Hematopathology Practice Questions

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.

Q: To replenish the inventory, blood banks routinely issue blood packets which are close to the expiry date. Which of the following will be the closest to expiry date, according to the anticoagulant used and the method of storage of the packet:

SAGM, 25 days with irradiation. ***SAGM, 25 days with irradiation*** - **SAGM** (Saline-Adenine-Glucose-Mannitol) is an additive solution that extends the storage life of red blood cells significantly. - While SAGM usually allows for storage up to **42 days**, if the blood is **irradiated**, the shelf life is reduced to **28 days from collection or 14 days from irradiation, whichever is sooner**. Given the options, 25 days with irradiation falls within this reduced shelf life, making it the closest to expiry among the provided choices (implying the Irradiation was done earlier). *ACD, 14 days* - **ACD** (Acid Citrate Dextrose) is an older anticoagulant primarily used for apheresis products. - Red blood cells collected with ACD typically have a maximum storage duration of **21 days**. While 14 days is within this, other anticoagulant-additive combinations offer longer storage, and this option is not the closest to expiry when considering the maximum allowed. *CPDA, 27 days* - **CPDA** (Citrate Phosphate Dextrose Adenine) provides a standard shelf life of **35 days** for red blood cells. - While 27 days represents blood that has been stored for a significant period, it still has 8 more days until its maximum expiry, making it less "close to expiry" than the irradiated SAGM option which would expire sooner. *SAGM, 35 days* - **SAGM** (Saline-Adenine-Glucose-Mannitol) allows for the storage of red blood cells for up to **42 days** from collection. - At 35 days, a unit stored in SAGM still has 7 days until its maximum expiry date (if not irradiated), making it less "close to expiry" than a unit that had been irradiated.

Q: Which of the following is not expressed in majority of cases of pediatric B-cell acute lymphoblastic leukemia?

CD7. ***CD7*** - **CD7** is a **T-cell associated antigen** and is typically **not expressed** on B-cell acute lymphoblastic leukemia (ALL) cells [1]. - Its presence would suggest a **T-cell ALL** or an atypical mixed phenotype leukemia, rather than a B-cell ALL. *Terminal deoxynucleotidyl transferase (TdT)* - **TdT** is a **nuclear enzyme** that adds random nucleotides to V(D)J gene segments during lymphoid development and is a **marker of immaturity** in both B and T cell lymphoblasts [2]. - It is **expressed in the majority of B-cell ALL cases** and is crucial for diagnosis. *CD19* - **CD19** is an **early and pan-B-cell marker** that is consistently expressed by B-cell lymphoblasts throughout their development [1]. - It is a **key diagnostic marker** for B-cell ALL. *CD10* - **CD10**, also known as **common acute lymphoblastic leukemia antigen (CALLA)**, is expressed in most progenitor and pre-B ALLs [1]. - Its presence is an important marker used in the immunophenotyping of **B-cell ALL**, particularly the common ALL subtype [2]. **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. 598. [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. 599-600.

Q: t(2,8) is associated with:

B cell ALL. ***B cell ALL*** - The translocation **t(2;8)(p11;q24)** is a **variant cytogenetic abnormality** specifically associated with **Burkitt lymphoma/leukemia**, a highly aggressive form of mature B-cell neoplasm, which can present as B-cell ALL. [1] - This variant translocation (occurring in ~15% of Burkitt lymphoma cases) leads to the **dysregulation of the MYC oncogene** on chromosome 8q24 due to its juxtaposition with the **kappa (κ) immunoglobulin light chain gene (IGK)** on chromosome 2p11. [1] - The most common translocation in Burkitt lymphoma is **t(8;14)(q24;q32)** involving MYC and the immunoglobulin heavy chain gene IGH (~80% of cases), while **t(8;22)** involving the lambda light chain occurs in ~5% of cases. [1] *T cell ALL* - T-cell ALL is primarily associated with translocations involving **T-cell receptor genes (e.g., TCRα/δ on 14q11, TCRβ on 7q34)** and various oncogenes like *TAL1*, *LMO1*, *LMO2*, *HOXA*, and *NKX2-5*. - It does not typically involve the **t(2;8) translocation**. *CML* - **Chronic Myeloid Leukemia (CML)** is classically defined by the presence of the **Philadelphia chromosome**, an acquired reciprocal translocation **t(9;22)(q34;q11)**. - This translocation results in the formation of the **BCR-ABL1 fusion gene**, which encodes a constitutively active tyrosine kinase. *CLL* - **Chronic Lymphocytic Leukemia (CLL)** is most frequently associated with cytogenetic abnormalities such as **deletions of 13q14, 11q22-23 (ATM gene), and 17p13 (TP53 gene)**, and **trisomy 12**. - The **t(2;8) translocation** is not characteristic of CLL. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 324-325.

Q: Which one of the following blood fractions is stored at -18°C?

Cryoprecipitate. ***Cryoprecipitate*** - **Cryoprecipitate** contains high concentrations of **Factor VIII**, **von Willebrand factor**, **Factor XIII**, and **fibrinogen**, which are unstable at room temperature [1]. - Storage at **-18°C or colder** is essential to maintain the activity and stability of these coagulation factors for up to one year. *Packed red cells* - **Packed red cells** are typically stored at **2-6°C** for up to 42 days, not frozen, as freezing would damage the red blood cells. - Their primary function is to increase **oxygen-carrying capacity** in patients with anemia or acute blood loss. *Human albumin* - **Human albumin** is a stable protein and is typically stored at **room temperature (2-25°C)**, as freezing is not required to preserve its function. - It is used for **volume expansion** and to maintain **oncotic pressure** in conditions like hypovolemia or hypoalbuminemia. *Platelet concentrate* - **Platelet concentrates** must be stored at **20-24°C** with continuous agitation for up to 5-7 days to maintain their viability and function [2]. - Freezing would irreversibly damage the platelets, making them ineffective for treating **thrombocytopenia** or platelet dysfunction [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 582-583. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 581-582.

Q: In polycythemia vera all are true except

High ESR. ***High ESR*** - In **polycythemia vera**, the **increased red blood cell mass** leads to **hyperviscosity** and often a **decreased erythrocyte sedimentation rate (ESR)**, as the red blood cells settle more slowly due to hindrance. - A high ESR would be **atypical** for polycythemia vera and would suggest an underlying inflammatory process or another condition. *Increased leukocyte alkaline phosphatase* - **Leukocyte alkaline phosphatase (LAP) score** is typically **increased** in polycythemia vera, reflecting the **overproduction of mature neutrophils**. - This is a distinguishing feature from disorders like **chronic myeloid leukemia (CML)**, where the LAP score is low [3]. *Thrombocytosis* - **Thrombocytosis** (elevated platelet count) is a common finding in polycythemia vera due to the **panmyelosis** associated with the disease [1], [3]. - The **JAK2V617F mutation**, frequently present in PV, stimulates megakaryocyte production, leading to increased platelets [1]. *Decreased levels of erythropoietin* - In polycythemia vera, red blood cell production is **independent of erythropoietin**, leading to a **suppressed** or **very low erythropoietin level** in response to the high red cell mass [1], [2]. - This differentiates PV from **secondary polycythemia**, where erythropoietin levels are elevated [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 663-664. [3] 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. 626-627.

Question 1

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 2

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 3

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 4

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 5

Blood is stored at what temperature:

Accepted Answer

4degC

Answer

-20degC

Answer

-70degC

Answer

Room temperature

Question 6

To replenish the inventory, blood banks routinely issue blood packets which are close to the expiry date. Which of the following will be the closest to expiry date, according to the anticoagulant used and the method of storage of the packet:

Accepted Answer

SAGM, 25 days with irradiation

Answer

ACD, 14 days

Answer

CPDA, 27 days

Answer

SAGM, 35 days

Question 7

Which of the following is not expressed in majority of cases of pediatric B-cell acute lymphoblastic leukemia?

Accepted Answer

CD7

Answer

Terminal deoxynucleotidyl transferase (TdT)

Answer

CD19

Answer

CD10

Question 8

t(2,8) is associated with:

Accepted Answer

B cell ALL

Answer

T cell ALL

Answer

CML

Answer

CLL

Question 9

Which one of the following blood fractions is stored at -18°C?

Accepted Answer

Cryoprecipitate

Answer

Packed red cells

Answer

Human albumin

Answer

Platelet concentrate

Question 10

In polycythemia vera all are true except

Accepted Answer

High ESR

Answer

Increased leukocyte alkaline phosphatase

Answer

Thrombocytosis

Answer

Decreased levels of erythropoietin

Hematopathology — MCQs

Hematopathology — MCQs

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