Hematopathology Practice Questions

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.

Q: Basophils are decreased in which of the following conditions?

Cushing's syndrome. **Explanation:** The correct answer is **Cushing’s syndrome**. **1. Why Cushing’s Syndrome is Correct:** Basopenia (a decrease in basophil count) is a characteristic finding in states of **hypercortisolism**, such as Cushing’s syndrome or exogenous steroid administration [1]. Glucocorticoids cause a redistribution of basophils from the peripheral blood into other compartments and inhibit their release from the bone marrow. Additionally, basopenia can be seen in acute stress (due to endogenous cortisol release), hyperthyroidism, and acute hypersensitivity reactions (where basophils degranulate and are sequestered in tissues). **2. Why the Other Options are Incorrect:** * **Polycythemia Vera:** This is a myeloproliferative neoplasm (MPN) characterized by the overproduction of all three cell lines (panmyelosis). Basophilia (increased basophils) is a common diagnostic clue in MPNs. * **Basophilic Leukemia:** This is a rare form of acute myeloid leukemia where primary proliferation consists of basophilic precursors, leading to a massive increase in basophil counts. * **Chronic Myeloid Leukemia (CML):** Basophilia is a hallmark of CML. In fact, a rising basophil count in a known CML patient is a significant clinical sign indicating **disease progression** toward the Accelerated Phase or Blast Crisis. **Clinical Pearls for NEET-PG:** * **Basophilia** is most commonly associated with Myeloproliferative Neoplasms (CML, Polycythemia Vera). * **Cushing’s Mnemonic:** Steroids cause an increase in "Neutrophils" but a decrease in "B.E.L." (**B**asophils, **E**osinophils, and **L**ymphocytes). * **High-Yield Fact:** Basophils contain coarse granules rich in **Heparin and Histamine**. Their membranes express **IgE receptors (FcεRI)**, playing a crucial role in Type I Hypersensitivity reactions. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, p. 1127.

Q: What is the minimum percentage of blasts in the peripheral blood or bone marrow required for the diagnosis of Acute Myeloid Leukemia?

20%. ### Explanation The diagnosis of **Acute Myeloid Leukemia (AML)** is primarily based on the quantification of myeloblasts in the bone marrow or peripheral blood. **1. Why 20% is the Correct Answer:** According to the **WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues**, the threshold for diagnosing AML is a blast count of **≥20%**. This criterion distinguishes acute leukemia from Myelodysplastic Syndromes (MDS), where the blast count is typically lower [1]. * **Exception (High-Yield):** If specific genetic abnormalities are present—namely **t(8;21), inv(16), or t(15;17)**—the diagnosis of AML can be made even if the blast count is **less than 20%**. **2. Why Other Options are Incorrect:** * **A (8%) & B (15%):** These percentages fall within the range of **Myelodysplastic Syndromes (MDS)**. Specifically, MDS with excess blasts (MDS-EB-1) involves 5-9% blasts, and MDS-EB-2 involves 10-19% blasts [1]. * **D (25%):** Historically, the **FAB (French-American-British) classification** required a blast count of **≥30%** for a diagnosis of AML. The 25% mark is an arbitrary number that does not align with current or historical diagnostic thresholds. **3. Clinical Pearls for NEET-PG:** * **Auer Rods:** Their presence in blasts is pathognomonic for myeloid lineage (AML), even if the blast count is low. * **MPO (Myeloperoxidase):** The most specific histochemical stain for AML. * **Flow Cytometry:** Essential for lineage markers; **CD33 and CD13** are common pan-myeloid markers. * **Pure Erythroid Leukemia:** A rare subtype where blasts are ≥80% of the marrow cells. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 613-614.

Q: Falsely elevated hemoglobin is seen in which condition?

Burns. **Explanation:** The correct answer is **Burns**. In patients with extensive burns, there is a massive shift of fluid from the intravascular compartment to the interstitial space due to increased capillary permeability. This leads to **hemoconcentration** (a decrease in plasma volume relative to red cell mass) [1]. Consequently, the concentration of hemoglobin appears falsely elevated on a Complete Blood Count (CBC), even though the total red cell mass has not increased. This is a form of "relative polycythemia" [1]. **Analysis of Incorrect Options:** * **Chronic Renal Failure (CRF):** This typically causes **anemia** (decreased hemoglobin) due to the deficiency of Erythropoietin (EPO) produced by the peritubular interstitial cells of the kidney. * **Paroxysmal Nocturnal Hemoglobinuria (PNH) & Paroxysmal Cold Hemoglobinuria (PCH):** Both are types of hemolytic anemias. Intravascular hemolysis leads to a **decrease** in hemoglobin levels. While free hemoglobin is present in the plasma (hemoglobinemia), the automated analyzers measure total hemoglobin, which will be low due to red cell destruction. **High-Yield Clinical Pearls for NEET-PG:** * **Other causes of falsely elevated Hb:** Hyperlipidemia (lipemic index), severe leukocytosis (>100,000/µL), and presence of paraproteins (e.g., Multiple Myeloma) which increase turbidity in the spectrophotometric assay. * **Rule of Three:** In a normal CBC, Hemoglobin x 3 ≈ Hematocrit (Hct). If this ratio is significantly disturbed, suspect an analytical error or hemoconcentration. * **Management Tip:** In burn patients, the elevated hematocrit/hemoglobin is used as a guide for fluid resuscitation (Parkland Formula) rather than a sign of true polycythemia. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 663-664.

Question 1

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 2

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 3

Bone marrow findings in AL amyloidosis typically include:

Accepted Answer

Plasmacytosis

Answer

Granulomatous reaction

Answer

Fibrosis

Answer

Giant cell formation

Question 4

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 5

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

Question 6

In Beta thalassemia, what is the typical alteration in globin chain production?

Accepted Answer

Decrease in beta chain, increase in alpha chain

Answer

Increase in beta chain, decrease in alpha chain

Answer

Decrease in beta chain, decrease in alpha chain

Answer

Increase in beta chain, increase in alpha chain

Question 7

Basophils are decreased in which of the following conditions?

Accepted Answer

Cushing's syndrome

Answer

Polycythemia

Answer

Basophilic leukemia

Answer

Chronic myeloid leukemia (CML)

Question 8

A 32-year-old man has mild anemia diagnosed on routine testing. His clinical examination is normal, and a blood film reveals some target cells with some red cells having intraerythrocytic crystals. For the above patient with a hemoglobin abnormality, select the most likely diagnosis?

Accepted Answer

HbC disease

Answer

beta-thalassemia major

Answer

HbH disease

Answer

sickle cell disease

Question 9

What is the minimum percentage of blasts in the peripheral blood or bone marrow required for the diagnosis of Acute Myeloid Leukemia?

Accepted Answer

20%

Answer

8%

Answer

15%

Answer

25%

Question 10

Falsely elevated hemoglobin is seen in which condition?

Accepted Answer

Burns

Answer

Chronic renal failure

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Paroxysmal cold hemoglobinuria

Hematopathology — MCQs

Hematopathology — MCQs

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