Hematology Practice Questions

Q: A patient presents with fever and altered consciousness. Investigations reveal anemia with fragmented red blood cells, a platelet count of 20,000/mm³, serum creatinine of 3.0 mg/dL, and normal prothrombin time (PT) and activated partial thromboplastin time (aPTT). Which of the following is the most appropriate treatment for this patient?

Plasma exchange therapy. ### Explanation The clinical presentation describes the classic **pentad of Thrombotic Thrombocytopenic Purpura (TTP)**: 1. **Microangiopathic Hemolytic Anemia (MAHA):** Fragmented RBCs (schistocytes) [2]. 2. **Thrombocytopenia:** Low platelet count (20,000/mm³) [3]. 3. **Neurological symptoms:** Altered consciousness. 4. **Renal dysfunction:** Elevated creatinine (3.0 mg/dL) [2]. 5. **Fever.** The hallmark of TTP is a deficiency of the **ADAMTS13** enzyme, leading to ultra-large von Willebrand factor (vWF) multimers that cause spontaneous platelet aggregation and microthrombi. **Why Plasma Exchange (PEX) is correct:** PEX is the **treatment of choice** and a medical emergency. It works by removing the autoantibodies against ADAMTS13 and replacing the deficient enzyme. It should be initiated immediately upon suspicion to prevent irreversible organ damage and death. **Why other options are incorrect:** * **B & C (Corticosteroids/IVIG):** While steroids are often used as an adjunct to PEX, they are not the primary treatment. IVIG is the treatment for Immune Thrombocytopenic Purpura (ITP), which lacks MAHA and renal failure [3]. * **D (Heparin):** Anticoagulation is ineffective and contraindicated as it increases the risk of bleeding in the setting of severe thrombocytopenia. **High-Yield Clinical Pearls for NEET-PG:** * **Coagulation Profile:** In TTP/HUS, PT and aPTT are **normal** (unlike DIC, where they are prolonged) [1]. * **HUS vs. TTP:** Hemolytic Uremic Syndrome (HUS) presents similarly but is more common in children, usually follows bloody diarrhea (EHEC O157:H7), and features **predominant renal failure** with fewer neurological symptoms [2]. * **Contraindication:** **Platelet transfusion** is generally contraindicated in TTP as it may "fuel the fire" by promoting further microthrombi formation.

Q: What is the recommended hemoglobin level in male patients with polycythemia rubra vera to avoid thrombotic episodes?

14 g/dL. In Polycythemia Vera (PV), the primary goal of management is to reduce the risk of arterial and venous thrombosis, which are the leading causes of morbidity and mortality [1]. The cornerstone of therapy is therapeutic phlebotomy. **1. Why 14 g/dL is correct:** The risk of thrombotic episodes in PV is directly correlated with blood viscosity, which rises exponentially once the hematocrit (Hct) exceeds 45%. Clinical guidelines (based on the landmark CYTO-PV trial) recommend maintaining a **hematocrit <45%** in men. Since the hemoglobin (Hb) level is roughly one-third of the hematocrit value, a Hct of 45% corresponds to an **Hb of 15 g/dL**. To ensure a safety margin and keep the Hct strictly below 45%, the target Hb is maintained at **14 g/dL**. (Note: In females, the target Hct is <42%, corresponding to an Hb of ~12-13 g/dL). **2. Why incorrect options are wrong:** * **12 g/dL (Option B):** While this is a safe level, it is the target for females rather than males. Aiming for 12 g/dL in males may lead to unnecessary phlebotomies and symptomatic iron deficiency. * **10 g/dL and 8 g/dL (Options C & D):** These levels are unnecessarily low. Maintaining such levels would induce severe iron deficiency anemia and related symptoms (pica, glossitis, fatigue) without providing additional protection against thrombosis compared to the 14 g/dL target. **High-Yield Clinical Pearls for NEET-PG:** * **Mutation:** >95% of PV patients have the **JAK2 V617F** mutation [1]. * **Diagnostic Hallmark:** Low serum Erythropoietin (EPO) levels (unlike secondary polycythemia). * **Symptom:** **Aquagenic pruritus** (itching after a warm bath) is a classic board-exam clue [1]. * **Treatment:** Low-dose Aspirin is indicated for all patients unless contraindicated. Hydroxyurea is the first-line myelosuppressive agent for high-risk patients (age >60 or prior thrombosis).

Q: Microcytic hypochromic anaemia is seen in all of the following conditions except?

Malaria. Microcytic hypochromic anemia is characterized by a Mean Corpuscular Volume (MCV) < 80 fL and a Mean Corpuscular Hemoglobin Concentration (MCHC) < 32 g/dL [3]. This occurs due to defects in hemoglobin synthesis (either heme or globin). **Why Malaria is the correct answer:** Malaria typically causes a **Normocytic Normochromic anemia** [4]. The primary mechanism is the destruction of mature red blood cells (hemolysis) and splenic sequestration, rather than a defect in hemoglobin production [3, 5]. While chronic or severe malaria can occasionally lead to dyserythropoiesis, it is not a classic cause of microcytosis [4]. **Analysis of incorrect options:** * **Iron Deficiency Anemia (IDA):** The most common cause of microcytic hypochromic anemia worldwide [1, 4]. Lack of iron leads to decreased heme synthesis. * **Beta Thalassemia:** A quantitative defect in globin chain synthesis. It presents with significant microcytosis (often very low MCV) and a characteristic "target cell" appearance on peripheral smear. * **Lead Poisoning:** Lead inhibits enzymes in the heme synthesis pathway (ferrochelatase and ALA dehydratase), resulting in microcytic anemia with characteristic **basophilic stippling**. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for Microcytic Anemia (TAILS):** **T**halassemia, **A**nemia of chronic disease (some cases), **I**ron deficiency, **L**ead poisoning, **S**ideroblastic anemia [1]. * **Mentzer Index:** (MCV/RBC count). If ** 13**, it suggests Iron Deficiency Anemia. * **RDW (Red Cell Distribution Width):** Usually increased in IDA (anisocytosis) but normal in uncomplicated Thalassemia trait.

Q: What is a hallmark of hemophilia?

Hemarthrosis. **Explanation:** **Hemophilia** (A or B) is a classic example of a **secondary hemostasis defect** (clotting factor deficiency) [1]. Unlike primary hemostasis defects (platelet disorders), which present with superficial bleeding like petechiae and epistaxis, secondary hemostasis defects typically manifest as deep-seated bleeding [3]. **Why Hemarthrosis is the Correct Answer:** **Hemarthrosis** (bleeding into joint spaces) is the clinical hallmark of hemophilia [1]. It most commonly affects large weight-bearing joints like the knees, elbows, and ankles [1]. Recurrent hemarthrosis leads to synovial hypertrophy and chronic joint destruction, known as **hemophilic arthropathy** [1]. **Analysis of Incorrect Options:** * **A. Epistaxis:** This is more characteristic of **primary hemostasis defects** (e.g., Von Willebrand Disease or thrombocytopenia) [3]. While it can occur in hemophilia, it is not the "hallmark" feature. * **C. Abdominal pain:** While internal bleeding (like iliopsoas hematoma) can cause pain, it is a complication rather than a diagnostic hallmark [1]. * **D. Anemia:** Anemia is a non-specific consequence of chronic or acute blood loss and is not diagnostic of any specific bleeding disorder. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Both Hemophilia A (Factor VIII deficiency) and B (Factor IX deficiency) are **X-linked recessive** [2]. * **Lab Profile:** Characterized by **Prolonged aPTT** with a **Normal PT and Normal Bleeding Time**. * **Mixing Study:** A prolonged aPTT that **corrects** upon mixing with normal plasma indicates a factor deficiency (like Hemophilia), whereas failure to correct suggests an inhibitor. * **Treatment:** Factor replacement is the mainstay. For mild Hemophilia A, **Desmopressin (DDAVP)** can be used to release stored Factor VIII and vWF.

Q: All of the following are poor prognostic factors for Hodgkin's disease, except?

Younger age. In Hodgkin’s Lymphoma (HL), prognosis is determined by the clinical stage, histological subtype, and specific clinical markers. **Explanation of the Correct Answer:** **Option A (Younger age)** is the correct answer because it is actually a **favorable** prognostic factor. In HL, **older age (>45–50 years)** is consistently associated with a poorer prognosis due to a higher frequency of comorbidities, decreased tolerance to intensive chemotherapy, and a higher prevalence of unfavorable histological subtypes (like Mixed Cellularity). Younger patients typically have better performance status and a higher cure rate. **Explanation of Incorrect Options (Poor Prognostic Factors):** * **Option B (Systemic manifestations):** Also known as **"B symptoms"** (fever, night sweats, weight loss), these indicate a higher tumor burden and more aggressive disease, correlating with a worse prognosis. * **Option C (Lymphocyte depletion):** This is the rarest histological subtype of HL and carries the **worst prognosis**. Conversely, Lymphocyte Predominant HL has the best prognosis. * **Option D (Mediastinal disease):** Specifically "Bulky disease" (mediastinal mass >1/3 of the maximum intrathoracic diameter) is a major poor prognostic factor in early-stage HL, as it increases the risk of local recurrence. **High-Yield Clinical Pearls for NEET-PG:** * **Hasenclever Index (International Prognostic Score):** Used for advanced HL. Poor prognostic factors include: Age ≥45, Male sex, Stage IV disease, Albumin <4 g/dL, Hemoglobin <10.5 g/dL, WBC ≥15,000/µL, and Lymphocytes <600/µL (or <8%). * **Ann Arbor Staging:** The most important factor for determining treatment. * **Reed-Sternberg Cells:** The hallmark "Owl’s eye" appearance; CD15+ and CD30+ (except in Nodular Lymphocyte Predominant HL, which is CD20+).

Q: A 42-year-old female presents with bleeding gums for the past 20 days. Intra-oral examination shows thickened and friable gums. She also has hepatosplenomegaly and generalized non-tender lymphadenopathy. Laboratory findings include: Hemoglobin 11.4 g/dL, Platelet count 90,000/mm³, WBC count 4600/mm³. Bone marrow biopsy shows 100% cellularity with many large blasts that are peroxidase negative and non-specific esterase positive. What is the most likely diagnosis for this patient?

Acute monocytic leukemia. ### Explanation The correct diagnosis is **Acute Monocytic Leukemia (AML-M5)**. **1. Why Acute Monocytic Leukemia is correct:** The clinical hallmark of monocytic leukemia is **extramedullary involvement**, specifically **gingival hypertrophy** (thickened, friable gums) [1], hepatosplenomegaly, and lymphadenopathy [2]. Monoblasts have a high propensity to infiltrate tissues. * **Laboratory Findings:** The bone marrow shows large blasts that are **Myeloperoxidase (MPO) negative** (common in M5) but strongly **Non-Specific Esterase (NSE) positive**. NSE is the characteristic marker for the monocytic lineage. **2. Why other options are incorrect:** * **A. Acute Lymphoblastic Leukemia (ALL):** While common in children and presenting with lymphadenopathy [2], ALL blasts are typically **PAS positive** and would not show NSE positivity or specific gingival infiltration. * **B. Acute Megakaryocytic Leukemia (AML-M7):** This is often associated with myelofibrosis (dry tap) and Down Syndrome. Blasts are identified by platelet markers (CD41/CD61), not NSE. * **C. Acute Promyelocytic Leukemia (AML-M3):** Characterized by Auer rods, DIC, and strong **MPO positivity**. It does not typically present with gingival hypertrophy or NSE positivity. **3. NEET-PG High-Yield Pearls:** * **Gingival Hyperplasia:** Classic for AML-M4 (Myelomonocytic) and **AML-M5 (Monocytic)** [1]. * **NSE Stain:** Highly specific for monocytes. If the stain is inhibited by sodium fluoride, it confirms the monocytic origin. * **MPO Stain:** The most sensitive marker for the myeloid lineage (positive in M1, M2, M3, M4). * **M5 Subtypes:** M5a (mostly monoblasts) and M5b (monoblasts + promonocytes + monocytes).

Question 1

A patient presents with fever and altered consciousness. Investigations reveal anemia with fragmented red blood cells, a platelet count of 20,000/mm³, serum creatinine of 3.0 mg/dL, and normal prothrombin time (PT) and activated partial thromboplastin time (aPTT). Which of the following is the most appropriate treatment for this patient?

Accepted Answer

Plasma exchange therapy

Answer

Corticosteroids and intravenous immunoglobulin

Answer

Immunoglobulins

Answer

Anticoagulation with heparin

Question 2

What is the recommended hemoglobin level in male patients with polycythemia rubra vera to avoid thrombotic episodes?

Accepted Answer

14 g/dL

Answer

12 g/dL

Answer

10 g/dL

Answer

8 g/dL

Question 3

Microcytic hypochromic anaemia is seen in all of the following conditions except?

Accepted Answer

Malaria

Answer

Beta thalassemia

Answer

Iron deficiency

Answer

Lead poisoning

Question 4

A 39-year-old man is seeing red-colored urine in the morning. The CBC reveals anemia, low serum iron, and an elevated reticulocyte count. Laboratory studies show increased lysis of erythrocytes when incubated with either sucrose or acidified serum. Which of the following is the appropriate diagnosis?

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Anemia of chronic renal failure

Answer

Hereditary spherocytosis

Answer

Microangiopathic hemolytic anemia

Question 5

What is a hallmark of hemophilia?

Accepted Answer

Hemarthrosis

Answer

Epistaxis

Answer

Abdominal pain

Answer

Anemia

Question 6

Which of the following statements is true regarding Chronic Myeloid Leukemia (CML)?

Accepted Answer

The size of splenomegaly can indicate prognosis.

Answer

Phagocytic activity of white blood cells is reduced.

Answer

Sudan black stain is specific for myeloblasts.

Answer

Myeloblasts, granuloblasts, and lymphoblasts become Philadelphia chromosome positive following remission.

Question 7

A 42-year-old female diagnosed with iron deficiency anemia was treated with oral iron therapy. Her hemoglobin level during review was 8 g%. What is the most likely cause for the failure of treatment?

Accepted Answer

Poor compliance to oral iron

Answer

Acquired sideroblastic anemia

Answer

Inadequate iron dosage

Answer

Folate deficiency

Question 8

All of the following are poor prognostic factors for Hodgkin's disease, except?

Accepted Answer

Younger age

Answer

Systemic manifestations

Answer

Lymphocyte depletion

Answer

Mediastinal disease

Question 9

A 42-year-old female presents with bleeding gums for the past 20 days. Intra-oral examination shows thickened and friable gums. She also has hepatosplenomegaly and generalized non-tender lymphadenopathy. Laboratory findings include: Hemoglobin 11.4 g/dL, Platelet count 90,000/mm³, WBC count 4600/mm³. Bone marrow biopsy shows 100% cellularity with many large blasts that are peroxidase negative and non-specific esterase positive. What is the most likely diagnosis for this patient?

Accepted Answer

Acute monocytic leukemia

Answer

Acute lymphoblastic leukemia

Answer

Acute megakaryocytic leukemia

Answer

Acute promyelocytic leukemia

Question 10

Chronic hemolysis from which of the following conditions may be associated with iron deficiency?

Accepted Answer

Paroxysmal cold Hemoglobinuria

Answer

Thalassemia

Answer

Hereditary spherocytosis

Answer

G 6PD deficiency

Hematology — MCQs

Hematology — MCQs

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