Hematopathology Practice Questions

Q: In which of the following conditions are Gamma-Gandy bodies typically not seen?

Chronic myeloid leukemia. ***Chronic myeloid leukemia*** - **Gamma-Gandy bodies** are fibrotic nodules in the spleen with **hemosiderin** and **calcium deposits**, typically resulting from **chronic passive congestion** or **repeated hemorrhages**. - **CML** causes massive **splenomegaly** due to **infiltration by leukemic cells** and extramedullary hematopoiesis, not the chronic vascular congestion or infarction pattern that produces Gamma-Gandy bodies. - While CML involves increased splenic iron turnover, it does NOT typically form the organized fibrotic nodules characteristic of Gamma-Gandy bodies. *Cirrhosis with portal hypertension* - **Portal hypertension** leads to **chronic passive congestion** of the spleen and splenomegaly. - This chronic congestion with elevated venous pressure and repeated microhemorrhages creates the ideal environment for **Gamma-Gandy body formation**. - This is one of the **classic associations** with Gamma-Gandy bodies. *Hemosiderosis* - While hemosiderosis involves **diffuse hemosiderin deposition** in tissues including the spleen, it represents generalized iron overload without the specific pathologic features of Gamma-Gandy bodies. - The organized **fibrotic nodules with calcium** that characterize Gamma-Gandy bodies are not a primary feature of hemosiderosis. - However, some sources consider hemosiderosis can be associated with these lesions in the context of chronic congestion. *Sickle Cell anemia* - **Repeated splenic infarctions** from vaso-occlusive crises lead to chronic injury, fibrosis, and hemosiderin deposition. - This creates the pathologic substrate for **Gamma-Gandy body formation**. - These bodies reflect chronic splenic damage in sickle cell disease, especially before autosplenectomy occurs.

Q: A 12-month-old girl of Punjabi parents developed pallor since 3 months of age. One unit of blood transfusion was done at 5 months of age. She now presents with pallor and hepatosplenomegaly. Her hemoglobin level is 3.8 g/dL, MCV is 68, and MCH is 19. A peripheral smear examination showed schistocytes, and bone marrow examination revealed erythroid hyperplasia. What is the diagnosis?

Beta-thalassemia (major). ***Beta-thalassemia (major)*** - The combination of **severe microcytic hypochromic anemia** (Hb 3.8 g/dL, MCV 68, MCH 19), early onset of pallor, **hepatosplenomegaly**, and **erythroid hyperplasia** with a need for transfusions is highly characteristic of **beta-thalassemia major** [1]. - The presence of **schistocytes** on peripheral smear indicates significant ineffective erythropoiesis and hemolysis, which is common in severe thalassemias due to the precipitation of unstable globin chains [1]. *Sickle cell disease* - This condition is characterized by **sickle-shaped red blood cells** and recurrent painful vaso-occlusive crises, which are not mentioned in the presentation, and the peripheral smear showed schistocytes, not sickled cells [1]. - While it can cause anemia, the **MCV is typically normal to high**, unlike the microcytosis seen here. *Alpha-thalassemia* - Severe forms like **Hb Barts hydrops fetalis** usually present *in utero* or at birth with massive edema and profound anemia, and are often fatal [1]. - Less severe forms might cause microcytic anemia, but **hepatosplenomegaly and severe transfusion dependence from 3 months of age** are more typical of beta-thalassemia major [1]. *Glucose-6-phosphate dehydrogenase deficiency* - This condition typically presents with **acute hemolytic anemia** triggered by certain drugs, infections, or fava beans, rather than chronic severe anemia and hepatosplenomegaly from early infancy. - Peripheral smear would show **bite cells** and **Heinz bodies** during a hemolytic episode, not schistocytes as the primary finding. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 644-650.

Q: What is the most common hematological response to bacterial infections?

Neutrophilia. Neutrophilia - **Neutrophilia** is a hallmark of acute bacterial infections, as the body ramps up production and release of **neutrophils** to combat the invading pathogens [1], [2]. - This increase in **neutrophils** is part of the innate immune response, directly targeting and phagocytosing bacteria [1]. *Neutropenia* - **Neutropenia** (a low neutrophil count) is less common in typical bacterial infections and is often associated with severe, overwhelming infections or conditions like **sepsis**, where neutrophil consumption exceeds bone marrow production. - It can also be seen in certain viral infections, bone marrow disorders, or as a side effect of some medications. *Eosinophilia* - **Eosinophilia** (an elevated eosinophil count) is primarily associated with **allergic reactions**, **parasitic infections**, and certain **drug reactions** or dermatological conditions [2]. - It does not typically occur in response to bacterial infections. *Lymphocytosis* - **Lymphocytosis** (an elevated lymphocyte count) is most commonly seen in **viral infections**, such as **infectious mononucleosis**, or in chronic bacterial infections like **tuberculosis**. - It is not the most common hematological response to acute bacterial infections.

Q: Which of the following statements about Transfusion-Related Acute Lung Injury (TRALI) is false?

Anti-HLA antibodies are never implicated in TRALI.. ***Anti-HLA antibodies are never implicated in TRALI*** - This statement is **false**, as **anti-HLA antibodies**, specifically anti-HLA class I and II antibodies, are **frequently implicated** in the pathogenesis of TRALI [1]. - These antibodies in donor plasma can react with recipient neutrophils, leading to their activation and subsequent lung injury [1]. *Leucocytes aggregate in the pulmonary vasculature* - This statement is **true**; the aggregation of **activated neutrophils** and other leukocytes in the pulmonary capillaries is a key pathophysiological event in TRALI. - This aggregation contributes to the inflammatory cascade and damage to the **alveolar-capillary membrane**. *Develops signs of non cardiogenic pulmonary edema* - This statement is **true**; TRALI is characterized by **acute respiratory distress** and evidence of **non-cardiogenic pulmonary edema**. - This means the pulmonary edema is not due to elevated left atrial pressure, but rather increased vascular permeability caused by inflammatory mediators. *Implicated donors are frequently multiparous women* - This statement is **true**; **multiparous women** are often implicated as donors in TRALI cases because they are more likely to have developed **anti-HLA antibodies** due to exposure to fetal antigens during pregnancy. - These antibodies, if present in high titers in donor plasma, can trigger TRALI in susceptible recipients.

Q: Which of the following red cell disorders is characterized by the presence of macrocytes in the peripheral smear?

Megaloblastic anemia. ***Megaloblastic anemia*** - The presence of **macropolycytes** (larger than normal red blood cells) is a hallmark of megaloblastic anemia, commonly due to vitamin B12 or folate deficiency [1]. - This condition leads to **impaired DNA synthesis**, resulting in ineffective erythropoiesis and abnormal cell morphology [1]. *Hereditary spherocytosis* - Characterized by the presence of **spherocytes** which are small, round, and lack central pallor, not macropolycytes. - It is associated with **hemolytic anemia** but does not show the abnormal cell size seen in megaloblastic anemia. *Iron deficiency anemia* - Typically shows **microcytic, hypochromic red blood cells**, not enlarged cells like macropolycytes. - This disorder results from insufficient iron, leading to inadequate hemoglobin synthesis rather than abnormal cell size. *Sickle cell anemia* - Characterized by **sickle-shaped cells** due to hemoglobin S polymerization; it does not feature macropolycytes. - The primary issue is the **deformation of red blood cells**, leading to vaso-occlusive crises rather than increased cell size. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 593-594.

Q: Pappenheimer bodies are composed of:

Iron. ***Iron*** - **Pappenheimer bodies** are composed of **iron granules** that have not been incorporated into hemoglobin. - They appear as small, irregular, basophilic inclusions within red blood cells on a **Wright-Giemsa stain**. *Copper* - **Copper** is an essential trace element but does not form these specific erythrocyte inclusions. - Its presence in excess can lead to conditions like **Wilson's disease**, affecting organs like the liver and brain. *Lead* - **Lead poisoning** can cause various red blood cell abnormalities, notably **basophilic stippling**, which represents aggregated ribosomes and not iron. - It interferes with heme synthesis, leading to **anemia**. *Zinc* - **Zinc** is a critical component of many enzymes, but it does not form the characteristic inclusions known as Pappenheimer bodies. - **Zinc deficiency** can impact immune function and growth, while excess can interfere with copper absorption.

Q: The characteristic chromosomal translocation in acute promyelocytic leukemia is:

t(15;17). ***+ (15;17)*** - The **translocation t(15;17)** is a hallmark of **acute promyelocytic leukemia (APL)**, leading to the formation of the **PML**-**RARA** fusion gene [1,2]. - This genetic anomaly is associated with **clinical features** such as **promyelocyte predominance** and can lead to coagulopathy [1]. *+ (22;9)* - This translocation is not associated with APL; instead, it is indicative of **chronic myelogenous leukemia (CML)**. - In CML, the **Philadelphia chromosome (BCR-ABL)** fusion is the primary genetic abnormality, leading to different clinical presentations. *+ (21;17)* - This specific translocation does not typically occur in APL; it is generally found in other types of **hematological malignancies**. - APL is specifically linked with the **t(15;17)** translocation, which distinctly separates its genetic features [1]. *+ (9;22)* - The **9;22** translocation is primarily found in **CML** and results in the **BCR-ABL** fusion gene, not APL. - APL is characterized by its unique translocation involving chromosomes 15 and 17, emphasizing the importance of genetic identification for accurate diagnosis [1]. **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. 620-622. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 326.

Q: The coagulation profile in a 13-year-old girl with menorrhagia having von Willebrand's disease is

Isolated prolonged PTT with a normal PT. ***Isolated prolonged PTT with a normal PT*** - von Willebrand Factor (vWF) is a carrier protein for **Factor VIII**, protecting it from degradation. - Since factor VIII is part of the **intrinsic coagulation pathway**, its deficiency or malfunction (as seen in von Willebrand's disease) leads to a **prolonged PTT** without affecting the extrinsic pathway (PT). *Isolated prolonged PT with a normal PTT* - This pattern suggests a defect in the **extrinsic coagulation pathway**, which is not primarily affected in von Willebrand's disease. - Conditions like **Factor VII deficiency** or certain stages of liver disease would cause an isolated prolonged PT. *Prolongation of both PT and PTT* - This indicates a defect in the **common coagulation pathway** or severe deficiencies affecting both intrinsic and extrinsic pathways. - Examples include deficiencies of **Factor X, V, II (prothrombin)**, or fibrinogen, or severe liver failure. *Prolongation of thrombin time* - A prolonged **thrombin time** points to an issue with **fibrinogen** concentration or function, or the presence of thrombin inhibitors like heparin. - This is not a primary finding in von Willebrand's disease, which primarily impacts platelet adhesion and factor VIII.

Question 1

In which of the following conditions are Gamma-Gandy bodies typically not seen?

Accepted Answer

Chronic myeloid leukemia

Answer

Cirrhosis with portal hypertension

Answer

Hemosiderosis

Answer

Sickle Cell anemia

Question 2

A 52-year-old man presents with back pain, fatigue, polyuria, and polydipsia. Imaging reveals lytic lesions in the lumbar vertebrae. Laboratory tests show hypoalbuminemia, mild anemia, and thrombocytopenia. Serum electrophoresis indicates a monoclonal immunoglobulin peak, and bone marrow aspiration reveals atypical plasma cells. Urinalysis shows significant proteinuria. What amyloid precursor protein is most likely to be deposited in this patient?

Accepted Answer

Immunoglobulin light chain protein

Answer

Amylin protein

Answer

Apolipoprotein serum amyloid A

Answer

Fibrinogen protein

Question 3

A 12-month-old girl of Punjabi parents developed pallor since 3 months of age. One unit of blood transfusion was done at 5 months of age. She now presents with pallor and hepatosplenomegaly. Her hemoglobin level is 3.8 g/dL, MCV is 68, and MCH is 19. A peripheral smear examination showed schistocytes, and bone marrow examination revealed erythroid hyperplasia. What is the diagnosis?

Accepted Answer

Beta-thalassemia (major)

Answer

Alpha-thalassemia

Answer

Sickle cell disease

Answer

Glucose-6-phosphate dehydrogenase deficiency

Question 4

What is the most common hematological response to bacterial infections?

Accepted Answer

Neutrophilia

Answer

Neutropenia

Answer

Eosinophilia

Answer

Lymphocytosis

Question 5

Which of the following statements about Transfusion-Related Acute Lung Injury (TRALI) is false?

Accepted Answer

Anti-HLA antibodies are never implicated in TRALI.

Answer

Leucocytes aggregate in the pulmonary vasculature

Answer

Develops signs of non cardiogenic pulmonary edema

Answer

Implicated donors are frequently multiparous women

Question 6

Von Willebrand disease involves a deficiency of which factor?

Accepted Answer

Defect in platelet adhesion due to von Willebrand factor deficiency

Answer

Defect in fibrin formation affecting clot stabilization

Answer

Generalized defects involving small blood vessels

Answer

Defect in clotting factors affecting secondary hemostasis

Question 7

Which of the following red cell disorders is characterized by the presence of macrocytes in the peripheral smear?

Accepted Answer

Megaloblastic anemia

Answer

Hereditary spherocytosis

Answer

Iron deficiency anemia

Answer

Sickle cell anemia

Question 8

Pappenheimer bodies are composed of:

Accepted Answer

Iron

Answer

Copper

Answer

Lead

Answer

Zinc

Question 9

The characteristic chromosomal translocation in acute promyelocytic leukemia is:

Accepted Answer

t(15;17)

Answer

t(21;17)

Answer

t(9;22)

Answer

t(8;21)

Question 10

The coagulation profile in a 13-year-old girl with menorrhagia having von Willebrand's disease is

Accepted Answer

Isolated prolonged PTT with a normal PT

Answer

Isolated prolonged PT with a normal PTT

Answer

Prolongation of both PT and PTT

Answer

Prolongation of thrombin time

Hematopathology — MCQs

Hematopathology — MCQs

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