Hematology Practice Questions

Q: Which of the following is the first symptom of iron deficiency anemia?

Reduced ferritin. ### Explanation Iron deficiency anemia (IDA) develops in a sequential manner, progressing through three distinct stages [3]. Understanding these stages is crucial for identifying the earliest markers of the disease. **1. Why Reduced Ferritin is Correct:** The first stage of iron deficiency is **Iron Depletion**. In this stage, the body’s iron stores are exhausted to maintain normal serum iron levels for erythropoiesis [3]. **Serum Ferritin** is the most sensitive and earliest laboratory marker because it directly reflects the body's total iron stores. When ferritin levels drop, it indicates that the "reserve tank" is emptying, even though circulating iron and hemoglobin levels remain normal. **2. Why the Other Options are Incorrect:** * **Low iron concentration in blood (Serum Iron):** This occurs during the second stage, **Iron Deficient Erythropoiesis**. Serum iron only drops after the storage iron (ferritin) is significantly depleted [2]. * **Reduced Hemoglobin (Hb):** This is a late manifestation occurring in the third stage, **Iron Deficiency Anemia**. Hb only falls after iron stores are gone and the supply to the bone marrow is insufficient to produce mature red cells [1], [2]. * **Reduced PCV (Packed Cell Volume):** Like hemoglobin, PCV (Hematocrit) decreases only in the final stage of the disease [2]. **Clinical Pearls for NEET-PG:** * **Sequence of Depletion:** Ferritin ↓ → Serum Iron ↓ & TIBC ↑ → Hemoglobin ↓ (Microcytic Hypochromic). * **Best Screening Test:** Serum Ferritin (Note: It is an acute-phase reactant, so it may be falsely normal in inflammation). * **Gold Standard Test:** Bone marrow aspiration with **Prussian Blue staining** (demonstrates absent hemosiderin), though rarely done clinically. * **Earliest Morphological Change:** An increase in **Red Cell Distribution Width (RDW)** often precedes the drop in MCV.

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: A 23-year-old female has presented with anemia and jaundice for 2 years. Peripheral smear shows spherocytes. What is the most appropriate investigation to be done?

Coombs test. ### Explanation The clinical presentation of anemia, jaundice, and the presence of **spherocytes** on a peripheral smear indicates **extravascular hemolysis** [1]. In such cases, the primary differential diagnosis lies between **Hereditary Spherocytosis (HS)** and **Autoimmune Hemolytic Anemia (AIHA)**. **1. Why Coombs Test is the Correct Answer:** The most critical step in evaluating spherocytosis is to differentiate between an inherited membrane defect (HS) and an acquired immune-mediated destruction (AIHA). The **Direct Antiglobulin Test (Coombs test)** is the initial investigation of choice because AIHA is more common in young females and is a treatable condition [1]. A positive Coombs test confirms AIHA, while a negative test points toward HS [1]. **2. Why Other Options are Incorrect:** * **Reticulocyte count:** While this will be elevated in any hemolytic anemia, it is a non-specific marker of marrow response and does not help in identifying the specific etiology of spherocytosis [1]. * **Osmotic fragility test (OFT):** Historically used for HS, it is now largely replaced by the **Eosin-5-maleimide (EMA) binding test**. Furthermore, OFT can be positive in both HS and AIHA, making it less specific than the Coombs test for initial differentiation. * **Bone marrow aspiration:** This is generally not indicated in the workup of hemolytic anemias unless a primary bone marrow failure or malignancy is suspected. It would merely show erythroid hyperplasia. **Clinical Pearls for NEET-PG:** * **Spherocytes** lack a central pallor because they have the lowest surface-area-to-volume ratio [1]. * **AIHA vs. HS:** If the question mentions a positive family history or splenomegaly since childhood, think HS. If it mentions a young female (potential SLE association), think AIHA [1]. * **Gold Standard for HS:** The EMA Binding test (Flow cytometry) is now preferred over the Osmotic Fragility Test.

Q: Which of the following is not a type of hypoproliferative anemia?

G6PD deficiency. Anemias are broadly classified based on the reticulocyte count into **hypoproliferative** (low production) and **hyperproliferative** (increased destruction or loss). **Why G6PD Deficiency is the Correct Answer:** G6PD deficiency is a **hemolytic anemia**, which falls under the **hyperproliferative** category [1]. In this condition, the deficiency of the G6PD enzyme leads to inadequate production of NADPH, making red blood cells vulnerable to oxidative stress [2]. This results in episodic hemolysis. Because the bone marrow is healthy, it responds to the low hemoglobin by increasing erythropoiesis, leading to an **elevated reticulocyte count**. **Why the other options are Hypoproliferative:** * **Parvovirus B19:** This virus specifically infects and destroys erythroid progenitor cells in the bone marrow, leading to a transient cessation of RBC production (Pure Red Cell Aplasia). * **Anemia of Chronic Disease (ACD):** Driven by high levels of **Hepcidin**, this condition involves iron sequestration and a blunted erythropoietin response, resulting in decreased RBC production [3]. * **Fanconi Anemia:** This is an inherited form of **Aplastic Anemia** (pancytopenia). It is a primary bone marrow failure syndrome where the marrow cannot produce sufficient cells. **NEET-PG High-Yield Pearls:** * **Reticulocyte Production Index (RPI):** RPI 3% indicates hemolysis or acute blood loss. * **G6PD Hallmark:** Look for **"Heinz bodies"** (denatured hemoglobin) and **"Bite cells"** (degluticytes) on a peripheral smear. * **ACD Hallmark:** Characterized by **High Ferritin** and **Low TIBC** (Total Iron Binding Capacity) [3]. * **Parvovirus B19:** Classically causes "Aplastic Crisis" in patients with pre-existing hemolytic conditions like Sickle Cell Anemia or Hereditary Spherocytosis.

Q: A 20-year-old female presents with features of anemia. Blood tests show Hb of 5 g/dL, MCV of 52 fL, MCHC of 20 g/dL, and PCV of 32%. What is the most likely diagnosis?

Hookworm infection. ### Explanation **1. Analysis of the Correct Answer (Hookworm Infection)** The patient presents with severe anemia (Hb 5 g/dL) characterized by a low **MCV (52 fL)** and low **MCHC (20 g/dL)**. These findings are diagnostic of **Microcytic Hypochromic Anemia**. In the context of a young female in a tropical setting, the most common cause of microcytic hypochromic anemia is **Iron Deficiency Anemia (IDA)** [3]. Hookworms (*Ancylostoma duodenale* and *Necator americanus*) are a leading cause of chronic intestinal blood loss [1]. They attach to the intestinal mucosa and suck blood, leading to a progressive depletion of iron stores [1][2]. This results in the classic laboratory profile seen here: decreased hemoglobin, decreased cell volume (MCV), and decreased hemoglobin concentration within the cells (MCHC). **2. Analysis of Incorrect Options** * **Phenytoin Toxicity:** Phenytoin interferes with folate metabolism, typically causing **Megaloblastic (Macrocytic) Anemia**, where the MCV would be >100 fL. * **Fish Tapeworm Infection (*Diphyllobothrium latum*):** This parasite competes for Vitamin B12 absorption in the ileum, leading to Vitamin B12 deficiency and **Megaloblastic Anemia** (Macrocytic). * **Blind Loop Syndrome:** This condition causes bacterial overgrowth, which leads to Vitamin B12 malabsorption (due to bacterial utilization of B12), resulting in **Megaloblastic Anemia** (Macrocytic). **3. NEET-PG High-Yield Pearls** * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia. * **Hookworm Fact:** *Ancylostoma duodenale* causes more blood loss (0.15–0.2 ml/day) than *Necator americanus* (0.03 ml/day). * **Classic Triad of Hookworm:** Ground itch (dermatitis), Loeffler’s syndrome (pneumonitis), and Iron deficiency anemia [1]. * **MCHC:** This is the most specific indicator of true hypochromia. Normal range is 32–36 g/dL; a value of 20 g/dL is severely low.

Q: A 16-year-old girl has had frequent nosebleeds since childhood. Her gums bleed easily even with routine tooth brushing. She has experienced menorrhagia since menarche at age 13 years. On physical examination, there are no abnormal findings. Laboratory studies show hemoglobin, 14.1 g/dL; hematocrit, 42.5%; MCV, 90 mm3; platelet count, 277,400/mm3; and WBC count, 5920/mm3. Her platelets fail to aggregate in response to ADP, collagen, epinephrine, and thrombin. The ristocetin agglutination test result is normal. There is a deficiency of glycoprotein IIb/IIIa. Prothrombin time is 12 seconds, and partial thromboplastin time is 28 seconds. What is the most likely diagnosis?

Glanzmann thrombasthenia. The clinical presentation of mucosal bleeding (epistaxis, gingival bleeding, and menorrhagia) in a young patient with a **normal platelet count** and **normal PT/PTT** points toward a qualitative platelet disorder rather than a quantitative deficiency or a coagulation factor defect [1]. **Why Glanzmann Thrombasthenia (GT) is correct:** GT is an autosomal recessive disorder caused by a deficiency or dysfunction of **Glycoprotein IIb/IIIa (GPIIb/IIIa)**, a receptor essential for platelet-to-platelet aggregation via fibrinogen bridging [2]. * **Key Diagnostic Feature:** Platelets in GT **fail to aggregate** with all physiological agonists (ADP, collagen, epinephrine, thrombin). * **The Ristocetin Clue:** The **Ristocetin agglutination test is normal** in GT because ristocetin-induced binding depends on GPIb and von Willebrand Factor (vWF), which are intact in this condition. **Why the other options are incorrect:** * **DIC:** Characterized by consumption of platelets (thrombocytopenia) and clotting factors (prolonged PT/PTT), usually in an acutely ill patient [3]. * **ITP:** Presents with isolated **thrombocytopenia** (low platelet count) due to immune destruction [3]; platelet function is typically normal. * **Vitamin C Deficiency (Scurvy):** Causes bleeding due to defective collagen synthesis in blood vessel walls (perifollicular hemorrhages, corkscrew hairs); it does not involve GPIIb/IIIa deficiency. **NEET-PG High-Yield Pearls:** * **Glanzmann Thrombasthenia:** Defect in **Aggregation** (GPIIb/IIIa); Normal Ristocetin test [2]. * **Bernard-Soulier Syndrome:** Defect in **Adhesion** (GPIb-IX-V); **Abnormal** Ristocetin test (not corrected by adding normal plasma); characterized by **Giant Platelets** and mild thrombocytopenia [2], [3]. * **Von Willebrand Disease:** Most common inherited bleeding disorder; **Abnormal** Ristocetin test (corrected by adding normal plasma) [2].

Question 1

All of the following statements about the treatment of typical Chronic Lymphocytic Leukemia (CLL) are true, except:

Accepted Answer

Treatment should be initiated as soon as diagnosis is established.

Answer

Chlorambucil and Fludarabine are commonly used agents.

Answer

Fludarabine is generally preferred in younger patients, while Chlorambucil may be preferred in the elderly.

Answer

Younger patients may be candidates for bone marrow transplantation.

Question 2

Which of the following is the first symptom of iron deficiency anemia?

Accepted Answer

Reduced ferritin

Answer

Low iron concentration in blood

Answer

Reduced hemoglobin

Answer

Reduced PCV

Question 3

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 4

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 5

A young female presents with a history of abdominal pain, headache, nausea, and vomiting, along with sudden loss of movement of the right upper limb. What is the most likely diagnosis?

Accepted Answer

Acute intermittent porphyria

Answer

Conversion disorder

Answer

Migraine

Answer

Right lower limb paralysis

Question 6

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

Question 7

A 23-year-old female has presented with anemia and jaundice for 2 years. Peripheral smear shows spherocytes. What is the most appropriate investigation to be done?

Accepted Answer

Coombs test

Answer

Reticulocyte count

Answer

Osmotic fragility test

Answer

Bone marrow aspiration

Question 8

Which of the following is not a type of hypoproliferative anemia?

Accepted Answer

G6PD deficiency

Answer

Anemia caused by Parvovirus B19 infection

Answer

Anemia of chronic disease/inflammation

Answer

Fanconi anemia

Question 9

A 20-year-old female presents with features of anemia. Blood tests show Hb of 5 g/dL, MCV of 52 fL, MCHC of 20 g/dL, and PCV of 32%. What is the most likely diagnosis?

Accepted Answer

Hookworm infection

Answer

Phenytoin toxicity

Answer

Fish tapeworm infection

Answer

Blind loop syndrome

Question 10

A 16-year-old girl has had frequent nosebleeds since childhood. Her gums bleed easily even with routine tooth brushing. She has experienced menorrhagia since menarche at age 13 years. On physical examination, there are no abnormal findings. Laboratory studies show hemoglobin, 14.1 g/dL; hematocrit, 42.5%; MCV, 90 mm3; platelet count, 277,400/mm3; and WBC count, 5920/mm3. Her platelets fail to aggregate in response to ADP, collagen, epinephrine, and thrombin. The ristocetin agglutination test result is normal. There is a deficiency of glycoprotein IIb/IIIa. Prothrombin time is 12 seconds, and partial thromboplastin time is 28 seconds. What is the most likely diagnosis?

Accepted Answer

Glanzmann thrombasthenia

Answer

Disseminated intravascular coagulation

Answer

Immune thrombocytopenic purpura

Answer

Vitamin C deficiency

Hematology — MCQs

Hematology — MCQs

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