy, affecting older children and adults, not localized head and neck involvement. *Letterer-siwe disease* - This represents the acute, disseminated form of Langerhans cell histiocytosis, affecting infants, and is marked by systemic symptoms like **fever**, **rash**, and **hepatosplenomegaly** [1]. - Typically presents with serious manifestations and not specifically localized in the **head and neck area** as seen in eosinophilic granuloma. **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. 630.

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: 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 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].

Question 1

Donath-Landsteiner antibody is seen in?

Accepted Answer

Paroxysmal cold hemoglobinuria

Answer

PNH

Answer

Waldenstrom's macroglobulinemia

Answer

Malaria

Question 2

Which of the following statements about Polycythemia vera is false?

Accepted Answer

Decreased LAP score

Answer

Increased vitamin B12 levels

Answer

Leukocytosis is present

Answer

Increased platelet count

Question 3

MALT lymphoma is positive for which of the following markers?

Accepted Answer

CD20

Answer

CD19

Answer

CD43

Answer

CD5

Question 4

Thalassemia gives protection against ?

Accepted Answer

Protection against malaria

Answer

Protection against filaria

Answer

Protection against kala-azar

Answer

Protection against leptospirosis

Question 5

Which of the following statements about sickle cell anemia is false?

Accepted Answer

Ringed sideroblasts are associated with sickle cell anemia.

Answer

Sickle cells are present in sickle cell anemia.

Answer

Target cells are commonly seen in sickle cell anemia.

Answer

Howell Jolly bodies can be found in sickle cell anemia.

Question 6

Intracorpuscular hemolytic anemia is seen in ?

Accepted Answer

Thalassemia

Answer

Infection

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Autoimmune hemolytic anemia

Question 7

Localized Langerhans cell histiocytosis affecting head and neck is?

Accepted Answer

Eosinophilic granuloma

Answer

Letterer-siwe disease

Answer

Pulmonary Langerhans cell histiocytosis

Answer

Hand-Schuller-Christian disease

Question 8

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 9

The immunoglobulin most commonly involved in Multiple Myeloma is:

Accepted Answer

IgG

Answer

IgM

Answer

IgA

Answer

IgD

Question 10

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

Accepted Answer

PNH

Answer

Sickle cell anemia

Answer

Thalassemia

Answer

Duffy blood group

Hematopathology — MCQs

Hematopathology — MCQs

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