Hematopathology Practice Questions

Q: BCR-ABL fusion gene is MOST CHARACTERISTICALLY seen in?

CML. ***CML*** - The **BCR-ABL gene mutation** is characteristic of **Chronic Myeloid Leukemia (CML)**, resulting from a translocation between chromosomes 9 and 22 [1]. - This mutation leads to the production of the **BCR-ABL fusion protein**, which promotes cell proliferation and inhibits apoptosis [1]. *AML* - Acute Myeloid Leukemia (AML) does not typically exhibit the **BCR-ABL fusion gene**; rather, it is associated with various other genetic mutations. - Key features of AML include **myeloblast proliferation** and it presents with different cytogenetic abnormalities like **FLT3 or NPM1 mutations**. *CLL* - Chronic Lymphocytic Leukemia (CLL) is characterized by the accumulation of **mature lymphocytes**, not the **BCR-ABL mutation**. - It is often associated with mutations such as **TP53** and **NOTCH1**, distinct from myeloid malignancies. *ALL* - Acute Lymphoblastic Leukemia (ALL) is primarily linked with **chromosomal translocations** involving **the TCF3** gene or others, but not specifically with **BCR-ABL**. - In ALL, **lymphoid progenitor cells** proliferate, whereas CML is primarily a **myeloid process** associated with the BCR-ABL gene [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. 624-625.

Q: The subtype of Hodgkin's disease which is histogenetically distinct from all the other subtypes is:

Lymphocyte predominant. ***Lymphocyte predominant*** - This subtype is characterized by the presence of **popcorn cells** (a variant of Reed-Sternberg cells) and is histogenically distinct from other forms of Hodgkin's lymphoma [1]. - It typically features a **predominance of lymphocytes**, contrasting with the other subtypes that may show mixed cellularity or different cell backgrounds [1]. *Lymphocyte depleted* - This subtype has a high number of **Reed-Sternberg cells** and is often associated with **immunocompromised states**, making it less distinct than lymphocyte predominant [2]. - It typically presents with a poor prognosis and is characterized by **depletion** of lymphocytes rather than predominance [2]. *Nodular sclerosis* - This is one of the most common subtypes of Hodgkin's lymphoma, featuring **fibrosis** and often affecting mediastinal lymph nodes. - While distinct, it shares histopathological features with other standard forms of Hodgkin's lymphoma, thus not being **histogenically distinct**. *Mixed cellularity* - This subtype presents a heterogeneous mix of cells, including **Reed-Sternberg cells** and leukocytes, and is the most frequent subtype after nodular sclerosis [2]. - It is associated with lower lymphocyte predominance, thus lacking the unique histological characteristics found in lymphocyte predominant Hodgkin's lymphoma. **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] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 559-560.

Q: A 62-year-old man presents with back pain for 4 months. Laboratory tests show a white blood cell count of 3700/microliter, hemoglobin of 10.3 g/dL, hematocrit of 31.1%, mean corpuscular volume of 85 fL, and a platelet count of 110,000/microliter. His total serum protein is 8.5 g/dL, with an albumin level of 4.1 g/dL. A chest radiograph shows lucencies in the vertebral bodies. A sternal bone marrow aspirate yields a dark red jelly-like material. Which cell type is most likely to be numerous in this aspirate?

Plasma cells. ***Plasma cells*** - The presence of **dark red jelly-like material** in the bone marrow aspirate indicates a possible **myeloma**, which is characterized by an abundance of plasma cells [1]. - The lucencies in the vertebral bodies combined with low white blood cell count and **anemia** further support a diagnosis related to plasma cell dyscrasias like multiple myeloma [1][2][3]. *Osteoblasts* - Osteoblasts are responsible for **bone formation** and would not be expected to be numerous in this scenario where there is evidence of **bone loss** (lucencies). - Their presence would typically indicate **active bone remodeling**, not consistent with the findings of anemia and low cellularity in the context presented. *Fibroblasts* - Fibroblasts are involved in **connective tissue formation** and repair, and their numbers are not typically indicative of any hematologic disorders. - They do not directly relate to the findings of **anemia** and **bone lesions**, making them unlikely to be predominant in this aspirate. *Giant cells* - Giant cells, often associated with **inflammatory responses** or granulomatous conditions, do not correlate with the clinical picture of myeloma or the findings outlined in the case. - Their presence would suggest a different pathological process, unrelated to the presence of **plasma cells** in bone marrow aspirate [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 617-618. [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. 606-609.

Q: A 9-year-old girl has a bleeding diathesis, with prolonged activated partial thromboplastin time (APTT) but normal prothrombin time (PT) and normal platelet count. The diagnosis is?

Von Willebrand's disease. ***Von Willebrand's disease*** - This condition is characterized by a **deficiency or dysfunction of von Willebrand factor (vWF)**, leading to impaired platelet adhesion and stabilization of factor VIII [2], [3]. - While it primarily affects **platelet function**, it can also cause a prolonged **APTT** due to the role of vWF in protecting factor VIII from degradation, thus impacting the intrinsic coagulation pathway [3]. *Factor V deficiency* - Factor V is a component of the **common pathway** of coagulation. - A deficiency would typically prolong both the **PT and APTT** [1]. *Haemophilia A* - This is a deficiency of **Factor VIII**, which is part of the **intrinsic pathway** [1]. - Haemophilia A causes a prolonged **APTT** but a normal PT, similar to the patient's presentation. However, vWD is more common and can also present with similar findings. *Haemophilia B* - This is a deficiency of **Factor IX**, also part of the **intrinsic pathway** [1]. - Haemophilia B also causes a prolonged **APTT** but a normal PT. However, vWD is a more common inheritable bleeding disorder.

Q: Which of the following findings is characteristic of megaloblastic anemia?

All of the options. ***All of the above*** - In megaloblastic anemia, various atypical red blood cell characteristics can be observed, including **Cabot rings**, **Howell-Jolly bodies**, and **basophilic stippling** [1]. - These features arise due to ineffective erythropoiesis and increased reticulocyte production, which are hallmark traits of megaloblastic changes [1]. *Cabot ring* - These are **red cell inclusions** that can be seen in conditions associated with **disrupted hemoglobin synthesis** or **abnormal nucleic acid metabolism**, but are not specific to megaloblastic anemia. - More commonly observed in conditions like **lead poisoning** or **myelodysplastic syndromes**. *Howell Jolly bodies* - These are nuclear remnants seen in red blood cells, typically present after **splenectomy** or in cases of **hemolytic anemia**, rather than specifically indicating megaloblastic anemia. - While they can be seen in megaloblastic anemia, they are not definitive; they signify **poor splenic function**. *Basophilic stippling* - Generally associated with **lead poisoning**, **thalassemias**, and certain types of **anemia**, but not exclusively indicative of megaloblastic anemia. - It results from **RNA remnants** in red blood cells and is more frequently noted in other forms of anemia. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 594-595.

Q: Which of the following Non-Hodgkin's lymphomas was historically classified as intermediate grade in the Working Formulation?

Diffuse, small cleaved cell lymphoma. ***Diffuse, small cleaved cell lymphoma*** - This subtype was classified under the **intermediate-grade** category in the Working Formulation, which was an older classification system for non-Hodgkin lymphomas. - The Working Formulation aimed to group lymphomas based on their **prognostic behavior**, with intermediate grade indicating a moderate clinical course. *Small non-cleaved cell lymphoma* - This lymphoma, now recognized as **Burkitt lymphoma**, was classified as **high-grade** in the Working Formulation due to its aggressive nature and rapid progression [3]. - It is characterized by a very high **proliferative rate** and aggressive clinical course [3]. *Lymphoblastic lymphoma* - This aggressive lymphoma was also classified as **high-grade** in the Working Formulation [2]. - It arises from immature lymphoid precursors and is notorious for its rapid growth and tendency to involve the **bone marrow** and **CNS** [2],[3]. *Large cell immunoblastic lymphoma* - This aggressive subtype was categorized under the **high-grade** category in the Working Formulation. - It is histologically defined by large, immature-appearing immunoblasts and typically has an **unfavorable prognosis** if left untreated [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 563-564. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 560-561. [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, p. 606.

Q: Which one of the following is used for the preservation of blood for transfusions?

Citrate phosphate dextrose. ***Citrate phosphate dextrose*** - This anticoagulant solution is commonly used for blood preservation in blood banks because **citrate** chelates **calcium ions**, preventing coagulation. - The **phosphate** acts as a buffer to maintain pH, and **dextrose** provides energy for red blood cell metabolism during storage. - **CPD** allows blood storage for up to **21 days**, and when adenine is added (CPDA-1), storage can be extended to **35 days**. *Heparin* - **Heparin** is an anticoagulant that works by enhancing the activity of **antithrombin III**, but it is generally not used for long-term blood storage for transfusion due to its short half-life and potential to activate platelets upon prolonged contact. - While effective as an anticoagulant, heparin is not suitable for routine blood bank storage as it does not adequately support the viability of red blood cells over extended periods. *Thrombin* - **Thrombin** is a procoagulant enzyme involved in the final stages of the coagulation cascade, converting fibrinogen to fibrin, thus promoting clot formation. - Using thrombin would cause immediate blood clotting, making it entirely unsuitable for preserving blood for transfusion purposes. *EDTA* - **EDTA (Ethylenediaminetetraacetic acid)** is a chelating anticoagulant commonly used in **laboratory samples** for hematological testing (e.g., CBC). - However, it is **not used for blood transfusion** because it can cause morphological changes in cells and is toxic when transfused, making it unsuitable for therapeutic blood storage.

Q: What is the most common type of non-Hodgkin's lymphoma found in the orbit?

B-cell. ***B-cell*** - The most common type of non-Hodgkin's lymphoma in the orbit is **B-cell lymphoma**, which typically presents as a **painless mass** [1]. - **B-cell lymphomas** are more frequently associated with systemic lymphoproliferative disorders and have a higher incidence in this region. *NK-cell* - NK-cell lymphomas are rare compared to B-cell and usually present with more aggressive features. - They are less common in the orbit and are often associated with other systemic diseases like **HIV**. *Plasma cell* - Plasma cell neoplasms, such as **multiple myeloma**, are more related to bone marrow involvement rather than orbit-specific lymphomas. - These do not typically arise in the orbit and are less frequently classified under non-Hodgkin's lymphomas. *T-cell* - T-cell lymphomas are less prevalent compared to B-cell lymphomas and have different clinical presentations. - They can be aggressive and may occur in the orbit, but not as commonly as B-cell types. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Eye, pp. 1341-1342.

Question 1

BCR-ABL fusion gene is MOST CHARACTERISTICALLY seen in?

Accepted Answer

CML

Answer

AML

Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

Acute Lymphoblastic Leukemia (ALL)

Question 2

The subtype of Hodgkin's disease which is histogenetically distinct from all the other subtypes is:

Accepted Answer

Lymphocyte predominant

Answer

Nodular sclerosis

Answer

Mixed cellularity

Answer

Lymphocyte depleted

Question 3

A 62-year-old man presents with back pain for 4 months. Laboratory tests show a white blood cell count of 3700/microliter, hemoglobin of 10.3 g/dL, hematocrit of 31.1%, mean corpuscular volume of 85 fL, and a platelet count of 110,000/microliter. His total serum protein is 8.5 g/dL, with an albumin level of 4.1 g/dL. A chest radiograph shows lucencies in the vertebral bodies. A sternal bone marrow aspirate yields a dark red jelly-like material. Which cell type is most likely to be numerous in this aspirate?

Accepted Answer

Plasma cells

Answer

Osteoblasts

Answer

Giant cells

Answer

Fibroblasts

Question 4

A 9-year-old girl has a bleeding diathesis, with prolonged activated partial thromboplastin time (APTT) but normal prothrombin time (PT) and normal platelet count. The diagnosis is?

Accepted Answer

Von Willebrand's disease

Answer

Factor V deficiency

Answer

Haemophilia A

Answer

Haemophilia B

Question 5

Which of the following findings is characteristic of megaloblastic anemia?

Accepted Answer

All of the options

Answer

Macrocytic red blood cells

Answer

Reticulocytopenia

Answer

Hypersegmented neutrophils

Question 6

The 'hair on end' appearance is characteristically seen in which of the following conditions?

Accepted Answer

Thalassemia major

Answer

Sickle cell anaemia

Answer

Hereditary spherocytosis

Answer

G6PD deficiency

Question 7

Which of the following Non-Hodgkin's lymphomas was historically classified as intermediate grade in the Working Formulation?

Accepted Answer

Diffuse, small cleaved cell lymphoma

Answer

Small non-cleaved cell lymphoma

Answer

Lymphoblastic lymphoma

Answer

Large cell immunoblastic lymphoma

Question 8

Which one of the following is used for the preservation of blood for transfusions?

Accepted Answer

Citrate phosphate dextrose

Answer

Heparin

Answer

Thrombin

Answer

EDTA

Question 9

What is the most common type of non-Hodgkin's lymphoma found in the orbit?

Accepted Answer

B-cell

Answer

T-cell

Answer

NK-cell

Answer

Plasma cell

Question 10

An intensive care patient develops sepsis. His blood pressure drops to 60/15 mmHg, and he starts to bleed from venipuncture sites. Disseminated intravascular coagulation is suspected. Which of the following sets of stat laboratory values would confirm the likely diagnosis?

Accepted Answer

Elevated PT, elevated PTT, decreased platelets

Answer

Decreased PT, elevated PTT, decreased platelets

Answer

Decreased PT, elevated PTT, elevated platelets

Answer

Decreased PT, decreased PTT, decreased platelets

Hematopathology — MCQs

Hematopathology — MCQs

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