Hematopathology Practice Questions

Q: A patient presents with intermittent fever, no weight loss and enlarged retroperitoneal lymph nodes. Peripheral smear is normal. Gross sample and its histopathology slide is shown below. Comment on the diagnosis.

Castleman disease. ***Castleman disease*** - The image shows **"lollipop lesions"** or **"onion-skinning"** of follicles with **regressed germinal centers** and prominent mantle zones, characteristic of the hyaline-vascular variant of Castleman disease. - Clinical features like **intermittent fever** and **enlarged retroperitoneal lymph nodes** with **no weight loss** can be seen in Castleman disease, particularly the unicentric type. *Non-Hodgkin's lymphoma* - This would typically show effacement of the normal lymph node architecture by a **monotonous proliferation of atypical lymphocytes**. - While it can cause lymphadenopathy and fever, the specific histological features presented do not align with typical non-Hodgkin's lymphoma. *Angiolymphoid hyperplasia* - Characterized by **multiple vascular channels** lined by plump endothelial cells with **lymphoid infiltrates**. - It often presents as subcutaneous nodules, usually in the head and neck region, and lacks the follicular changes seen here. *IgG4 related disease* - Histologically, this disease is characterized by a **dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells**, storiform fibrosis, and obliterative phlebitis. - While it can cause lymph node enlargement, the specific follicular changes in the image are not typical of IgG4-related disease.

Q: A macrophage engulfs different cells as shown in the image. This is known as?

Emperipolesis. ***Emperipolesis*** - The image shows a large cell (likely a macrophage or megakaryocyte) containing **intact various blood cells** within its cytoplasm without signs of degeneration. - **Emperipolesis** is specifically defined as the **active penetration of one cell by another**, where both the engulfed and engulfing cells remain viable. *Phagocytosis* - **Phagocytosis** involves the ingestion and subsequent **destruction or degradation** of foreign particles, microorganisms, or cellular debris. - The cells within the macrophage in the image appear **morphologically intact** and not in a state of degradation. *Killing* - **Killing** implies the process by which a cell actively destroys another cell, often through mechanisms like **apoptosis or necrosis**. - There are **no morphological features** in the image to suggest that the engulfed cells are being actively killed or are undergoing degeneration. *Cytotoxicity* - **Cytotoxicity** refers to the ability of certain immune cells (e.g., cytotoxic T lymphocytes, NK cells) to **kill target cells**. - This process usually involves specific recognition and induction of target cell death, which is not what is depicted by the presence of intact cells within another cell.

Q: Which of the following hematological findings are seen in pregnant women with thalassemia trait?

Raised HbA₂ and low MCV. ***Raised HbA₂ and low MCV*** - Beta-thalassemia trait is characterized by a **compensatory increase in HbA₂** (alpha2-delta2 globin chains) synthesis and **microcytic (low MCV)** red blood cells [1]. - This combination is a classic finding that helps differentiate thalassemia trait from iron deficiency anemia in pregnant women. *Low serum total iron binding capacity* - **Low total iron binding capacity (TIBC)** is typically seen in **anemia of chronic disease**, where iron stores are often adequate or high. - In thalassemia trait, iron stores are usually normal or increased, and TIBC is usually normal or slightly increased. *Low HbA₂ and raised MCV* - **Low HbA₂** is seen in alpha-thalassemia trait or iron deficiency anemia, not beta-thalassemia trait [2]. - **Raised MCV (macrocytosis)** is characteristic of conditions like **folate or B12 deficiency** or megaloblastic anemia, which is not associated with uncomplicated thalassemia trait [1]. *Low MCHC* - **Low MCHC (mean corpuscular hemoglobin concentration)** indicates hypochromic red blood cells and is found in various microcytic anemias, including **iron deficiency anemia** [1]. - While it can be present in thalassemia trait, it is not as specific as the combination of **raised HbA₂** and **low MCV** for distinguishing beta-thalassemia trait from other microcytic conditions [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 588-591. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 649-650.

Q: The genetic inheritance of Haemophilia is

Sex-linked recessive. ***Sex-linked recessive*** - Hemophilia, specifically hemophilia A and B, is inherited in an **X-linked recessive** pattern. - Males, having only one X chromosome, are predominantly affected, as a single copy of the mutated gene on the X chromosome leads to the condition. *Autosomal recessive* - Autosomal recessive disorders require **two copies** of the mutated gene (one from each parent) to manifest, and affect males and females equally. - This inheritance pattern does not explain the male predominance and carrier status in females seen in hemophilia. *Autosomal dominant* - Autosomal dominant disorders require only **one copy** of the mutated gene to cause the disease, and also affect males and females equally. - This pattern would mean an affected parent has a 50% chance of passing the condition to each child, which is not characteristic of hemophilia. *Sex-linked dominant* - Sex-linked dominant disorders would affect both males and females, with affected fathers passing the trait to **all their daughters** but none of their sons. - This pattern is not consistent with the inheritance of hemophilia, where affected mothers can pass it to their sons.

Q: Which of the following blood components has the shortest shelf life?

Platelets. ***Platelets*** - **Platelets** have the shortest shelf life among the listed blood components, typically stored for **5 days** at room temperature with continuous agitation. - Their short shelf life is due to the risk of **bacterial contamination** and reduced therapeutic efficacy over time. *Fresh frozen plasma* - **Fresh frozen plasma (FFP)** can be stored for up to **1 year** at temperatures of -18°C or colder. - Once thawed, it must be used within **24 hours** if stored at 1-6°C. *Cryoprecipitate* - **Cryoprecipitate**, like FFP, can be stored for up to **1 year** at -18°C or colder. - After thawing, it is stable for **6 hours** at room temperature or **24 hours** if pooled and stored at room temperature. *Red Blood Cells* - **Red blood cells (RBCs)**, when stored in additive solutions, have a shelf life of up to **42 days** at 1-6°C. - This longer shelf life is due to specialized storage conditions that minimize metabolic activity and preserve cell integrity.

Q: Consider the following statements: Haemophilia A (haemophilia) and Haemophilia B (christmas disease) 1. are variants of the same disease process 2. are due to congenital deficiency of factor VIII and factor IX respectively 3. both are sex linked characteristics and transmitted by asymptomatic females 4. can occur both in males and females Select the correct answer using the code given below:

2 and 3. ***2 and 3*** - Hemophilia A is caused by a deficiency of **Factor VIII**, and Hemophilia B is caused by a deficiency of **Factor IX** [1]. These are distinct but clinically similar genetic disorders of the coagulation cascade. - Both hemophilia A and B are **X-linked recessive disorders**, meaning they predominantly affect males and are transmitted by asymptomatic female carriers [2]. *1, 2 and 4* - While both hemophilia A and B result in similar bleeding phenotypes, they are due to deficiencies in different **coagulation factors** (Factor VIII and Factor IX, respectively), making them distinct diseases, not variants of the same process [2]. - While hemophilia mainly affects males, a female can be affected if she inherits two affected X chromosomes (one from each parent) or has **Turner syndrome** (XO) with an affected X chromosome, but this is extremely rare. *2 only* - This option correctly identifies the specific factor deficiencies for Hemophilia A (**Factor VIII**) and Hemophilia B (**Factor IX**) [1]. - However, it omits the crucial aspect of their **sex-linked inheritance** and transmission by asymptomatic females, which is a fundamental characteristic of both conditions [2]. *3 only* - This option correctly states that both hemophilia A and B are **sex-linked characteristics** and are transmitted by **asymptomatic females** [2]. - However, it fails to mention the specific deficient factors (Factor VIII and Factor IX), which is key to understanding the underlying pathology of these two distinct disorders.

Q: The ideal temperature to store the whole blood in blood-bank is

4°C. ***4°C*** - Whole blood is typically stored at **1 to 6°C**, with **4°C** being the optimal compromise to preserve red blood cell viability and minimize bacterial growth [1]. - This temperature range allows for a standard storage duration of **21 to 42 days**, depending on the anticoagulant-preservative solution used [1]. *-4°C* - Temperatures below freezing point (**0°C**) would cause **hemolysis** due to ice crystal formation within the red blood cells, making the blood unsuitable for transfusion. - While frozen storage is used for specific blood components (e.g., cryopreserved red blood cells or plasma), it requires different protocols and cryoprotectants. *8°C* - Storing whole blood at **8°C** is above the recommended range and would significantly decrease the storage time due to increased metabolic activity and **bacterial proliferation**. - This temperature also leads to a faster decline in **red blood cell viability** and function. *0°C* - Storing whole blood at **0°C** is at the freezing point of water and close to the freezing point of blood, which can lead to initial **ice crystal formation** and subsequent hemolysis. - Although it's within the recommended 1-6°C range, maintaining exactly 0°C without fluctuations into freezing territory is challenging and poses a risk to blood quality [1].

Q: A 34-year-old female presented with low hemoglobin, platelet count of 25,000 / mm³, raised PT and APTT. The image shows a peripheral smear. Which of the following fusion genes is affected?

PML-RARA. ***PML-RARA*** - The image shows **promyelocytes** with prominent granules and **Auer rods** (indicated by the arrow), which are characteristic of Acute Promyelocytic Leukemia (APL). [1] - APL is strongly associated with the **t(15;17)** translocation, leading to the **PML-RARA** fusion gene. The patient’s clinical presentation of pancytopenia (low hemoglobin, low platelet count) and prolonged PT/APTT leading to **DIC**, is highly consistent with APL. [1] *RUNX1-RUNX1T1* - This fusion gene is associated with **Acute Myeloid Leukemia (AML) M2** (AML with maturation), often due to t(8;21) translocation. [1] - While it is a type of AML, the peripheral smear and clinical features are more specific to APL, not AML M2. *INV 16* - Inversion 16, denoted as **inv(16)(p13q22)**, is associated with an AML subtype that typically presents with **myelomonocytic differentiation** and **abnormal eosinophils** in the bone marrow. [1] - The image and clinical findings do not suggest myelomonocytic morphology or the presence of abnormal eosinophils, making APL a more likely diagnosis. *IGH-NSD2* - This fusion gene is characteristic of **Multiple Myeloma**, a plasma cell malignancy. - Multiple myeloma would present with plasma cell proliferation, bone lesions, and hypercalcemia, which differ significantly from the acute leukemia presentation seen here. **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.

Question 1

A patient presents with intermittent fever, no weight loss and enlarged retroperitoneal lymph nodes. Peripheral smear is normal. Gross sample and its histopathology slide is shown below. Comment on the diagnosis.

Accepted Answer

Castleman disease

Answer

Non-Hodgkin's lymphoma

Answer

Angiolymphoid hyperplasia

Answer

IgG4 related disease

Question 2

A macrophage engulfs different cells as shown in the image. This is known as?

Accepted Answer

Emperipolesis

Answer

Phagocytosis

Answer

Killing

Answer

Cytotoxicity

Question 3

Which of the following hematological findings are seen in pregnant women with thalassemia trait?

Accepted Answer

Raised HbA₂ and low MCV

Answer

Low serum total iron binding capacity

Answer

Low MCHC

Answer

Low HbA₂ and raised MCV

Question 4

Which of the following are metabolic causes of splenic enlargement?

Accepted Answer

Amyloid and Gaucher's disease

Answer

Porphyria and Felty's syndrome

Answer

Myelofibrosis and Weil's disease

Answer

Rickets and Still's disease

Question 5

Folic acid deficiency is characterized by the following features except

Accepted Answer

Microcytes

Answer

Macrocytes

Answer

Howell-Jolly bodies

Answer

Hypersegmented neutrophils

Question 6

The genetic inheritance of Haemophilia is

Accepted Answer

Sex-linked recessive

Answer

Autosomal recessive

Answer

Autosomal dominant

Answer

Sex-linked dominant

Question 7

Which of the following blood components has the shortest shelf life?

Accepted Answer

Platelets

Answer

Fresh frozen plasma

Answer

Cryoprecipitate

Answer

Red Blood Cells

Question 8

Consider the following statements: Haemophilia A (haemophilia) and Haemophilia B (christmas disease)

1. are variants of the same disease process
2. are due to congenital deficiency of factor VIII and factor IX respectively
3. both are sex linked characteristics and transmitted by asymptomatic females
4. can occur both in males and females

Select the correct answer using the code given below:

Accepted Answer

2 and 3

Answer

1, 2 and 4

Answer

2 only

Answer

3 only

Question 9

The ideal temperature to store the whole blood in blood-bank is

Accepted Answer

4°C

Answer

-4°C

Answer

8°C

Answer

0°C

Question 10

A 34-year-old female presented with low hemoglobin, platelet count of 25,000 / mm³, raised PT and APTT. The image shows a peripheral smear. Which of the following fusion genes is affected?

Accepted Answer

PML-RARA

Answer

RUNX1-RUNX1T1

Answer

INV 16

Answer

IGH-NSD2

Hematopathology — MCQs

Hematopathology — MCQs

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