Hematopathology Practice Questions

Q: Reduced platelet count is found in all the conditions except:

Von Willebrand disease. **Explanation:** The correct answer is **Von Willebrand disease (vWD)** because it is primarily a **qualitative platelet disorder** (defect in platelet adhesion) rather than a quantitative one [1]. In most types of vWD (Type 1 and Type 2), the platelet count remains **normal**. The pathology lies in the deficiency or dysfunction of Von Willebrand Factor (vWF), which acts as a bridge between platelets and the subendothelial collagen [3]. **Analysis of Options:** * **Disseminated Intravascular Coagulation (DIC):** Characterized by widespread activation of the coagulation cascade, leading to the **consumption** of platelets and clotting factors [2]. Thrombocytopenia is a hallmark finding. * **Aplastic Anemia:** A bone marrow failure syndrome where there is peripheral pancytopenia due to marrow hypoplasia. Reduced production leads to a **low platelet count**. * **Acute Myelocytic Leukemia (AML):** Malignant proliferation of blast cells in the bone marrow "crowds out" normal hematopoiesis (myelophthisic effect), resulting in **decreased production** of mature platelets. **High-Yield Clinical Pearls for NEET-PG:** * **Exception in vWD:** **Type 2B vWD** is a unique variant where "gain-of-function" mutations cause increased binding of vWF to platelets, leading to their clearance and **mild thrombocytopenia** [2]. However, as a general rule for exams, vWD presents with a normal platelet count. * **Lab Findings in vWD:** Increased Bleeding Time (BT), normal or increased aPTT (due to low Factor VIII levels), and **abnormal Ristocetin cofactor assay** (the gold standard for diagnosis) [3]. * **Treatment of Choice:** Desmopressin (DDAVP) for Type 1; vWF concentrate for Type 3 [3].

Q: Dohle bodies can be seen in which of the following conditions?

Sepsis. **Explanation:** **Dohle bodies** are small, light blue-grey, oval inclusions found in the periphery of the cytoplasm of **neutrophils**. They represent remnants of **rough endoplasmic reticulum (RER)** arranged in parallel rows. **Why Sepsis is Correct:** Dohle bodies are a hallmark of **toxic granulation** and "left shift" seen in states of intense inflammation or cytokine stress. In **sepsis** (or severe bacterial infections), there is accelerated granulopoiesis. The rapid maturation of neutrophils in the bone marrow leads to defects in cytoplasmic maturation, causing fragments of RER to persist in the mature cell. They are frequently seen alongside toxic granules and cytoplasmic vacuoles. **Analysis of Incorrect Options:** * **Plasma cell myeloma:** This is a malignancy of plasma cells characterized by Russell bodies (intracellular Ig) or Mott cells, not neutrophil inclusions. * **Chronic granulomatous disease (CGD):** This is a functional defect of the NADPH oxidase enzyme. Neutrophils appear morphologically normal under light microscopy but fail the Nitroblue Tetrazolium (NBT) test. * **Rheumatoid arthritis:** While a chronic inflammatory state, it does not typically manifest with Dohle bodies unless there is a concurrent acute systemic infection or Felty’s syndrome. **High-Yield Clinical Pearls for NEET-PG:** * **Composition:** Dohle bodies consist of **Ribosomal RNA/Rough ER**. * **Differential Diagnosis:** Apart from sepsis/infection, they are classically seen in **May-Hegglin Anomaly** (associated with giant platelets and thrombocytopenia), burns, and normal pregnancy. * **Stain:** They are best visualized using **Romanowsky stains** (Leishman or Giemsa). * **Toxic Triad:** In severe infection, look for the triad of **Dohle bodies, Toxic granulations, and Cytoplasmic vacuolation** in neutrophils.

Q: A patient presents with bleeding due to platelet function defects. Which of the following features is typically observed?

Normal platelet count and increased bleeding time. **Explanation:** The core concept in this question lies in distinguishing between **platelet quantity** and **platelet quality**. 1. **Why Option B is correct:** The question specifies a "platelet function defect" (qualitative defect). In such conditions (e.g., Glanzmann thrombasthenia or Bernard-Soulier syndrome), the bone marrow produces a sufficient number of platelets, so the **platelet count remains normal**. However, because these platelets cannot adhere or aggregate properly to form a primary hemostatic plug, the **Bleeding Time (BT) is increased**. BT is the clinical gold standard for assessing platelet function and the formation of the primary platelet plug. 2. **Why the other options are incorrect:** * **Option A:** A normal BT would imply functional platelets, which contradicts the diagnosis of a function defect. * **Option C:** This describes **Thrombocytopenia** (quantitative defect). While BT would be increased here, the primary pathology in the question is functional, not numerical. * **Option D:** A decreased bleeding time is clinically rare and usually associated with hypercoagulable states, not bleeding disorders. **NEET-PG High-Yield Pearls:** * **Bleeding Time (BT):** Measures primary hemostasis (Platelets + Vessel wall). Normal: 2–7 minutes. * **Glanzmann Thrombasthenia:** Deficiency of **GPIIb/IIIa** (failure of aggregation). * **Bernard-Soulier Syndrome:** Deficiency of **GPIb** (failure of adhesion); characterized by **giant platelets** and mild thrombocytopenia. * **Von Willebrand Disease (vWD):** The most common inherited bleeding disorder; causes increased BT because vWF is required for platelet adhesion. * **Drug Alert:** Aspirin irreversibly inhibits COX-1, leading to a functional defect (increased BT) despite a normal platelet count.

Q: Anemia, splenomegaly, and structural defects are typically seen in which of the following conditions?

Thalassemia major. **Explanation:** **Thalassemia Major** (also known as Cooley’s Anemia) is the correct answer because it represents the most severe form of beta-thalassemia, characterized by a near-total lack of beta-globin chain synthesis. This leads to a triad of clinical features: 1. **Severe Anemia:** Ineffective erythropoiesis and hemolysis of defective red blood cells lead to profound microcytic hypochromic anemia, usually manifesting within the first 6–9 months of life. 2. **Splenomegaly:** Massive enlargement occurs due to both extramedullary hematopoiesis and the sequestration of damaged RBCs. 3. **Structural Defects:** To compensate for chronic anemia, the bone marrow undergoes massive expansion. This results in characteristic skeletal changes such as **"Chipmunk facies"** (prominent maxilla, malocclusion of teeth) and a **"Hair-on-end"** appearance on skull X-rays. **Incorrect Options:** * **Thalassemia Minor:** Usually asymptomatic or presents with mild anemia. Patients do not exhibit significant splenomegaly or skeletal deformities. * **Mediterranean Anemia:** This is a historical synonym for Thalassemia. However, in the context of this question, "Thalassemia Major" is the specific clinical entity that defines the severe phenotype described. * **Polycythemia:** This condition involves an *increase* in red blood cell mass, which is the physiological opposite of anemia. **NEET-PG High-Yield Pearls:** * **Target Cells:** Commonly seen on peripheral smears of Thalassemia patients. * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia. * **Hb Electrophoresis:** In Thalassemia Major, there is a marked increase in **HbF** and absence/reduction of HbA. * **Iron Overload:** The primary cause of mortality in these patients (due to repeated transfusions), leading to restrictive cardiomyopathy and endocrinopathies.

Q: Examination of a lymph node from the neck of a 26-year-old man with bilateral cervical lymphadenopathy reveals total effacement of nodal architecture, and at higher power, the characteristic cell shown below. Which of the following additional studies is best suited for confirmation of diagnosis in this case?

Immunohistochemistry. ***Immunohistochemistry*** - **CD15** and **CD30** positivity is the **gold standard** for confirming **Hodgkin's lymphoma** with **Reed-Sternberg cells**, which show characteristic **owl's eye** appearance with large nucleoli. - **Immunohistochemistry** provides definitive identification of the **Reed-Sternberg cell phenotype** and distinguishes it from other large cell lymphomas through specific marker expression. *Serum protein electrophoresis* - Used primarily to detect **monoclonal gammopathy** and **multiple myeloma** through identification of **M-protein** spikes. - Not specific for **lymphoma diagnosis** and cannot identify the characteristic **Reed-Sternberg cell** markers needed for Hodgkin's lymphoma confirmation. *Flow cytometry* - **Reed-Sternberg cells** are typically **too large** and **fragile** to be effectively analyzed by flow cytometry, often getting lost during sample preparation. - More suitable for **B-cell** and **T-cell lymphomas** where smaller, intact lymphocytes can be analyzed for surface markers. *Gene rearrangement studies* - Primarily used to detect **clonal B-cell** or **T-cell populations** in **non-Hodgkin lymphomas** through **immunoglobulin** or **T-cell receptor** gene rearrangements. - **Hodgkin's lymphoma** diagnosis relies on **morphology** and **immunophenotype** rather than specific gene rearrangement patterns.

Q: A 28-year-old woman with sickle cell anemia marries a 31-year-old man with no abnormality. What are the respective probabilities of having (i) sickle cell disease and (ii) sickle cell trait in their offspring?

0% and 100%. ### Explanation **1. Understanding the Genetics (The Correct Answer)** Sickle cell anemia is an **autosomal recessive** disorder [1]. To determine the offspring's risk, we must identify the parental genotypes: * **Mother:** Has sickle cell disease (SCD), meaning her genotype is **SS** [1]. * **Father:** Has no abnormality (Normal), meaning his genotype is **AA**. Using a Punnett square for an **SS × AA** cross: * All possible offspring will receive an 'S' allele from the mother and an 'A' allele from the father. * **Genotype of all offspring:** **AS** (Sickle Cell Trait). * **Probability of Disease (SS):** 0% * **Probability of Trait (AS):** 100% **2. Why Other Options are Incorrect** * **Option B (25% and 50%):** This occurs in a cross between two carriers (**AS × AS**). In that case, there is a 25% chance of SS, 50% chance of AS, and 25% chance of AA. * **Option C (50% and 25%):** This does not follow standard Mendelian inheritance patterns for a single-gene recessive trait. * **Option D (25% and 25%):** This distribution is incorrect for any combination of these specific parental genotypes. **3. NEET-PG High-Yield Clinical Pearls** * **Molecular Basis:** A point mutation (missense) in the β-globin gene on **Chromosome 11**, where **Glutamic acid** is replaced by **Valine** at the 6th position [1]. * **Sickle Cell Trait (AS):** Usually asymptomatic but provides a selective advantage against *Plasmodium falciparum* malaria [1]. * **Screening:** Solubility tests (e.g., Sodium dithionite) are used for screening, while **Hb Electrophoresis** or HPLC is the gold standard for diagnosis. * **Metabisulfite Test:** Induces sickling in both trait (AS) and disease (SS) by reducing oxygen tension [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 598-599.

Question 1

Reduced platelet count is found in all the conditions except:

Accepted Answer

Von Willebrand disease

Answer

Disseminated intravascular coagulation

Answer

Aplastic anemia

Answer

Acute myelocytic leukemia

Question 2

Bence Jones proteins are found in all of the following conditions except?

Accepted Answer

Reactive plasmacytosis

Answer

Multiple myeloma

Answer

Monoclonal gammopathy of undetermined significance

Answer

Waldenstroms macroglobulinemia

Question 3

Which among the following is not a feature of follicular lymphoma?

Accepted Answer

(11;14) translocation

Answer

BCL2 over expression

Answer

BCL6 rearrangement

Answer

CD5 negative

Question 4

All are examples of microangiopathic hemolytic anemia except?

Accepted Answer

Immune thrombocytopenic purpura (ITP)

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Hemolytic uremic syndrome (HUS)

Answer

Disseminated intravascular coagulation (DIC)

Question 5

Dohle bodies can be seen in which of the following conditions?

Accepted Answer

Sepsis

Answer

Plasma cell myeloma

Answer

Chronic granulomatous disease

Answer

Rheumatoid arthritis

Question 6

A patient presents with bleeding due to platelet function defects. Which of the following features is typically observed?

Accepted Answer

Normal platelet count and increased bleeding time

Answer

Normal platelet count and normal bleeding time

Answer

Decreased platelet count and increased bleeding time

Answer

Normal platelet count and decreased bleeding time

Question 7

Anemia, splenomegaly, and structural defects are typically seen in which of the following conditions?

Accepted Answer

Thalassemia major

Answer

Thalassemia minor

Answer

Mediterranean anemia

Answer

Polycythemia

Question 8

Examination of a lymph node from the neck of a 26-year-old man with bilateral cervical lymphadenopathy reveals total effacement of nodal architecture, and at higher power, the characteristic cell shown below. Which of the following additional studies is best suited for confirmation of diagnosis in this case?

Accepted Answer

Immunohistochemistry

Answer

Serum protein electrophoresis

Answer

Flow cytometry

Answer

Gene rearrangement studies

Question 9

A 28-year-old woman with sickle cell anemia marries a 31-year-old man with no abnormality. What are the respective probabilities of having (i) sickle cell disease and (ii) sickle cell trait in their offspring?

Accepted Answer

0% and 100%

Answer

25% and 50%

Answer

50% and 25%

Answer

25% and 25%

Question 10

If both parents have sickle cell anemia, what is the likelihood of their offspring inheriting the disease?

Accepted Answer

100%

Answer

10%

Answer

25%

Answer

50%

Hematopathology — MCQs

Hematopathology — MCQs

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