Hematopathology Practice Questions

Q: PNH patients have deficient surface proteins that normally protect red blood cells from activated complements. Which two surface proteins are deficient in these patients?

CD 45 and CD 55. ***CD 55 and CD 59*** - **CD 55 (decay accelerating factor)** and **CD 59 (protectin)** are crucial for inhibiting the complement system, protecting red blood cells from lysis in PNH. - Deficiencies in these proteins lead to increased **susceptibility** of red cells to **complement-mediated hemolysis**. *CD58 and CD 59* - **CD 58** is involved in T-cell interaction but does not solely protect red cells from complement. - While **CD 59** is a correct answer, the absence of **CD 55** makes this option incorrect for PNH. *CD 45 and CD 59* - **CD 45** is primarily a **leukocyte common antigen**, not involved in protecting red cells from complement activation. - This orrectly mentions **CD 59**, but lacks **CD 55**, making it unsuitable as an answer. *CD 51 and CD 59* - **CD 51** is associated with integrin functions and does not play a role in protecting red blood cells from complements. - Although **CD 59** is relevant, the deficiency in **CD 55** highlights the incompleteness of this option.

Q: Which type of immunoglobulin is primarily associated with cold agglutinin disease?

IgM. ***IgM*** - Cold agglutinins are primarily composed of **IgM antibodies** [1] which are produced in response to certain infections, notably Mycoplasma pneumoniae. - They can cause **cold agglutinin disease**, leading to hemolytic anemia when the blood is exposed to cooler temperatures [2]. *IgG* - While IgG is the most abundant antibody in blood, it is not responsible for cold agglutination. - IgG typically functions in **immune memory** and neutralization rather than agglutination at cold temperatures. *IgD* - IgD is mainly found on the surface of **B cells** and has a role in initiating B cell activation. - It plays a minimal role in the antibody response and does not participate in cold agglutination. *IgA* - IgA is primarily involved in mucosal immunity, found in secretions like saliva and tears. - It does not function as a cold agglutinin; hence, it is not associated with cold temperature-related agglutination. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 154-155. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 651-652.

Q: Which of the following best describes the shape of Birbeck granules?

Resembling a tennis racket. ***Tennis racket*** [1] - Birbeck granules are distinctive for their **tennis racket shape**, consisting of an elongated area and a bulbous end [1]. - They are typically found in **Langerhans cell histiocytosis** and are associated with **CD1a+** staining [1]. *Bat* - The term "bat" does not accurately describe the structure or morphology of Birbeck granules. - Unlike bats, Birbeck granules have a **specific elongated shape** with a bulbous tip rather than bat wings. *Hockey stick* - This shape implies a straight handle and a flat blade, which does not represent the **widely tapered ends** of Birbeck granules. - The **characteristic morphology** does not align with any hockey stick features. *Ball* - The term "ball" indicates a spherical shape, which is not fitting for Birbeck granules, as they are **elongated** and striated. - Birbeck granules are defined specifically by their **tapered structure**, contrasting markedly with a circular form. **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. 630.

Q: Helmet cells are characteristic of anemia of?

Hemolytic uremic syndrome (HUS). ***Hemolytic uremic syndrome*** - Helmet cells are **fragmented red blood cells** associated with **microangiopathic hemolytic anemia** [1], commonly seen in hemolytic uremic syndrome. - This condition frequently results in **thrombocytopenia** and acute renal failure. *Acanthocytosis* - Acanthocytosis is characterized by **spiky red blood cells** (acanthocytes) rather than helmet cells. - It is commonly associated with **liver disease** and **abetalipoproteinemia**, not hemolytic anemia. *Polysplenia* - Polysplenia is a condition involving multiple spleens but does not typically relate to the **formation of helmet cells**. - It may cause **asplenic complications**, but anemia characteristics do not include helmet cells. *Spherocytosis* - Spherocytosis involves the presence of **spherical red blood cells** rather than fragmented (helmet) cells. - It is associated with **hereditary conditions** like hereditary spherocytosis, which leads to increased hemolysis but not typically to helmet cells. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 596-597.

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: 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: 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: 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.

Question 1

PNH patients have deficient surface proteins that normally protect red blood cells from activated complements. Which two surface proteins are deficient in these patients?

Accepted Answer

CD 45 and CD 55

Answer

CD 55 and CD 58

Answer

CD 55 and CD 59

Answer

CD 51 and CD 55

Question 2

Which type of immunoglobulin is primarily associated with cold agglutinin disease?

Accepted Answer

IgM

Answer

IgG

Answer

IgA

Answer

IgD

Question 3

Which of the following best describes the shape of Birbeck granules?

Accepted Answer

Resembling a tennis racket

Answer

Resembling a hockey stick

Answer

Resembling a bat

Answer

Resembling a ball

Question 4

Helmet cells are characteristic of anemia of?

Accepted Answer

Hemolytic uremic syndrome (HUS)

Answer

Disseminated intravascular coagulation (DIC)

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Autoimmune hemolytic anemia (AIHA)

Question 5

A 68-year-old woman was admitted with a history of weakness for two months. On examination, cervical lymph nodes were found enlarged and the spleen was palpable 2 cm below the costal margin. Her hemoglobin was 10.5 g/dL, platelet count 27 × 10^9/L, and total leukocyte count 40 × 10^9/L, which included 80% mature lymphoid cells with coarse clumped chromatin. Bone marrow revealed a nodular lymphoid infiltrate. The peripheral blood lymphoid cells were positive for CD19, CD5, CD20, and CD23, and negative for CD79B and FMC-7. The histopathological examination of the lymph node in this patient will most likely exhibit effacement of lymph node architecture by?

Accepted Answer

A monomorphic lymphoid proliferation with admixed proliferation centers

Answer

A polymorphous population of lymphocytes, plasma cells, eosinophils and scattered large binucleated cells

Answer

A predominantly follicular pattern with variably-sized follicles effacing nodal architecture

Answer

A diffuse proliferation of medium to large lymphoid cells with high mitotic rate

Question 6

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 7

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 8

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 9

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 10

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

Hematopathology — MCQs

Hematopathology — MCQs

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