Hematopathology Practice Questions

Q: 'Hairy cell leukemia' is a neoplastic proliferation of which type of cells?

B lymphocytes. ***B lymphocytes*** - **Hairy cell leukemia** is a rare, chronic lymphoproliferative disorder characterized by the clonal proliferation of **mature B lymphocytes** [1]. - These malignant B cells have distinctive cytoplasmic projections, giving them a "hairy" appearance under a microscope [1]. *T cells* - While T-cell leukemias exist (e.g., T-cell prolymphocytic leukemia), **hairy cell leukemia** specifically originates from B lymphocytes, not T cells. - T-cell neoplasms have different clinical presentations and immunophenotypes [2]. *Myeloid cells* - Myeloid cells give rise to conditions like **acute myeloid leukemia** or **chronic myeloid leukemia**. - These are distinct from lymphoid malignancies like **hairy cell leukemia**, which involves lymphocytes. *Macrophages* - Macrophages are phagocytic cells involved in immune responses and do not typically proliferate in a neoplastic manner to cause **leukemia**. - **Hairy cell leukemia** is a lymphoid malignancy, not a disorder of macrophages. **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. 612. [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, pp. 596-598.

Q: A 15-year-old female presented to the emergency department with a history of recurrent epistaxis, hematuria, and hematochezia. There was a history of profuse bleeding from the umbilical stump at birth. Previous investigations revealed normal prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen levels. Her platelet counts as well as platelet function tests were normal, but the urea clot lysis test was positive. Which clotting factor is most likely to be deficient?

Factor XIII. ***Factor XIII*** - A positive **urea clot lysis test** indicates deficiency of factor XIII, which is responsible for stabilizing the **fibrin clot**. - **Recurrent bleeding** from various sites, including the umbilical stump, with normal standard coagulation tests (PT, aPTT, TT, fibrinogen, platelet count, and function) is characteristic of factor XIII deficiency. *Factor X* - Deficiency of factor X would lead to abnormal **PT and aPTT**, as it is involved in both the intrinsic and extrinsic pathways, which is not the case here [1]. - Patients typically present with **severe bleeding manifestations**, and routine coagulation tests would be prolonged. *Factor XI* - Factor XI deficiency primarily causes an abnormally **prolonged aPTT**, as it is part of the intrinsic pathway, which is normal in this patient [1]. - Bleeding symptoms can be variable, but it does not specifically present with a **positive urea clot lysis test**. *Factor XII* - Factor XII deficiency causes a markedly prolonged **aPTT** [1] but is usually **asymptomatic** and not associated with significant bleeding. - It would not result in a **positive urea clot lysis test** or the severe bleeding symptoms described.

Q: Hemolytic anemia may be characterized by all of the following except which of the following?

Increased plasma haptoglobin level. ***Increased plasma haptoglobin level*** - **Haptoglobin** binds to free hemoglobin released during **hemolysis** and is then cleared from the circulation [1]. Therefore, in hemolytic anemia, the plasma haptoglobin level would be **decreased**, not increased. - An increased haptoglobin level typically indicates an **inflammatory response** or is observed in conditions without significant intravascular hemolysis. *Hyperbilirubinemia* - **Increased breakdown of red blood cells** releases large amounts of **heme**, which is metabolized into **unconjugated bilirubin** [2]. - This leads to an overload of the liver's capacity to conjugate bilirubin, resulting in **unconjugated hyperbilirubinemia** [2]. *Reticulocytosis* - The body responds to the loss of red blood cells through hemolysis by **increasing production of new red blood cells** in the bone marrow. - This increased production leads to a higher-than-normal percentage of immature red blood cells, known as **reticulocytes**, in the peripheral blood. *Hemoglobinuria* - When **intravascular hemolysis** occurs, free hemoglobin is released into the bloodstream and exceeds the binding capacity of haptoglobin [1]. - This excess unbound hemoglobin is filtered by the kidneys and excreted in the urine, leading to **dark-colored urine** due to hemoglobin.

Q: What is the estimated amount of iron delivered from the transfusion of 2 units of packed red cells?

500mg. ***500mg*** - Each unit of **packed red blood cells (PRBCs)** contains approximately **250 mg of iron**. - Therefore, two units would deliver approximately **500 mg of iron** to the recipient. *1g* - This amount would typically be derived from four units of PRBCs, which is double the number of units specified in the question. - This level of iron is usually associated with more significant transfusion burden, as seen in patients with chronic transfusions. *100mg* - This amount represents less than half the iron content of a single unit of PRBCs, making it an underestimation. - One unit of PRBCs contains approximately 200-250 mg of iron, so 100 mg is insufficient. *250mg* - This is the approximate iron content of a single unit of PRBCs, not the two units mentioned in the question. - The question specifically asks for the iron content from two units of PRBCs, requiring a cumulative calculation.

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: Neoplasm associated with Waldenström's macroglobulinemia is also known as:

Lymphoplasmacytic lymphoma. ***Lymphoplasmacytic lymphoma*** - **Waldenström's macroglobulinemia** is a type of **lymphoplasmacytic lymphoma (LPL)** that produces large amounts of **monoclonal IgM** protein [1]. - LPL is a **B-cell non-Hodgkin lymphoma** characterized by the presence of **lymphocytes, plasma cells, and plasmacytoid lymphocytes** in the bone marrow [1]. *Smoldering myeloma* - **Smoldering multiple myeloma (SMM)** is an **asymptomatic precursor** to multiple myeloma, characterized by high levels of **monoclonal protein (IgG or IgA)** and clonal plasma cells, but without end-organ damage. - It is distinct from Waldenström's macroglobulinemia, which involves **IgM paraprotein** and lymphoplasmacytic infiltration [1]. *Primary CNS lymphoma* - **Primary CNS lymphoma** is a rare and aggressive **non-Hodgkin lymphoma** that originates in the brain, spinal cord, or eyes, and is not typically associated with systemic **IgM paraproteinemia**. - Its clinical presentation involves **neurological symptoms** specific to CNS involvement, rather than symptoms of hyperviscosity or IgM-related coagulopathy. *MGUS (Monoclonal gammopathy of undetermined significance)* - **Monoclonal gammopathy of undetermined significance (MGUS)** is an asymptomatic condition with a **monoclonal protein (M-protein)** in the blood but no evidence of malignancy or end-organ damage. - While it can involve any immunoglobulin type, **IgM MGUS** is a precursor to Waldenström's macroglobulinemia, not the neoplasm itself. **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. 609-610.

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: 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: 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].

Question 1

'Hairy cell leukemia' is a neoplastic proliferation of which type of cells?

Accepted Answer

B lymphocytes

Answer

Myeloid cells

Answer

Macrophages

Answer

T cells

Question 2

A 15-year-old female presented to the emergency department with a history of recurrent epistaxis, hematuria, and hematochezia. There was a history of profuse bleeding from the umbilical stump at birth. Previous investigations revealed normal prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen levels. Her platelet counts as well as platelet function tests were normal, but the urea clot lysis test was positive. Which clotting factor is most likely to be deficient?

Accepted Answer

Factor XIII

Answer

Factor XII

Answer

Factor X

Answer

Factor XI

Question 3

Hemolytic anemia may be characterized by all of the following except which of the following?

Accepted Answer

Increased plasma haptoglobin level

Answer

Hyperbilirubinemia

Answer

Reticulocytosis

Answer

Hemoglobinuria

Question 4

What is the estimated amount of iron delivered from the transfusion of 2 units of packed red cells?

Accepted Answer

500mg

Answer

1g

Answer

100mg

Answer

250mg

Question 5

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 6

Neoplasm associated with Waldenström's macroglobulinemia is also known as:

Accepted Answer

Lymphoplasmacytic lymphoma

Answer

Smoldering myeloma

Answer

Primary CNS lymphoma

Answer

MGUS (Monoclonal gammopathy of undetermined significance)

Question 7

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 8

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 9

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 10

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

Accepted Answer

Hyperkalemia

Answer

Hyponatremia

Answer

Hypercalcemia

Answer

Increased serum albumin

Hematopathology — MCQs

Hematopathology — MCQs

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