Hematopathology Practice Questions

Q: Which of the following immunophenotypic features is characteristic of mantle cell lymphoma?

CD 5+, CD 23-. ***CD 5+, CD 23-*** - Mantle cell carcinoma is characterized by **CD5 positivity** and **CD23 negativity**, helping differentiate it from other B-cell neoplasms [1][2]. - This immunophenotype is significant in identifying the **unique biological behavior** of mantle cell lymphoma [2]. *CD 5+, CD 10+* - CD10 positivity is more commonly associated with **follicular lymphoma** or **Burkitt lymphoma**, not mantle cell carcinoma. - Therefore, this oes not accurately reflect the immunophenotypic profile required for a diagnosis. *CD 5+, CD 23+* - CD23 positivity typically indicates **chronic lymphocytic leukemia (CLL)**, which contrasts with the profile seen in mantle cell carcinoma [1]. - This means that mantle cell carcinoma will not be identified with both CD5 and CD23 positivity together. *CD 5+, CD 25-* - CD25 is often expressed in conditions like **Hairy cell leukemia**, and its absence is not characteristic of mantle cell carcinoma. - Thus, while CD5+ is present, the lack of CD25 alone does not define mantle cell features. **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. 610-612. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 562-563.

Q: A 15-year-old boy presented with anemia and jaundice. On examination, his hemoglobin was 6 g/dL, ultrasound showed gallstones, and the peripheral smear showed the following. What is the most likely diagnosis?

Hereditary ovalocytosis. ***Hereditary ovalocytosis*** - Displays **anemia and jaundice** due to increased destruction of oval-shaped red blood cells [1]. - Associated with **gallstones** due to hemolysis, leading to bilirubin overload [1]. *Hereditary spherocytosis* - Characterized by **spherically shaped red blood cells**, leading to hemolytic anemia but not specifically ovalocytes [4,5]. - Typically presents with **splenomegaly** and increased fragility in osmotic fragility tests, which may not apply in this case [3]. *Hereditary acanthocytosis* - Involves irregularly shaped **acanthocytes** and primarily causes **neuroacanthocytosis syndrome**. - Symptoms include **movement disorders** and are not typically associated with uncomplicated jaundice and gallstones. *Hereditary xerocytosis* - Characterized by **dehydrated, stiff red blood cells**, leading to osmotically fragile cells but does not present with typical oval-shaped cells. - It is less common and mainly associated with **hyperbilirubinemia** but without typical jaundiced presentations. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 640. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 641-642. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598.

Q: All of the following are features of juvenile chronic myeloid leukemia except one.

Increased lymphadenopathy. ***Increased lymphadenopathy*** **(Correct Answer - This is the EXCEPTION)** - While juvenile chronic myeloid leukemia (JCML) can cause some **lymphadenopathy**, it is typically described as **mild** to **moderate**, not significantly increased. - **Prominent or markedly increased lymphadenopathy is NOT a typical feature of JCML** and would suggest a different diagnosis such as acute lymphoblastic leukemia or other lymphoproliferative disorders [1]. - This is the exception among the given options, as it does NOT characterize JCML. *Thrombocytopenia* - **Thrombocytopenia** (low platelet count) is a **common and typical feature** of juvenile chronic myeloid leukemia (JCML) due to bone marrow infiltration and increased platelet turnover [2]. - It often contributes to bruising and bleeding tendencies observed in affected children. *Mild lymphadenopathy* - **Mild lymphadenopathy** is a feature often observed in juvenile chronic myeloid leukemia (JCML), reflecting involvement of the reticuloendothelial system [1]. - This mild degree of lymph node enlargement is generally not prognostically significant and helps differentiate from more aggressive lymphoproliferative disorders. *Philadelphia chromosome is negative* - Juvenile chronic myeloid leukemia (JCML) is characterized by the **absence of the Philadelphia chromosome (Ph chromosome)**, which is a key diagnostic criterion. - This distinguishes JCML from adult chronic myeloid leukemia (CML), where the Ph chromosome (t(9;22) translocation) is present in >95% of cases. - The Ph-negative status is an essential feature of JCML diagnosis. **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. 625-629. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 604-605.

Q: A 50-year-old woman is discovered to have metastatic breast cancer and develops bacterial pneumonia one week after receiving her first dose of chemotherapy. Which of the following best explains this patient's susceptibility to bacterial infection?

Neutropenia. ***Neutropenia*** - **Chemotherapy** often causes **bone marrow suppression**, leading to a decrease in the absolute number of **neutrophils**, a condition known as neutropenia. - Neutrophils are crucial for the primary defense against **bacterial and fungal infections**, and their depletion significantly increases susceptibility, especially to **bacterial pneumonia**. *Depletion of serum complement* - While complement deficiency can increase susceptibility to certain infections, it is not a direct or common side effect of typical **chemotherapy regimens**. - Complement deficiencies are often **genetic** or related to specific **autoimmune diseases**, which are not indicated as the primary cause here. *Impaired neutrophil respiratory burst* - Impaired **neutrophil respiratory burst** (e.g., in **chronic granulomatous disease**) leads to a reduced ability to kill ingested bacteria, resulting in recurrent infections. - While chemotherapy can affect neutrophil function, severe impairment of the respiratory burst is not the primary mechanism of increased infection risk one week post-treatment; **neutropenia** is more immediate and profound. *Inhibition of clotting factor activation* - **Inhibition of clotting factor activation** would primarily manifest as **bleeding disorders**, not increased susceptibility to bacterial infections. - Chemotherapy can affect platelet count and function, but this mechanism does not directly explain increased risk of **bacterial pneumonia**.

Q: A 22-year-old woman presents with painless cervical lymphadenopathy, night sweats, and generalized pruritus. An enlarged cervical lymph node is removed for diagnosis, which shows broad bands of fibrosis on cut surface and histologically contains a mixture of cells, including lymphocytes, histiocytes, eosinophils, plasma cells, and scattered large cells with prominent nucleoli. Which of the following is the most likely condition?

Hodgkin disease. ***Hodgkin disease*** - The presence of **painless cervical lymphadenopathy**, night sweats, and **generalized pruritus** is classical for Hodgkin lymphoma [3][4]. - Histological findings of **broad bands of fibrosis** and a mixture of cell types including **Reed-Sternberg cells** confirm the diagnosis [1][2][3]. *Non-Hodgkin lymphoma* - Typically presents with **rapidly enlarging lymph nodes** and is often associated with extra-nodal involvement, differing from the features in this case [3]. - Histology usually shows a more homogeneous population of **malignant lymphoid cells**, not the fibrotic bands seen in Hodgkin disease [3]. *Reactive non-specific lymphadenitis* - This condition is characterized by **hyperplastic lymphoid tissue** and **neutrophilic infiltration**, often following infection, which does not match the findings. - It typically lacks the distinct cell types and fibrosis observed in the biopsy of Hodgkin disease. *Cat-scratch disease* - Caused by *Bartonella henselae*, it often presents with **granulomatous lymphadenopathy** rather than the mixed cellular response seen in Hodgkin disease. - Symptoms usually include a history of cat exposure and may present with **fever, fatigue**, and localized lymphadenopathy, not generalized pruritus. **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. 616-618. [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, p. 616. [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. 614-616. [4] 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.

Q: Lethal midline granuloma arises from which cell type?

NK cells. ***NK cells*** - **Lethal midline granuloma**, also known as **extranodal NK/T-cell lymphoma, nasal type**, is **predominantly derived from Natural Killer (NK) cells** in 70-80% of cases. - These malignant NK cells are **CD56 positive** and show a **cytotoxic phenotype** with expression of cytotoxic molecules (granzyme B, perforin, TIA-1). - The disease is strongly associated with **Epstein-Barr virus (EBV)** infection and presents with **destructive midline facial lesions** involving the nasal cavity, paranasal sinuses, and palate. - **Angioinvasion and necrosis** are characteristic histologic features. *T-cells* - While a **minority of cases** (20-30%) can arise from **cytotoxic T-cells** with a similar clinical presentation, NK cell origin is the predominant type. - T-cell derived cases are CD3 positive but CD56 negative, distinguishing them from the more common NK cell type. - The term "NK/T-cell lymphoma" reflects that both origins are possible, but NK cells are the primary cell of origin. *B-cells* - **B-cell lymphomas** are distinct entities that do not typically present as lethal midline granuloma. - B-cell lymphomas may involve extranodal sites but lack the characteristic aggressive destruction of midline facial structures seen in NK/T-cell lymphoma. *Macrophages* - **Macrophages** are phagocytic cells of the mononuclear phagocyte system and are not the origin of lymphomas. - Histiocytic disorders (like Langerhans cell histiocytosis) arise from cells of macrophage/dendritic cell lineage, but these are distinct from lymphoid malignancies.

Q: Which parameter is primarily used to diagnose macrocytosis in a complete blood count (CBC)?

Mean Corpuscular Volume (MCV). ***Mean Corpuscular Volume (MCV)*** - **MCV** measures the **average volume of red blood cells**, making it the primary indicator for classifying them as microcytic, normocytic, or macrocytic. - An **elevated MCV** (typically above 100 fL) indicates **macrocytosis**, meaning the red blood cells are larger than normal. *Mean Corpuscular Hemoglobin Concentration (MCHC)* - **MCHC** reflects the **average concentration of hemoglobin** in red blood cells, primarily used to classify cells as normochromic or hypochromic. - It does **not directly measure cell size** and therefore is not used to diagnose macrocytosis. *Hematocrit* - **Hematocrit** measures the **percentage of red blood cells** in a given volume of blood. - While it indicates the overall red cell mass, it **does not provide information about the average size** of individual red blood cells. *None of the options* - As **MCV** is explicitly listed and is the correct parameter for diagnosing macrocytosis, this option is incorrect. - The other parameters listed are important for other aspects of red blood cell analysis but not for classifying cell size.

Q: Flow cytometry is best used in the measurement of:

CD4+/CD8+ T-cell ratio in AIDS. **CD4+/CD8+ T-cell ratio in AIDS** - **Flow cytometry** is an ideal technique for identifying and quantifying specific cell populations based on their surface markers, like **CD4+** and **CD8+ T-cells**. - Monitoring the **CD4+/CD8+ ratio** is crucial in managing **AIDS** to assess immune status and disease progression. *Serum level of LDH* - **LDH (lactate dehydrogenase)** is an enzyme measured through **biochemical assays** in serum, not flow cytometry. - Flow cytometry measures cellular characteristics, while LDH levels reflect enzyme activity or tissue damage. *To count the RBCs in blood* - While flow cytometry can count cells, a **complete blood count (CBC)**, typically performed by automated hematology analyzers, is the standard and more efficient method for **RBC counting**. - Counting RBCs individually is not the primary or best use case for the advanced capabilities of flow cytometry. *Separation of proteins* - **Protein separation** is achieved through techniques like **electrophoresis**, chromatography, or mass spectrometry, not flow cytometry. - Flow cytometry is designed for analyzing and sorting individual cells or particles, not isolating protein mixtures.

Q: What is the most effective method to prevent transfusion-associated graft-versus-host disease?

Irradiation. Irradiation - **Irradiation** of blood products inactivates donor T-lymphocytes, which are responsible for initiating the immune attack in **transfusion-associated graft-versus-host disease (TA-GVHD)**. - This method is highly effective in preventing TA-GVHD, especially in immunocompromised patients or those receiving blood from biologically related donors. *Washing* - **Washing** removes plasma proteins, antibodies, and some leukocytes but is not effective in removing or inactivating all viable T-lymphocytes that can cause TA-GVHD. - It's primarily used to prevent allergic reactions or to remove excess potassium in certain patient populations. *Chemical treatment* - While experimental chemical treatments for pathogen inactivation exist, there is no widely accepted or clinically proven chemical treatment specifically for preventing **TA-GVHD** by inactivating lymphocytes. - Such methods are not part of routine blood banking practices for this purpose. *None of the options* - This option is incorrect because **irradiation** is a recognized and effective method for preventing **transfusion-associated graft-versus-host disease**.

Q: Which of the following is the most common myeloproliferative disorder?

Essential Thrombocythemia. ***Essential Thrombocythemia*** - **Essential thrombocythemia (ET)** is the **most common myeloproliferative neoplasm**, with an incidence of approximately 1.5-2.4 per 100,000 per year. - It is characterized by **persistent thrombocytosis** (platelet count >450,000/μL) and megakaryocytic proliferation [1]. - Commonly associated with **JAK2 V617F mutation** (~55-60%), **CALR mutations** (~25-30%), and **MPL mutations** (~3-5%) [2]. *Polycythemia rubra vera* - **Polycythemia vera (PV)** is the **second most common** classic MPN, with an incidence of approximately 0.8-2.3 per 100,000 per year. - Characterized by increased red blood cell mass, often with leukocytosis and thrombocytosis [1]. - Strongly associated with **JAK2 V617F mutation** (present in >95% of cases) [2][3]. *CML* - **Chronic myeloid leukemia (CML)** has similar incidence to PV (approximately 1-2 per 100,000 per year). - Defined by the presence of the **Philadelphia chromosome (BCR-ABL1 fusion gene)** [2]. - Treated distinctly with tyrosine kinase inhibitors (TKIs). *Myelofibrosis* - **Primary myelofibrosis (PMF)** is the **least common** of the classic MPNs, with an incidence of approximately 0.3-1.5 per 100,000 per year. - Characterized by bone marrow fibrosis, extramedullary hematopoiesis, and splenomegaly [3]. - Associated with **JAK2, CALR, or MPL mutations** [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, pp. 627-628. [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, p. 624. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.

Question 1

Which of the following immunophenotypic features is characteristic of mantle cell lymphoma?

Accepted Answer

CD 5+, CD 23-

Answer

CD 5+, CD 25-

Answer

CD 5+, CD 10+

Answer

CD 5+, CD 23+

Question 2

A 15-year-old boy presented with anemia and jaundice. On examination, his hemoglobin was 6 g/dL, ultrasound showed gallstones, and the peripheral smear showed the following. What is the most likely diagnosis?

Accepted Answer

Hereditary ovalocytosis

Answer

Hereditary acanthocytosis

Answer

Hereditary spherocytosis

Answer

Hereditary xerocytosis

Question 3

All of the following are features of juvenile chronic myeloid leukemia except one.

Accepted Answer

Increased lymphadenopathy

Answer

Thrombocytopenia

Answer

Mild lymphadenopathy

Answer

Philadelphia chromosome is negative

Question 4

A 50-year-old woman is discovered to have metastatic breast cancer and develops bacterial pneumonia one week after receiving her first dose of chemotherapy. Which of the following best explains this patient's susceptibility to bacterial infection?

Accepted Answer

Neutropenia

Answer

Depletion of serum complement

Answer

Impaired neutrophil respiratory burst

Answer

Inhibition of clotting factor activation

Question 5

A 22-year-old woman presents with painless cervical lymphadenopathy, night sweats, and generalized pruritus. An enlarged cervical lymph node is removed for diagnosis, which shows broad bands of fibrosis on cut surface and histologically contains a mixture of cells, including lymphocytes, histiocytes, eosinophils, plasma cells, and scattered large cells with prominent nucleoli. Which of the following is the most likely condition?

Accepted Answer

Hodgkin disease

Answer

Cat-scratch disease

Answer

Reactive non-specific lymphadenitis

Answer

Non-Hodgkin lymphoma

Question 6

Lethal midline granuloma arises from which cell type?

Accepted Answer

NK cells

Answer

B-cells

Answer

T-cells

Answer

Macrophages

Question 7

Which parameter is primarily used to diagnose macrocytosis in a complete blood count (CBC)?

Accepted Answer

Mean Corpuscular Volume (MCV)

Answer

Hematocrit

Answer

Mean Corpuscular Hemoglobin Concentration (MCHC)

Answer

None of the options

Question 8

Flow cytometry is best used in the measurement of:

Accepted Answer

CD4+/CD8+ T-cell ratio in AIDS

Answer

Serum level of LDH

Answer

To count the RBCs in blood

Answer

Separation of proteins

Question 9

What is the most effective method to prevent transfusion-associated graft-versus-host disease?

Accepted Answer

Irradiation

Answer

Washing

Answer

Chemical treatment

Answer

None of the options

Question 10

Which of the following is the most common myeloproliferative disorder?

Accepted Answer

Essential Thrombocythemia

Answer

Polycythemia rubra vera

Answer

CML

Answer

Myelofibrosis

Hematopathology — MCQs

Hematopathology — MCQs

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