Hematopathology Practice Questions

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: Which of the following myeloid leukemias is not classified as a myeloproliferative neoplasm?

Acute myeloid leukemia. ***Acute myeloid leukemia*** - Acute myeloid leukemia is classified as a **malignant hematological disorder**, not a myeloproliferative disease, which includes chronic diseases with increased blood cell production. - It is characterized by the **rapid accumulation of immature blood cells**, leading to acute symptoms rather than a gradual proliferation. *Polycythemia vera* - Polycythemia vera is a myeloproliferative neoplasm resulting in an **increase in red blood cells**, causing symptoms like increased blood viscosity [1][2]. - It is associated with a mutation in the **JAK2 gene**, which leads to overproduction of erythrocytes [1][2]. *Chronic myeloid leukemia* - Chronic myeloid leukemia is a myeloproliferative disorder characterized by the proliferation of myeloid cells and often involves the **Philadelphia chromosome** [2][3]. - It shows a gradual increase in cell number and is typically diagnosed based on the presence of **elevated white blood cell counts**. *Essential thrombocytosis* - Essential thrombocytosis is a myeloproliferative neoplasm characterized by an **elevated platelet count**, increasing the risk of thrombotic events [1][2]. - Like other myeloproliferative disorders, it is associated with mutations in **JAK2** or other myeloid-related genes [1][2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615. [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. 624. [3] 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. 624-625.

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: Anemia with reticulocytosis is seen in -

Hemolysis. ***Hemolysis*** - Reticulocytosis indicates a compensatory response to anemia, often occurring in hemolytic processes where the **bone marrow increases red blood cell production** in response to red blood cell destruction. - Conditions like **sickle cell disease** or **autoimmune hemolytic anemia** lead to hemolysis, further confirming increased reticulocyte count. *Iron deficiency anemia* - Typically presents with a **low reticulocyte count** as the bone marrow does not have sufficient iron to produce new red blood cells. - This condition is characterized by **microcytic, hypochromic** red blood cells due to inadequate iron stores. *Vitamin B12 deficiency* - Often results in a **macrocytic anemia** with a variable reticulocyte count; however, reticulocytosis is generally not seen initially. - This deficiency affects DNA synthesis, leading to ineffective erythropoiesis and the presence of **megaloblastic changes**. *Aplastic anemia* - Characterized by a **decrease in all types of blood cells** (pancytopenia) and typically has a **low reticulocyte count** due to bone marrow failure. - There is insufficient production of red blood cells, hence **reticulocytosis is not observed**.

Q: Which is not a feature of paroxysmal nocturnal hemoglobinuria?

Increased LAP score. ***Increased LAP score*** - In paroxysmal nocturnal hemoglobinuria, the **LAP score** is typically **low** due to ineffective hematopoiesis and not elevated. - The presence of a low LAP score is inconsistent with the features of this condition, making it the correct choice. *Thrombosis* - Paroxysmal nocturnal hemoglobinuria is **associated with a high risk of thrombosis**, particularly in the **venous system** [2]. - This is due to **increased platelet activation** and excessive thrombin generation resulting from hemolysis. *Hemolysis* - **Hemolysis** is a hallmark feature of paroxysmal nocturnal hemoglobinuria, where there is **destruction of red blood cells** [2,3]. - Patients often present with signs of hemolytic anemia including **elevated bilirubin** and **low haptoglobin** levels. *Thrombocytopenia* - **Thrombocytopenia** is a common finding in paroxysmal nocturnal hemoglobinuria due to **expanded consumption** of platelets during episodes of hemolysis. - This can lead to an **increased risk of bleeding** in affected patients. **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] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 650-651.

Q: In which condition are Pseudo-Pelger-Huët cells typically seen?

Myelodysplastic syndrome. ***Mylodysplastic syndrome*** - Pseudo-Pelger-Huet cells are characteristic and often observed in myelodysplastic syndromes, indicating an ineffective hematopoiesis [1]. - These cells appear as **hyposegmented neutrophils** and are associated with dysplastic changes in the bone marrow [1]. *Hairy cell leukemia* - Typically presents with **hairy cells** in peripheral blood and often involves splenomegaly; pseudo-Pelger-Huet cells are not usual in this condition. - Associated with **PANCYTOPENIA** and reticulin fibrosis, differing from myelodysplastic syndrome. *Hodgkin's lymphoma* - Characterized by the presence of **Reed-Sternberg cells** and typically involves lymphadenopathy. - Peripheral blood findings generally do not include pseudo-Pelger-Huet cells; the focus is on lymphatic tissue. *Multiple myeloma* - Commonly presents with **plasma cells** and related symptoms like bone pain and renal failure, not associated with pseudo-Pelger-Huet cells. - It primarily causes an increase in monoclonal proteins rather than dysplastic changes seen in myelodysplastic syndrome. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 613-614.

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

Increased 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: Which of the following statements is false regarding hereditary spherocytosis?

Decreased MCHC. ***Decreased MCHC*** - Hereditary spherocytosis typically presents with an **increased MCHC** due to the spherocytes being more concentrated. - MCHC is a measure of the hemoglobin concentration in red blood cells, and in spherocytosis, this value is often elevated rather than decreased. *Defect in ankyrin* - This is a true statement; hereditary spherocytosis is associated with a defect in **ankyrin**, a protein that helps maintain the cell's membrane structure [2]. - Mutations in ankyrin lead to instability of the red blood cell membrane, resulting in spherocyte formation [2]. *Decreased MCV* - In hereditary spherocytosis, MCV is often **normal or slightly increased**, as it reflects the volume of red blood cells, which can be misinterpreted due to the presence of spherocytes. - Spherocytes are smaller cells, which can mistakenly suggest a falsely decreased MCV if not properly interpreted [1]. *Reticulocytosis* - This condition typically presents with **reticulocytosis** as a response to hemolysis, indicating the bone marrow is producing more red blood cells to compensate [1]. - The presence of reticulocytosis is a common finding in hereditary spherocytosis due to increased destruction of spherocytes. **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.

Question 1

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 2

Which of the following myeloid leukemias is not classified as a myeloproliferative neoplasm?

Accepted Answer

Acute myeloid leukemia

Answer

Chronic myeloid leukemia

Answer

Chronic lymphocytic leukemia

Answer

Acute lymphoblastic leukemia

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

Anemia with reticulocytosis is seen in -

Accepted Answer

Hemolysis

Answer

Iron deficiency anemia

Answer

Vitamin B12 deficiency

Answer

Aplastic anemia

Question 6

Which is not a feature of paroxysmal nocturnal hemoglobinuria?

Accepted Answer

Increased LAP score

Answer

Thrombocytopenia

Answer

Hemolysis

Answer

Thrombosis

Question 7

In which condition are Pseudo-Pelger-Huët cells typically seen?

Accepted Answer

Myelodysplastic syndrome

Answer

Hairy cell leukemia

Answer

Multiple myeloma

Answer

Hodgkin's lymphoma

Question 8

Which of the following statements about Polycythemia vera is false?

Accepted Answer

Increased LAP score

Answer

Increased vitamin B12 levels

Answer

Leukocytosis is present

Answer

Increased platelet count

Question 9

Persons with heterozygous sickle cell trait are protected from infection by:

Accepted Answer

P. falciparum

Answer

Pneumococcus

Answer

P. vivax

Answer

Salmonella

Question 10

Which of the following statements is false regarding hereditary spherocytosis?

Accepted Answer

Decreased MCHC

Answer

Defect in ankyrin

Answer

Reticulocytosis

Answer

Normal to increased MCV

Hematopathology — MCQs

Hematopathology — MCQs

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