Hematopathology Practice Questions

Q: A 25-year-old female presented with chronic fatigue, cyanosis with bluish lips, and arthralgia. A peripheral blood film is shown below. What is the likely cause?

Cold AIHA. ***Cold AIHA*** - **Red cell agglutination** on peripheral smear combined with **cyanosis** (bluish lips) and **arthralgia** forms the classic triad of cold autoimmune hemolytic anemia. - **IgM cold agglutinins** cause red cells to clump together at lower temperatures, leading to **vascular occlusion** and cyanosis in extremities. *Warm AIHA* - Typically shows **spherocytes** on peripheral smear rather than red cell agglutination or clumping. - **IgG antibodies** cause hemolysis at body temperature but do not produce the **cold-induced cyanosis** seen in this case. *Hemoglobinopathy* - Would show characteristic red cell morphology like **sickle cells** (sickle cell disease) or **target cells** (thalassemia). - Does not typically cause **red cell agglutination** or the specific combination of cyanosis with arthralgia. *G6PD Deficiency* - Peripheral smear would show **bite cells** and **Heinz bodies** during hemolytic episodes triggered by oxidative stress. - Does not cause **red cell agglutination** and typically presents with episodic hemolysis rather than chronic cyanosis.

Q: Bone marrow findings in AL amyloidosis typically include:

Plasmacytosis. **Explanation:** **AL (Amyloid Light-chain) Amyloidosis** is a plasma cell dyscrasia characterized by the deposition of insoluble amyloid fibrils derived from monoclonal immunoglobulin light chains. 1. **Why Plasmacytosis is correct:** The underlying pathology in AL amyloidosis is a clonal proliferation of plasma cells in the bone marrow [1]. These abnormal plasma cells produce excess light chains (usually Lambda more than Kappa) that misfold and deposit in tissues [1]. While the plasma cell count is often low (typically <10%), it is the primary source of the amyloidogenic protein, making **plasmacytosis** the hallmark finding. In some cases, it may coexist with overt Multiple Myeloma. 2. **Why the other options are incorrect:** * **Granulomatous reaction:** This is characteristic of chronic inflammatory or infectious diseases (e.g., Tuberculosis, Sarcoidosis), not plasma cell disorders. * **Fibrosis:** While marrow fibrosis can be seen in Myelofibrosis or advanced Myeloma, it is not a defining or typical feature of AL amyloidosis. * **Giant cell formation:** This is associated with foreign body reactions or specific infections and is not a feature of the monoclonal protein production seen in amyloidosis. **High-Yield Clinical Pearls for NEET-PG:** * **Staining:** Amyloid shows **Apple-green birefringence** under polarized light when stained with **Congo Red**. * **Most Common Type:** AL amyloidosis is the most common form of systemic amyloidosis. * **Organ Involvement:** Macroglossia (enlarged tongue) and Periorbital ecchymosis ("Raccoon eyes") are highly suggestive clinical signs. * **Diagnosis:** While bone marrow shows plasmacytosis, the most sensitive site for biopsy to confirm amyloid deposition is **Abdominal Fat Pad aspiration** or rectal biopsy. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-618.

Q: All are true regarding Hodgkin's lymphoma, except?

CNS is the commonest site of involvement. **Explanation:** **1. Why Option A is the correct (False) statement:** In Hodgkin’s Lymphoma (HL), **Central Nervous System (CNS) involvement is extremely rare** (occurring in <1% of cases). HL typically spreads in a predictable, contiguous fashion via the lymphatic system. The most common sites of involvement are the cervical, supraclavicular, and mediastinal lymph nodes. If a patient with lymphoma presents with CNS involvement, it is much more likely to be Non-Hodgkin Lymphoma (NHL), specifically Primary CNS Lymphoma or high-grade subtypes like Burkitt’s [1]. **2. Analysis of other options:** * **Option B:** The **Reed-Sternberg (RS) cell** is the diagnostic hallmark of HL [1]. These are large, multinucleated B-cells with prominent "owl-eye" nucleoli. * **Option C:** **Nodular Sclerosis (NS)** is the most common subtype of HL. It has a strong predilection for the **mediastinum** (seen in ~80% of NS cases) and typically affects young females [1]. * **Option D:** The bulk of a Hodgkin’s tumor is actually an **inflammatory infiltrate** (reactive background) consisting of T-cells, eosinophils, plasma cells, and neutrophils, recruited by cytokines (like IL-5) secreted by the RS cells. **Clinical Pearls for NEET-PG:** * **Bimodal Age Distribution:** Peaks at 15–35 years and again after 50 years. * **Pel-Ebstein Fever:** A classic but rare cyclic fever pattern associated with HL [1]. * **Alcohol-induced pain:** Pain in the lymph nodes after alcohol consumption is a highly specific (though rare) sign of HL. * **CD Markers:** Classic RS cells are typically **CD15+ and CD30+**, but CD45 negative. * **Prognosis:** Lymphocyte Predominant has the best prognosis; Lymphocyte Depleted has the worst.

Question 1

A 48-year-old woman presented with a two-month history of weakness. On examination, cervical lymph nodes were enlarged, and the spleen was palpable 2 cm below the costal margin. The platelet count was 237 x 10^9/L and the total leukocyte count was 40 x 10^9/L, with coarse clumped chromatin noted. Bone marrow revealed a nodular lymphoid infiltrate. Peripheral blood lymphoid cells were negative for CD19, CD5, CD20, and CD23, and also negative for CD79B and FMC-7. What is the most likely diagnosis?

Accepted Answer

Chronic lymphocytic leukemia

Answer

T-cell rich B-cell lymphoma with leukemic spillover in blood.

Answer

Mantle cell lymphoma

Answer

A definite diagnosis cannot be made in this patient without a lymph node biopsy.

Question 2

Which of the following is NOT a good prognostic factor for Acute Myeloid Leukemia (AML)?

Accepted Answer

Acute megakaryoblastic leukemia (AMkL)

Answer

Age less than 2 years

Answer

Acute promyelocytic leukemia (APL)

Answer

Associated with Down syndrome

Question 3

All of the following statements about Fanconi's anemia are true, EXCEPT?

Accepted Answer

Autosomal dominant inheritance

Answer

Hypocellular Bone Marrow

Answer

Congenital Anomalies

Answer

Usually normocytic / macrocytic cell morphology

Question 4

A 25-year-old female presented with chronic fatigue, cyanosis with bluish lips, and arthralgia. A peripheral blood film is shown below. What is the likely cause?

Accepted Answer

Cold AIHA

Answer

Warm AIHA

Answer

Hemoglobinopathy

Answer

G6PD Deficiency

Question 5

All of the following are true about blood transfusion protocols EXCEPT:

Accepted Answer

Blood transfusions should commence within 60 minutes of removing the blood bag from refrigeration.

Answer

Whole blood or packed red blood cells must be transfused within 4 hours.

Answer

Transfusion sets should include a standard filter of 170 micrometers.

Answer

The usual needle size for blood transfusion is 18-19 gauge.

Question 6

Which of the following tests may be abnormal in disseminated intravascular coagulation (DIC), except?

Accepted Answer

Clot solubility

Answer

Prothrombin time (PT)

Answer

Activated partial thromboplastin time (APTT)

Answer

D-dimer

Question 7

What is the primary cause of red blood cell sickling in sickle cell disease?

Accepted Answer

Decreased hemoglobin solubility

Answer

Decreased hemoglobin stability

Answer

Altered hemoglobin function

Answer

Altered oxygen binding capacity of hemoglobin

Question 8

Bone marrow findings in AL amyloidosis typically include:

Accepted Answer

Plasmacytosis

Answer

Granulomatous reaction

Answer

Fibrosis

Answer

Giant cell formation

Question 9

All are true regarding Hodgkin's lymphoma, except?

Accepted Answer

CNS is the commonest site of involvement

Answer

Characteristic cell is a Reed Sternberg cell

Answer

Mediastinal involvement is common in nodular sclerosis type

Answer

Eosinophils, plasma cells and neutrophils increase

Question 10

A 14-year-old boy presents with acute onset of right flank pain, which developed after he helped his father paint the ceiling of his bedroom. Physical examination demonstrates an area of ecchymosis in the right flank that is tender to palpation. The patient has a lifelong history of easy bruising. His brother shows the same tendency. The serum level of clotting factor VIII is less than 2% of normal. Which of the following is the most likely underlying mechanism for bleeding tendency in this patient?

Accepted Answer

Genetic defect involving the factor VIII gene

Answer

Circulating antibodies directed against factor VIII

Answer

Decreased hepatic synthesis of multiple coagulation factors

Answer

Deficiency of vitamin K

Hematopathology — MCQs

Hematopathology — MCQs

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