Hematopathology Practice Questions

Q: What is the neutrophil count for moderate neutropenia?

500 - 1000/mm3. ***500 - 1000/mm3*** - **Moderate neutropenia** is defined as an absolute neutrophil count (ANC) between **500 and 1000 cells/mm³**. - Patients in this range have an increased risk of infection, although generally less severe than those with very low counts. * 1000/mm3* - An absolute neutrophil count of **greater than 1000 cells/mm³** is generally considered within the **mild neutropenia** or even **normal** range, depending on the exact cutoff used in specific contexts (normal range typically 1500-8000 cells/mm³). - This level of neutropenia is usually not clinically significant in terms of increased infection risk. *100/mm3* - An absolute neutrophil count of **100 cells/mm³** falls into the category of **severe neutropenia** (also known as agranulocytosis), which is a critical level. - This count indicates an extremely high risk of serious bacterial and fungal infections.

Q: What is the maximum recommended storage period for platelets in blood banks under standard storage conditions?

5 days. ***5 days*** - The maximum recommended storage period for **platelets** in blood banks under standard conditions is **5 days** as per Indian guidelines (NACO, DGHS). - Platelets are stored at **20-24°C with continuous agitation** to maintain viability and function. - This duration balances optimal platelet function while minimizing the risk of **bacterial contamination**, which increases significantly with longer storage. *3 days* - This is an outdated storage limit that was used in earlier protocols. - While still safe, limiting storage to 3 days would significantly reduce the **availability of platelets** for transfusion and increase wastage. *7 days* - The **US FDA** has approved 7-day storage for platelets with advanced **bacterial detection systems** (since 2020). - However, this is **not the standard in India**; Indian blood banks follow the 5-day protocol unless equipped with approved bacterial testing technology. - Extended storage beyond 5 days requires special regulatory approval and validated bacterial detection methods. *10 days* - Storing platelets for 10 days significantly increases the risk of **bacterial contamination** and marked decline in **platelet viability** and hemostatic function. - There are **no current standard recommendations** that permit routine platelet storage for 10 days in any major blood banking guideline.

Q: On medical check-up of a Punjabi student, the following findings were seen: Hb of 9.9 gm/dL, RBC count of 5.1 million, MCV of 62.5 fL, and RDW of 13.51%. What is the most probable diagnosis?

Thalassemia trait. ***Thalassemia trait*** - The low **MCV (62.5 fl)** and normocytic **RBC count (5.1 million)** indicate microcytic anemia, typical of thalassemia trait. - The **RDW of 13.51%** is relatively normal, distinguishing it from iron deficiency anemia where RDW would be elevated. *Anemia of chronic disease* - This condition usually presents with **normocytic or microcytic anemia**, but typically involves a **low serum iron** and **high ferritin**, which are not mentioned here [1]. - It is often associated with chronic inflammatory conditions, not fitting the RBC parameters given [2]. *HbD* - HbD is a hemoglobin variant typically identified in **specific ethnic groups**, but it does not cause significant anemia like described here with low MCV. - Diagnosis is based on specific hemoglobin electrophoresis patterns, which are not indicated in this case. *Iron deficiency anemia* - Would typically show **lower ferritin levels** and higher RDW, indicating a variation in red cell size, which contrasts with the given RDW [1]. - The RBC count is normal, while iron deficiency often presents with *low RBC counts* and microcytic indices [2].

Q: What is the defect in Glanzmann's thrombasthenia?

GpIIb-IIIa. ***Gp1Ib-IlIa*** - Glanzmann's thrombasthenia is characterized by a defect in the **Gp1b-IlIa complex**, which is essential for platelet aggregation. - Patients with this condition exhibit **prolonged bleeding time** and poor clot formation due to ineffective platelet-fibrinogen binding. *GpIIIa-I lb* - This option refers to a component of the GpIIb-IIIa complex, which is primarily involved in **platelet aggregation** and is not the defect in Glanzmann's thrombasthenia. - Defects in GpIIb-IIIa lead to conditions such as **Glanzmann's thrombasthenia** [1], but specifically, the Gp1b-IlIa complex is implicated. *Gpfilb-I la* - This designation does not correspond correctly to either a known receptor complex related to platelet function or to Glanzmann's thrombasthenia. - Mislabeling of the glanzmann's complex results in confusion as it does not specify the **correct interaction involved in platelet aggregation**. *GpIla-Illb* - This combination refers to components that are not involved in Glanzmann's thrombasthenia, which specifically involves the **Gp1b-IlIa interaction**. - GpIla-Illb complex is part of the **integrin family**, but its defect does not lead to the bleeding problems seen in Glanzmann's thrombasthenia [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669.

Q: Blood is stored at what temperature in blood bank?

1 to 6 degrees Celsius. ***1 to 6 degrees Celsius*** - This temperature range is critical for maintaining the **viability of red blood cells** and slowing down metabolic processes, ensuring the blood is safe for transfusion. - At this temperature, the growth of most **pathogenic bacteria** is inhibited, and the degradation of blood components is minimized. *-2 to -4 degrees Celsius* - Temperatures below 0°C would cause **ice crystal formation** within the red blood cells, leading to hemolysis and making the blood unsuitable for transfusion. - This range is typically used for the **long-term storage of frozen plasma** or cryoprecipitate after special processing, not whole blood or red blood cell units. *-2 to 0 degrees Celsius* - This temperature range is incorrect for storing whole blood or packed red blood cells as it is still likely to cause **freezing and cellular damage**. - While close to freezing, even slight freezing can result in **hemolysis** and render the blood product unusable. *6 to 12 degrees Celsius* - Temperatures above 6°C significantly increase the **metabolic activity of red blood cells** and the risk of **bacterial growth**. - This range would lead to a more rapid decline in the viability and safety of the stored blood, reducing its shelf life considerably.

Q: In which subtype of Hodgkin's disease are popcorn cells predominantly found?

Lymphocyte predominant subtype. ***Lymphocyte predominant*** - Popcorn cells, or **"L&H" (Lymphocytic and Histiocytic) cells**, are characteristic of lymphocyte predominant Hodgkin's disease, indicating a **rich lymphocytic background** [1]. - This subtype is more commonly seen in younger patients and is associated with a better prognosis relative to other types of Hodgkin's lymphoma [1]. *Mixed cellularity* - This subtype is characterized by a **varied cellular composition** including numerous Reed-Sternberg cells but does not specifically feature popcorn cells [2]. - It tends to have a higher number of **eosinophils** and **plasma cells** rather than lymphocytes predominating [2]. *Lymphocyte depletion* - Lymphocyte depletion is marked by a **deficient lymphocytic component** and an abundance of Reed-Sternberg cells, lacking the characteristic popcorn cells. - This variant is often more aggressive and typically has a poorer prognosis. *Nodular sclerosis* - Nodular sclerosis is recognized by the presence of **collagen bands** separating lymphoid nodules and also does not include popcorn cells. - This form is the most common variant of Hodgkin's lymphoma and often presents with **mediastinal masses**. **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. 618. [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. 616-618.

Q: A child presents with intermittent jaundice and splenomegaly. There is a history of similar complaints in the elder brother. Peripheral smear shows the following finding. What is the most appropriate investigation for this condition?

Osmotic fragility test. ***Osmotic fragility test*** - The image shows **spherocytes** (dense, small red cells lacking central pallor), a hallmark of **hereditary spherocytosis**. The clinical picture of intermittent jaundice, splenomegaly, and a family history further supports this diagnosis. - The **osmotic fragility test** measures the red blood cell's susceptibility to hemolysis in hypotonic solutions and is the most appropriate test to confirm hereditary spherocytosis. *Coombs test* - The Coombs test (direct antiglobulin test) detects **antibodies on the surface of red blood cells** or in the serum. It is primarily used to diagnose **autoimmune hemolytic anemia**. - Hereditary spherocytosis is a membrane defect, not an immune-mediated condition, so the Coombs test would typically be negative. *G6PD deficiency testing* - **Glucose-6-phosphate dehydrogenase (G6PD) deficiency** leads to episodic hemolytic anemia triggered by oxidative stress (e.g., fava beans, certain drugs, infections). - While it causes episodic hemolysis and jaundice, the peripheral smear typically shows **Heinz bodies** and **bite cells** during hemolytic episodes, not spherocytes, and without specific triggers, it doesn't fit the clinical picture as well as hereditary spherocytosis. *Flow cytometry for PNH* - **Flow cytometry** is used to diagnose **paroxysmal nocturnal hemoglobinuria (PNH)** by detecting the absence of GPI-anchored proteins (e.g., CD55, CD59) on blood cells. - PNH is characterized by dark urine (hemoglobinuria), thrombosis, and bone marrow failure, and its peripheral smear does not typically show spherocytes.

Q: A child presents with recurrent chest infections and abdominal pain. There is a history of 1 blood transfusion in the past. On examination, he had icterus and mild splenomegaly. Electrophoresis shows increased HbA2, HbF, and S spike. What is the likely diagnosis?

Sickle Beta Thalassemia. ***Sickle Beta Thalassemia*** - The combination of **sickle cell disease manifestations** (recurrent chest infections, abdominal pain, icterus, splenomegaly) with **electrophoresis showing increased HbA2, elevated HbF, and S spike** is diagnostic of **Sickle Beta Thalassemia**. - **Increased HbA2 (>3.5%)** is the key distinguishing feature that differentiates this from pure sickle cell disease. It indicates co-inheritance of a **beta-thalassemia gene** along with the **sickle cell gene**. - Clinical presentation is similar to sickle cell disease with **vaso-occlusive crises**, **acute chest syndrome**, hemolytic anemia, and organomegaly. - The severity depends on the type: S/β⁰-thalassemia (no HbA production) is clinically more severe and similar to SS disease, while S/β⁺-thalassemia (reduced HbA) tends to be milder. *Sickle cell disease* - Pure sickle cell disease (HbSS) presents with similar clinical features: recurrent chest infections, abdominal pain, hemolysis, and splenomegaly. - However, electrophoresis would show **normal or only slightly elevated HbA2 (2-3%)**, not the increased HbA2 mentioned in this case. - The presence of significantly increased HbA2 rules out pure sickle cell disease. *HbC disease* - Patients with HbC disease typically have **mild chronic hemolytic anemia** and **splenomegaly** but usually lack severe vaso-occlusive crises. - Electrophoresis would show primarily **HbC**, not an S spike. - The clinical picture is much milder than described in this case. *Aplastic anemia* - Characterized by **pancytopenia** due to bone marrow failure, leading to fatigue, infections, and bleeding tendency. - Does not involve hemolysis, icterus, or abnormal hemoglobin variants on electrophoresis. - The electrophoresis findings completely exclude this diagnosis.

Q: All trans retinoic acid is used in the treatment of acute promyelocytic leukemia (APL) associated with which genetic mutation?

PML-RARA fusion gene. ***PML-RARA fusion gene*** - The **PML-RARA fusion gene** is the hallmark genetic abnormality in **acute promyelocytic leukemia (APL)**, which results from a **t(15;17) chromosomal translocation** [1]. - **All-trans retinoic acid (ATRA)** targets the PML-RARA fusion protein, inducing differentiation of the immature promyelocytes and promoting remission in APL patients. *BCR-ABL fusion gene* - The **BCR-ABL fusion gene** is associated with **chronic myeloid leukemia (CML)**, resulting from the **Philadelphia chromosome (t(9;22))** [2]. - While a target for therapy, it is treated with **tyrosine kinase inhibitors (TKIs)**, not ATRA [2]. *CMYC gene alteration* - **CMYC alterations**, such as translocations, are characteristic of **Burkitt lymphoma** and some cases of diffuse large B-cell lymphoma [2]. - These alterations are not specifically associated with APL or sensitive to ATRA therapy. *CEBPA mutation* - **CEBPA mutations** are found in a subset of patients with **acute myeloid leukemia (AML)**, particularly those with a normal karyotype. - While relevant to AML prognosis and treatment stratification, these mutations do not define APL or respond to ATRA.

Question 1

What is the neutrophil count for moderate neutropenia?

Accepted Answer

500 - 1000/mm3

Answer

<500/mm3

Answer

>1000/mm3

Answer

100/mm3

Question 2

What is the maximum recommended storage period for platelets in blood banks under standard storage conditions?

Accepted Answer

5 days

Answer

7 days

Answer

3 days

Answer

10 days

Question 3

On medical check-up of a Punjabi student, the following findings were seen: Hb of 9.9 gm/dL, RBC count of 5.1 million, MCV of 62.5 fL, and RDW of 13.51%. What is the most probable diagnosis?

Accepted Answer

Thalassemia trait

Answer

HbD

Answer

Iron deficiency anemia

Answer

Anemia of chronic disease

Question 4

What is the purpose of the Direct Coombs' test?

Accepted Answer

Detection of immune components bound to red blood cells (RBCs).

Answer

Detection of immune components in the serum.

Answer

Detection of cell surface antigens on red blood cells (RBCs).

Answer

Detection of antigens circulating in the serum.

Question 5

What is the defect in Glanzmann's thrombasthenia?

Accepted Answer

GpIIb-IIIa

Answer

GpIIIa-Ib

Answer

GpIIa-IIIb

Answer

GpIIb-Ia

Question 6

Blood is stored at what temperature in blood bank?

Accepted Answer

1 to 6 degrees Celsius

Answer

-2 to -4 degrees Celsius

Answer

-2 to 0 degrees Celsius

Answer

6 to 12 degrees Celsius

Question 7

In which subtype of Hodgkin's disease are popcorn cells predominantly found?

Accepted Answer

Lymphocyte predominant subtype

Answer

Nodular sclerosis subtype

Answer

Mixed cellularity subtype

Answer

Lymphocyte depletion subtype

Question 8

A child presents with intermittent jaundice and splenomegaly. There is a history of similar complaints in the elder brother. Peripheral smear shows the following finding. What is the most appropriate investigation for this condition?

Accepted Answer

Osmotic fragility test

Answer

Coombs test

Answer

G6PD deficiency testing

Answer

Flow cytometry for PNH

Question 9

A child presents with recurrent chest infections and abdominal pain. There is a history of 1    blood transfusion in the past. On examination, he had icterus and mild splenomegaly.    Electrophoresis shows increased HbA2, HbF, and S spike. What is the likely diagnosis?

Accepted Answer

Sickle Beta Thalassemia

Answer

HbC disease

Answer

Sickle cell disease

Answer

Aplastic anemia

Question 10

All trans retinoic acid is used in the treatment of acute promyelocytic leukemia (APL) associated with which genetic mutation?

Accepted Answer

PML-RARA fusion gene

Answer

BCR-ABL fusion gene

Answer

CMYC gene alteration

Answer

CEBPA mutation

Hematopathology — MCQs

Hematopathology — MCQs

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