Hematology Practice Questions

Q: What is true about hemochromatosis?

Mutation of C282Y. **Explanation:** Hereditary Hemochromatosis (HH) is an autosomal recessive disorder characterized by excessive intestinal iron absorption leading to multiorgan deposition [1]. **1. Why Option A is correct:** The most common cause of HH is a mutation in the **HFE gene** located on **Chromosome 6** [1]. The **C282Y mutation** (substitution of tyrosine for cysteine at position 282) is the most frequent genetic defect (found in >80% of cases). This mutation disrupts the interaction between the HFE protein and the transferrin receptor, leading to inappropriately low levels of **Hepcidin**, the master regulator of iron homeostasis [2]. **2. Why the other options are incorrect:** * **Option B:** While the genetic inheritance is equal, the clinical disease is **more common in men** (ratio ~5:1 to 10:1). Women are protected for decades due to physiological iron loss through menstruation and pregnancy. * **Option C:** **Phlebotomy** is the **gold standard treatment** [3]. It is highly effective, inexpensive, and reduces the risk of cirrhosis and hepatocellular carcinoma if started early. * **Option D:** HH has **low clinical penetrance**. While many individuals are homozygous for the C282Y mutation, only a small fraction (especially in women) develop the full clinical triad of "Bronze Diabetes" [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** Skin hyperpigmentation (bronzing), Diabetes mellitus, and Cirrhosis [1]. * **Early Sign:** Arthropathy (specifically involving the 2nd and 3rd MCP joints with "hook-like" osteophytes). * **Screening:** Transferrin saturation (>45%) is the best initial screening test; Ferritin levels reflect total body stores. * **Diagnosis:** MRI (T2*) can quantify liver iron; Genetic testing has largely replaced liver biopsy for diagnosis [3]. * **Cardiac:** Most common manifestation is restrictive cardiomyopathy (though dilated can occur).

Q: Which of the following is NOT considered a blood component?

Whole blood. In transfusion medicine, it is crucial to distinguish between **blood products** and **blood components**. [1] **Explanation of the Correct Answer:** **Whole blood (Option A)** is classified as a **blood product**, not a component. A blood component is a therapeutic constituent of blood (e.g., erythrocytes, leukocytes, platelets, or plasma) that is separated from whole blood using physical means like centrifugation or filtration. [1] Since whole blood contains all these elements together in their original state (plus an anticoagulant), it is the source material from which components are derived, rather than being a component itself. **Analysis of Incorrect Options:** * **Platelet concentrate (Option B):** A component prepared by centrifugation of whole blood or via apheresis. [1] It is used primarily in thrombocytopenia. * **Fresh frozen plasma (Option C):** The liquid portion of blood separated and frozen within 8 hours of collection. It contains all coagulation factors. * **RBC concentrate (Option D):** Also known as Packed Red Blood Cells (PRBCs), this component is prepared by removing most of the plasma from whole blood to increase oxygen-carrying capacity without volume overload. [1] **High-Yield Clinical Pearls for NEET-PG:** * **Component Separation:** One unit of whole blood can save up to three lives by being separated into PRBCs, Platelets, and FFP/Cryoprecipitate. * **Storage:** PRBCs are stored at **2–6°C** for up to 35–42 days; Platelets are stored at **20–24°C** with constant agitation for 5 days; FFP is stored at **-18°C or colder** for up to 1 year. * **Cryoprecipitate:** This is a component derived from FFP, rich in **Factor VIII, Fibrinogen, von Willebrand factor, and Factor XIII.**

Q: What is the most malignant form of Non-Hodgkin Lymphoma (NHL)?

Diffuse large cell lymphoma. **Explanation:** The classification of Non-Hodgkin Lymphoma (NHL) is primarily based on clinical behavior, categorized into **Indolent (Low-grade)** and **Aggressive (High-grade)** types [1]. **Why Diffuse Large B-Cell Lymphoma (DLBCL) is correct:** DLBCL is the most common subtype of NHL and is classified as an **aggressive (high-grade)** lymphoma [1]. It is characterized by rapidly enlarging lymph nodes and systemic "B" symptoms [1]. While it is highly malignant and fatal if left untreated, it is also potentially curable with intensive chemotherapy (e.g., R-CHOP regimen) because high-grade cells have a high mitotic index, making them more sensitive to cytotoxic drugs [1]. **Analysis of Incorrect Options:** * **B. Small cell lymphocytic lymphoma (SLL):** This is the tissue equivalent of Chronic Lymphocytic Leukemia (CLL) [1]. It is an **indolent** lymphoma. While it is generally incurable, it has a slow, protracted clinical course [1]. * **C. Follicular predominantly small cleaved cell lymphoma:** This is a Grade 1/2 Follicular Lymphoma. It is the classic example of an **indolent** lymphoma with a median survival of many years, though it can eventually transform into a more aggressive type (Richter’s transformation). * **D. Large cell follicular lymphoma:** This corresponds to Grade 3 Follicular Lymphoma. While more aggressive than small-cell types, it typically follows a less rapid course than the purely **diffuse** large cell variant. **NEET-PG High-Yield Pearls:** * **Most common NHL overall:** Diffuse Large B-Cell Lymphoma (DLBCL) [1]. * **Most common indolent NHL:** Follicular Lymphoma [1]. * **Most aggressive (fastest growing) NHL:** Burkitt Lymphoma (associated with c-myc translocation) [1]. * **Key distinction:** Indolent lymphomas are "slow but incurable," whereas aggressive lymphomas are "fast but potentially curable" [1].

Q: A 23-year-old man of northern European lineage presents with anemia. His father and paternal aunt had a similar illness that was treated successfully by splenectomy. His peripheral blood smear is similar to that shown in the illustration. Which of the following additional abnormalities is expected?

Polychromatophilic erythrocytes on peripheral blood smear. ***Polychromatophilic erythrocytes on peripheral blood smear*** - **Compensatory reticulocytosis** occurs in response to chronic hemolysis in hereditary spherocytosis, appearing as **polychromatophilic cells** on blood smear. - These immature red cells indicate active **bone marrow response** to replace hemolyzed spherocytes. *Bilirubinuria* - **Unconjugated bilirubin** from extravascular hemolysis is **protein-bound** and cannot be filtered by kidneys, so no bilirubinuria occurs. - Only **conjugated bilirubin** can appear in urine, which is not elevated in hereditary spherocytosis. *High mean corpuscular volume* - Hereditary spherocytosis typically shows **normal to slightly decreased MCV** due to membrane loss during splenic conditioning. - The characteristic finding is **increased MCHC** (mean corpuscular hemoglobin concentration), not increased MCV. *Increased direct (conjugated) serum bilirubin* - **Extravascular hemolysis** in hereditary spherocytosis produces **unconjugated hyperbilirubinemia**, not conjugated. - **Conjugated bilirubin elevation** suggests hepatocellular dysfunction or biliary obstruction, not hemolytic anemia.

Q: All of the following can cause hemolytic anemia except?

Propranolol. **Explanation:** The correct answer is **Propranolol**. Hemolytic anemia is a condition where red blood cells (RBCs) are destroyed faster than they can be produced [4]. This can be triggered by drugs through two primary mechanisms: **Drug-induced Immune Hemolytic Anemia (DIIHA)** [4] or oxidative stress in individuals with **G6PD deficiency** [2], [3]. * **Propranolol (Option D):** This is a non-selective beta-blocker used for hypertension, arrhythmias, and prophylaxis of migraines. It is **not** associated with hemolysis. It does not induce autoantibody production against RBCs nor does it cause oxidative damage. **Why the other options are incorrect:** * **Isoniazid (Option A):** A primary anti-tubercular drug known to cause DIIHA via the "hapten mechanism," where the drug binds to the RBC membrane, stimulating antibody production. * **Rifampicin (Option B):** Another key anti-tubercular agent that can cause acute intravascular hemolysis through the formation of "immune complexes" (innocent bystander mechanism) [4]. * **Co-trimoxazole (Option C):** This combination (Sulfamethoxazole + Trimethoprim) is a classic trigger for hemolysis, especially in patients with **G6PD deficiency**, due to its oxidant properties [2], [3]. **High-Yield Clinical Pearls for NEET-PG:** * **G6PD Deficiency Triggers:** Remember the mnemonic **"AAA"**: **A**ntimalarials (Primaquine) [1], **A**ntibiotics (Sulfonamides, Nitrofurantoin), and **A**ntipyretics (NSAIDs, though less common). * **Methyldopa:** The most common drug associated with a positive Direct Coombs Test and warm-type autoimmune hemolytic anemia. * **Drug-induced Hemolysis** should always be suspected in a patient presenting with sudden onset jaundice, dark urine (hemoglobinuria), and a drop in hemoglobin after starting a new medication [1].

Q: A 56-year-old female presents with headache, dizziness, generalized itching, especially after hot showers, and intense burning in her hands and feet. She reports relief with aspirin. Physical examination reveals splenomegaly and elevated blood pressure. Investigations show HB: 20.1 g/dl, Hematocrit: 60%, WBC: 15800, Platelet count: 500,000/µl, low spO2: 98%, and increased LAP. Which of the following is the most common mutation seen in the condition described?

JAK2. **Explanation:** The clinical presentation is a classic case of **Polycythemia Vera (PV)**, a myeloproliferative neoplasm (MPN). The patient exhibits the hallmark "triad" of PV: **panmyelosis** (elevated RBCs, WBCs, and platelets), **aquagenic pruritus** (itching after hot showers), and **erythromelalgia** (burning pain in extremities relieved by aspirin) [1]. Splenomegaly and low serum erythropoietin (implied by high Hb with normal SpO2) further support this diagnosis. **1. Why JAK2 is correct:** The **JAK2 V617F mutation** (located on exon 14) is the molecular hallmark of Polycythemia Vera, present in **>95% of cases** [1]. A small minority of the remaining cases (~3-4%) carry a mutation in **JAK2 exon 12**. This mutation leads to constitutive activation of the Janus Kinase 2 signaling pathway, causing erythropoietin-independent proliferation of myeloid lineages. **2. Why other options are incorrect:** * **CALR (Calreticulin):** Mutations are common in Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF) but are **never** seen in PV. * **RAS:** Mutations are associated with various solid tumors and certain leukemias (like CMML) but are not the primary drivers of MPNs. * **MYC:** This oncogene is associated with aggressive lymphomas, most notably Burkitt Lymphoma (t(8;14)). **Clinical Pearls for NEET-PG:** * **Diagnostic Criteria:** PV requires meeting 3 major criteria (High Hb/Hct, BM hypercellularity, JAK2 mutation) OR 2 major and 1 minor (Low EPO level). * **LAP Score:** Leukocyte Alkaline Phosphatase (LAP) is **increased** in PV and Leukemoid reaction, but **decreased** in CML. * **Treatment:** Phlebotomy (target Hct <45%) and low-dose aspirin are first-line. Hydroxyurea is used for high-risk patients.

Q: A decrease in serum iron and a decrease in total iron-binding capacity (TIBC) is seen in which condition?

Anemia of chronic disease. **Explanation:** The correct answer is **Anemia of Chronic Disease (ACD)**. The hallmark of ACD is the sequestration of iron within the reticuloendothelial system (macrophages), primarily driven by the inflammatory cytokine **IL-6**. IL-6 stimulates the liver to produce **Hepcidin**, which degrades ferroportin (the iron export channel). This leads to: 1. **Decreased Serum Iron:** Iron is trapped inside cells and cannot be released into the plasma. 2. **Decreased TIBC:** TIBC is a surrogate measure of Transferrin. In chronic inflammation, Transferrin (a negative acute-phase reactant) decreases. Furthermore, the body downregulates TIBC to limit iron availability to potential pathogens. **Analysis of Incorrect Options:** * **Iron Deficiency Anemia (IDA):** Characterized by **decreased** serum iron but **increased** TIBC. The body increases Transferrin production to maximize the transport of any available iron. * **Thalassemia:** Typically presents with **normal to increased** serum iron and TIBC, as it is a defect in globin chain synthesis, not iron metabolism. * **Sideroblastic Anemia:** Characterized by iron overload. Serum iron is **increased**, and TIBC is usually **normal or decreased** due to saturation. **NEET-PG High-Yield Pearls:** * **Ferritin:** The most crucial differentiator. Ferritin is **low** in IDA but **normal or high** in ACD (as it is a positive acute-phase reactant). * **Soluble Transferrin Receptor (sTfR):** Elevated in IDA but **normal** in ACD. This is the most sensitive test to distinguish the two when they coexist. * **Mentzer Index (MCV/RBC):** 13 suggests IDA.

Q: All except one are unfavourable signs in early Hodgkin's Lymphoma?

Stage A. In Hodgkin’s Lymphoma (HL), patients are categorized into **favorable** or **unfavorable** groups based on prognostic factors to determine the intensity of chemotherapy and radiotherapy. [1] ### **Why "Stage A" is the Correct Answer** In the Ann Arbor staging system, the suffix **"A"** denotes the **absence** of systemic symptoms (fever, night sweats, or weight loss). [1] The absence of these symptoms is a **favorable** prognostic sign. Conversely, the suffix **"B"** (presence of B-symptoms) is a well-established unfavorable factor. [1] ### **Analysis of Unfavorable Signs (Incorrect Options)** The European Organisation for Research and Treatment of Cancer (EORTC) and the German Hodgkin Study Group (GHSG) define unfavorable early-stage HL (Stage I-II) using the following criteria: * **Elevated ESR (Option A):** An ESR >50 mm/hr (without B-symptoms) or >30 mm/hr (with B-symptoms) is a classic unfavorable marker. * **Extranodal disease (Option B):** Presence of "E" lesions (localized extranodal involvement) indicates a higher risk profile. * **Number of Regions (Option D):** Involvement of **three or more** (EORTC) or **four or more** (GHSG) nodal areas is considered unfavorable. ### **Clinical Pearls for NEET-PG** * **Bulky Disease:** Defined as a mediastinal mass >1/3 of the maximum intrathoracic diameter or any mass >10 cm. This is one of the most significant unfavorable factors. [1] * **Age:** Age ≥50 years is often considered an unfavorable factor in early-stage HL. * **Mixed Cellularity & Lymphocyte Depleted:** These histological subtypes generally carry a worse prognosis compared to Lymphocyte Predominant or Nodular Sclerosis. * **Treatment:** Early favorable HL is typically treated with 2 cycles of ABVD + 20 Gy Involved-Site Radiation (ISRT), whereas unfavorable cases require 4 cycles + 30 Gy ISRT.

Question 1

What is true about hemochromatosis?

Accepted Answer

Mutation of C282Y

Answer

More common in women than in men

Answer

Cannot be treated with phlebotomy

Answer

Fully penetrant

Question 2

Which of the following is NOT considered a blood component?

Accepted Answer

Whole blood

Answer

Platelet concentrate

Answer

Fresh frozen plasma

Answer

RBC concentrate

Question 3

What is the most malignant form of Non-Hodgkin Lymphoma (NHL)?

Accepted Answer

Diffuse large cell lymphoma

Answer

Small cell lymphocytic lymphoma

Answer

Follicular predominantly small cleaved cell lymphoma

Answer

Large cell follicular lymphoma

Question 4

A 23-year-old man of northern European lineage presents with anemia. His father and paternal aunt had a similar illness that was treated successfully by splenectomy. His peripheral blood smear is similar to that shown in the illustration. Which of the following additional abnormalities is expected?

Accepted Answer

Polychromatophilic erythrocytes on peripheral blood smear

Answer

Bilirubinuria

Answer

High mean corpuscular volume

Answer

Increased direct (conjugated) serum bilirubin

Question 5

All of the following can cause hemolytic anemia except?

Accepted Answer

Propranolol

Answer

Isoniazid

Answer

Rifampicin

Answer

Co-trimoxazole

Question 6

A 56-year-old female presents with headache, dizziness, generalized itching, especially after hot showers, and intense burning in her hands and feet. She reports relief with aspirin. Physical examination reveals splenomegaly and elevated blood pressure. Investigations show HB: 20.1 g/dl, Hematocrit: 60%, WBC: 15800, Platelet count: 500,000/µl, low spO2: 98%, and increased LAP. Which of the following is the most common mutation seen in the condition described?

Accepted Answer

JAK2

Answer

CALR

Answer

RAS

Answer

MYC

Question 7

A decrease in serum iron and a decrease in total iron-binding capacity (TIBC) is seen in which condition?

Accepted Answer

Anemia of chronic disease

Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Sideroblastic anemia

Question 8

All of the following are true regarding DVT except?

Accepted Answer

May lead to lung infarction

Answer

Pulmonary emboli is the immediate risk

Answer

Associated with deficiency of protein C

Answer

Prior history of DVT is an important risk factor

Question 9

All except one are unfavourable signs in early Hodgkin's Lymphoma?

Accepted Answer

Stage A

Answer

Elevated ESR

Answer

Extranodal disease

Answer

Four or more regions involved

Question 10

A patient presents with a platelet count of 700x10^9/L with abnormalities in size, shape, and granularity of platelets. The WBC count is 12x10^9/L, hemoglobin is 11g/dL, and the Philadelphia chromosome is absent. What is the most likely diagnosis?

Accepted Answer

Essential thrombocythemia

Answer

Polycythemia vera

Answer

Chronic myeloid leukemia

Answer

Leukemoid reaction

Hematology — MCQs

Hematology — MCQs

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