Hematopathology Practice Questions

Q: A peripheral blood smear shows 'figure-8' shaped RBCs. Which additional finding would confirm Southeast Asian ovalocytosis?

Band 3 protein mutation. ***Band 3 protein mutation*** - **Southeast Asian ovalocytosis (SAO)** is caused by a specific mutation (deletion of 27 base pairs) in the gene encoding the **Band 3 protein**, leading to a truncated and rigid protein. - This rigidity of the **red blood cell (RBC) membrane**, due to the abnormal Band 3 protein, results in the characteristic **oval or 'figure-8' shape** seen on peripheral blood smears. *Heinz bodies* - **Heinz bodies** are precipitates of denatured hemoglobin, typically seen in conditions like **G6PD deficiency** or unstable hemoglobinopathies. - Their presence does not confirm SAO, which is a **membrane disorder**, not primarily a hemoglobinopathy. *Spectrin deficiency* - **Spectrin deficiency** is primarily associated with **hereditary spherocytosis** and **hereditary elliptocytosis**, causing spherocytes or elliptical RBCs, respectively [1]. - While it affects RBC shape, the specific **'figure-8' morphology** and the underlying genetic defect in SAO are distinct from spectrin abnormalities. *Increased osmotic fragility* - **Increased osmotic fragility** is a hallmark of conditions like **hereditary spherocytosis**, where RBCs are more susceptible to lysis in hypotonic solutions due to their spherical shape [1]. - In contrast, SAO RBCs often exhibit **decreased osmotic fragility** due to their increased membrane rigidity, providing protection against malaria. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-641.

Q: A cervical lymph node biopsy shows 'starry sky' pattern. Which immunophenotype would best support Burkitt lymphoma?

CD10+/c-myc+. ***CD10+/c-myc+*** - **Burkitt lymphoma** is characterized by the **t(8;14) translocation**, leading to **c-myc overexpression**, which is a key diagnostic marker. - **CD10** positivity is typical for **Burkitt lymphoma**, as it is a marker of germinal center B-cells from which this lymphoma originates [1]. *CD30+/ALK+* - This immunophenotype is characteristic of **anaplastic large cell lymphoma (ALCL)**, which presents with different histological features and clinical behavior. - ALK-positive ALCL is often seen in younger patients and has a distinct genetic alteration involving the **ALK gene**. *CD15+/CD30+* - This immunophenotype is classic for **classical Hodgkin lymphoma**, particularly the **nodular sclerosis** and **mixed cellularity** subtypes. - Hodgkin lymphoma involves large, atypical **Reed-Sternberg cells** surrounded by inflammatory cells, which is distinct from the "starry sky" pattern [1]. *CD5+/cyclin D1+* - This immunophenotype is highly suggestive of **mantle cell lymphoma**, which is associated with the **t(11;14) translocation** leading to **cyclin D1 overexpression**. - Mantle cell lymphoma typically has a diffuse or nodular growth pattern and does not exhibit the "starry sky" morphology. **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. 606.

Q: A blood smear shows RBCs with 'bite cells'. Which additional finding would best support G6PD deficiency?

Heinz bodies on supravital stain. ***Heinz bodies on supravital stain*** - **Bite cells** form when the spleen removes precipitated hemoglobin (Heinz bodies) from red blood cells, a hallmark of **oxidative stress** often seen in **G6PD deficiency** [1]. - **Heinz bodies** are denatured hemoglobin precipitates visible with **supravital stains** and their presence directly indicates oxidative damage to hemoglobin [1]. *Pappenheimer bodies* - These are **iron-containing granules** (siderosomes) found in red blood cells, typically associated with **sideroblastic anemia** or conditions of impaired iron utilization. - Their presence does not indicate the oxidative hemolysis characteristic of G6PD deficiency. *Cabot rings* - These are **thread-like inclusions** in red blood cells that are remnants of the **mitotic spindle**, often seen in conditions like **megaloblastic anemia** or lead poisoning. - They are not associated with hemoglobin precipitation or oxidative stress. *Howell-Jolly bodies* - These are **nuclear remnants** in red blood cells, indicating either **splenic hypofunction** (e.g., in sickle cell disease, post-splenectomy) or **hyposplenism**. - Their presence points to impaired splenic removal of cellular debris, not oxidative damage to hemoglobin like Heinz bodies. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 642-643.

Q: A bone marrow aspirate shows blast cells with multiple Auer rods arranged in bundles ('faggot cells'). Which molecular finding would confirm acute promyelocytic leukemia?

PML-RARA translocation. PML-RARA translocation - The presence of Auer rods arranged in bundles forming faggot cells is highly characteristic of acute promyelocytic leukemia (APL) [1]. - APL is genetically defined by the t(15;17) translocation, which creates the PML-RARA fusion gene [1], [2]. BCR-ABL translocation - This translocation, t(9;22), is characteristic of chronic myeloid leukemia (CML) and some cases of acute lymphoblastic leukemia (ALL), not APL [2]. - It does not typically present with the morphologic features of APL, such as faggot cells. AML1-ETO translocation - The t(8;21) translocation results in the AML1-ETO fusion gene and is associated with a specific subtype of acute myeloid leukemia (AML) with maturation, typically M2 [1]. - While Auer rods can be seen, the bundled faggot cells are not characteristic of this subtype [1]. inv(16) mutation - The inv(16)(p13q22) mutation is associated with acute myelomonocytic leukemia (AMML) with eosinophilia (M4Eo) [1]. - This subtype also features a specific morphology with abnormal eosinophils, not the faggot cells seen in APL [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. 620-621. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 605-607.

Q: Which of the following is a feature of Vit B12 deficiency anemia?

All of the options. **All of the options** - **Vitamin B12 deficiency anemia** is a type of **megaloblastic anemia** characterized by impaired DNA synthesis, leading to large, immature red blood cells and neutrophils [1]. - The presence of **macro-ovalocytes** and **hypersegmented neutrophils** are classic hematological features seen on a peripheral blood smear [1]. *Macro-ovalocytes* - **Macro-ovalocytes** are abnormally large, oval-shaped red blood cells, which result from arrested maturation due to the deficiency. - While a hallmark of B12 deficiency, it is not the sole identifying feature, as other megaloblastic anemias can also present with them [2]. *Megaloblastic anemia* - **Megaloblastic anemia** is a broad category of anemia characterized by large, immature, and dysfunctional red blood cells, which is the primary classification for B12 deficiency [2]. - While accurate, it doesn't encompass the specific morphological findings seen in the blood smear of B12 deficiency, unlike the other options. *Hypersegmented neutrophils* - **Hypersegmented neutrophils** are neutrophils with five or more nuclear lobes, a characteristic sign of impaired DNA synthesis affecting granulopoiesis [1]. - This feature is highly specific to **megaloblastic anemias**, distinguishing them from other causes of macrocytosis.

Q: A patient has MCV <80, MCH <23. Which type of anaemia shall be classified?

Microcytic hypochromic. ***Microcytic hypochromic*** - A **Mean Corpuscular Volume (MCV)** less than **80 fL** indicates **microcytosis** (small red blood cells) [1]. - A **Mean Corpuscular Hemoglobin (MCH)** less than **23 pg** indicates **hypochromia** (pale red blood cells due to reduced hemoglobin content) [1]. *Normocytic normochromic* - This classification refers to red blood cells with **normal MCV (80-100 fL)** and **normal MCH (27-32 pg)**. - Examples include anemia of chronic disease or acute blood loss, which do not fit the given lab values. *Normocytic hypochromic* - While **hypochromia (MCH 100 fL**, which is the opposite of the given MCV of <80. - The term "hyperchromic" is generally not used for anemia classification because red blood cells have a maximal hemoglobin concentration and cannot be truly hyperchromic. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Q: Blood is stored at what temperature:

4degC. *4degC* - Whole blood and red blood cell units are typically stored at **1-6°C** (commonly cited as 4°C) to maintain the viability and function of the red blood cells [1]. - This temperature range helps to slow down the metabolic activity of the cells and prevent bacterial growth, thereby extending their storage life to about 42 days [1]. * -20degC* - While some blood components, like **fresh frozen plasma**, are stored at even colder temperatures (e.g., -18°C or colder), -20°C is too cold for the routine storage of whole blood or red blood cells as it would cause cell damage due to freezing. - Freezing whole blood at this temperature would lead to **hemolysis** upon thawing, making it unsuitable for transfusion. * -70degC* - This extremely low temperature is used for storing very specific blood components, such as **cryopreserved red cells** (for rare blood types) or certain stem cell products, often requiring specialized freezers and cryoprotectants. - It is not the standard temperature for routine storage of whole blood or red blood cells due to the complexity and cost involved, as well as the need for cryoprotectants. * Room temperature* - Storing blood at room temperature (e.g., 20-24°C) would significantly reduce the storage time and increase the risk of **bacterial proliferation** and metabolic changes in the red blood cells. - Blood transfused after being at room temperature for extended periods carries a high risk of **bacterial contamination** and reduced red cell viability.

Question 1

A blood smear shows RBCs with fragmented, bizarre shapes and microspherocytes. Which additional finding would confirm hereditary pyropoikilocytosis?

Accepted Answer

Spectrin mutations

Answer

Osmotic fragility

Answer

G6PD deficiency

Answer

Hemoglobin H inclusions

Question 2

A lymph node biopsy shows 'onion skin' pattern of lymphoid cells. Which immunophenotype would confirm Castleman disease?

Accepted Answer

CD21+ follicular dendritic meshworks with polyclonal plasma cells

Answer

CD30+ Reed-Sternberg cells

Answer

CD5+/cyclin D1+ small cells

Answer

CD10+/BCL6+ large cells

Question 3

A peripheral blood smear shows 'figure-8' shaped RBCs. Which additional finding would confirm Southeast Asian ovalocytosis?

Accepted Answer

Band 3 protein mutation

Answer

Heinz bodies

Answer

Spectrin deficiency

Answer

Increased osmotic fragility

Question 4

A cervical lymph node biopsy shows 'starry sky' pattern. Which immunophenotype would best support Burkitt lymphoma?

Accepted Answer

CD10+/c-myc+

Answer

CD30+/ALK+

Answer

CD15+/CD30+

Answer

CD5+/cyclin D1+

Question 5

A blood smear shows RBCs with 'bite cells'. Which additional finding would best support G6PD deficiency?

Accepted Answer

Heinz bodies on supravital stain

Answer

Pappenheimer bodies

Answer

Cabot rings

Answer

Howell-Jolly bodies

Question 6

A bone marrow aspirate shows blast cells with multiple Auer rods arranged in bundles ('faggot cells'). Which molecular finding would confirm acute promyelocytic leukemia?

Accepted Answer

PML-RARA translocation

Answer

BCR-ABL translocation

Answer

AML1-ETO translocation

Answer

inv(16) mutation

Question 7

Which of the following has Autosomal Recessive inheritance?

Accepted Answer

Sickle cell anaemia

Answer

Osteogenesis imperfecta

Answer

Hereditary spherocytosis

Answer

von Willebrand Disease Type 1

Question 8

Which of the following is a feature of Vit B12 deficiency anemia?

Accepted Answer

All of the options

Answer

Macro-ovalocytes

Answer

Megaloblastic anemia

Answer

Hypersegmented neutrophils

Question 9

A patient has MCV <80, MCH <23. Which type of anaemia shall be classified?

Accepted Answer

Microcytic hypochromic

Answer

Normocytic normochromic

Answer

Normocytic hypochromic

Answer

Hyperchromic macrocytic

Question 10

Blood is stored at what temperature:

Accepted Answer

4degC

Answer

-20degC

Answer

-70degC

Answer

Room temperature

Hematopathology — MCQs

Hematopathology — MCQs

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