Hematopathology Practice Questions

Q: What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?

Morphology of cells. ***Morphology of cells*** - The **Working Formulation** primarily classified non-Hodgkin's lymphomas based on the **histological appearance** of the malignant cells, such as cell size, nuclear features, and growth patterns. - This classification aimed to group lymphomas with similar prognoses, broadly categorizing them into low, intermediate, and high-grade based on their **cytological features**. *Cell surface markers* - While cell surface markers (immunophenotyping) are crucial in modern lymphoma classification (e.g., WHO classification), they were not the **primary basis** for the Working Formulation. - Immunophenotyping identifies the lineage and differentiation stage of lymphoid cells (e.g., B-cell, T-cell) but became widely integrated into lymphoma classification later. *Survival characteristic of cells* - The Working Formulation did indirectly consider survival by grouping lymphomas with similar prognoses, but **survival characteristics** themselves were not the primary *basis* for classifying each specific lymphoma type. - Prognosis was an outcome derived from the morphological classification, not the initial classifying factor. *Cellular genetics* - **Cellular genetics**, including chromosomal translocations and gene mutations, are fundamental to current World Health Organization (WHO) classifications of lymphoma. - However, comprehensive genetic analysis was not readily available or the primary method for classifying lymphomas when the Working Formulation was developed.

Q: Auer rods are a characteristic feature of which type of leukemia?

Acute myeloid leukemia (AML). ***Auer rods*** - Auer rods are **needle-shaped cytoplasmic inclusions** found in myeloid leukemias, particularly in acute myeloid leukemia (AML) [1]. - They are indicative of myeloid differentiation and are a classic **diagnostic feature** observed in bone marrow or peripheral blood smears [1]. *Intercytoplasmic granules* - While **intercytoplasmic granules** may appear in various leukemias, they are not specific or characteristic for Auer rods. - These granules do not serve as a **specific diagnostic marker** for any particular type of leukemia. *Eosinophils* - Eosinophils are associated with **allergic reactions** and parasitic infections, majorly presenting with **bilobed nuclei** and prominent granules. - They do not have **Auer rods**, making this option incorrect regarding the context of leukemia. *Leukemic cells* - While leukemic cells represent neoplastic white blood cells, they do not specifically refer to the **presence of Auer rods**. - Different types of leukemic cells have various **morphologies** and features that may not include Auer rods. **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. 620.

Q: Which of the following is a potential complication of blood transfusion?

Hyperkalemia. ***Hyperkalemia*** - Stored red blood cells can release **intracellular potassium** into the storage solution, leading to elevated potassium levels in transfused blood. - Rapid or massive transfusions deliver a significant potassium load, potentially causing **cardiac arrhythmias**. *Hyponatremia* - This is generally not a direct complication of blood transfusions; instead, blood products themselves contain electrolytes, and massive transfusion can disrupt electrolyte balance, but usually not to cause hyponatremia. - Volume overload from transfusion could dilute existing electrolytes, but most commonly, other electrolyte imbalances such as hyperkalemia or hypocalcemia occur. *Hypercalcemia* - Often, the opposite, **hypocalcemia**, is a complication of massive transfusion due to **citrate toxicity**. - Citrate, an anticoagulant in transfused blood, chelates calcium, reducing free calcium levels in the recipient. *Increased serum albumin* - Blood transfusions primarily administer **red blood cells**, plasma, or platelets, not typically albumin in amounts that would significantly increase serum albumin levels in the absence of an albumin-specific infusion [1]. - Low albumin (hypoalbuminemia) is a common finding in critically ill patients, and a blood transfusion usually doesn't correct this unless plasma or albumin is specifically administered [1].

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: About Langerhans cell histiocytosis, all are true EXCEPT:

Gonadal involvement occurs. ***Gonadal involvement occurs*** - **Gonadal involvement is not characteristic** of Langerhans cell histiocytosis, which primarily affects bones, skin, and organs like the liver and lungs. - The condition typically presents with lesions in areas such as the skull, vertebrae, and lungs, rather than the gonads. *Diffuse form is known as litter-sewe-disease* - The diffuse form of Langerhans cell histiocytosis is indeed known as **Letterer-Siwe disease**, but this option is misleading as it uses an incorrect term. - Letterer-Siwe disease is a severe variant occurring in infants, presenting with systemic symptoms, which is distinct from gonadal involvement. *Peak incidence less than 3 years of age* - Langerhans cell histiocytosis does peak in incidence in **young children**, typically under the age of 3, making this statement true for the condition. - Common manifestations include **bone pain**, skin lesions, and lymphadenopathy in this age group. *Radiosensitive* - This condition is not considered **radiosensitive**; it usually requires chemotherapy for treatment rather than radiation. - Treatment effectiveness does not primarily depend on radiation therapy, as the lesions are often resistant to radiation. [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, p. 630.

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: 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: 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.

Question 1

What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?

Accepted Answer

Morphology of cells

Answer

Cell surface markers

Answer

Survival characteristic of cells

Answer

Cellular genetics

Question 2

Auer rods are a characteristic feature of which type of leukemia?

Accepted Answer

Acute myeloid leukemia (AML)

Answer

Chronic lymphocytic leukemia (CLL)

Answer

Myelodysplastic syndromes (MDS)

Answer

Acute lymphoblastic leukemia (ALL)

Question 3

Which of the following conditions is least likely to be associated with a high reticulocyte count?

Accepted Answer

Megaloblastic anemia (due to vitamin B12 or folate deficiency)

Answer

Acute bleed

Answer

Hemolytic anemia

Answer

Nutritional deficiency anemia under treatment

Question 4

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 5

Which of the following is a potential complication of blood transfusion?

Accepted Answer

Hyperkalemia

Answer

Hyponatremia

Answer

Hypercalcemia

Answer

Increased serum albumin

Question 6

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 7

About Langerhans cell histiocytosis, all are true EXCEPT:

Accepted Answer

Gonadal involvement occurs

Answer

Peak incidence less than 3 years of age

Answer

Diffuse form is known as Letterer-Siwe disease

Answer

Radiosensitive

Question 8

What is the most common hematological response to bacterial infections?

Accepted Answer

Neutrophilia

Answer

Neutropenia

Answer

Eosinophilia

Answer

Lymphocytosis

Question 9

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 10

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

Hematopathology — MCQs

Hematopathology — MCQs

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