Hematology — MCQs

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?

Q109Easy

Which of the following is false about hemolytic anemias?

Q110Easy

The TRUE statement about B cell Prolymphocytic leukemia is:

Hematology Indian Medical PG Practice Questions and MCQs

Question 101: Hemolytic anemia is associated with all of the following, except:

A. Increased indirect bilirubin in the serum
B. Decreased red cell survival
C. Increased number of reticulocytes
D. Decreased fecal Urobilinogen (Correct Answer)

Explanation: **Explanation:** Hemolytic anemia is characterized by the premature destruction of red blood cells (RBCs). To understand the biochemical changes, one must follow the pathway of hemoglobin degradation. **Why Option D is the Correct Answer:** In hemolytic anemia, there is an **increase** in fecal urobilinogen, not a decrease [3]. When RBCs break down, heme is converted into unconjugated bilirubin. This bilirubin is conjugated in the liver and excreted into the intestines via bile [2]. Intestinal bacteria then convert conjugated bilirubin into **urobilinogen**. A portion of this is excreted in the feces as stercobilin (fecal urobilinogen). Since hemolysis increases the "raw material" (bilirubin), the end product (fecal urobilinogen) must also increase. **Analysis of Incorrect Options:** * **A. Increased indirect bilirubin:** This is a hallmark of hemolysis. The liver's conjugating capacity is overwhelmed by the massive release of heme, leading to a rise in unconjugated (indirect) bilirubin [3]. * **B. Decreased red cell survival:** By definition, hemolytic anemia involves the destruction of RBCs before their normal 120-day lifespan ends [1]. * **C. Increased number of reticulocytes:** This represents the bone marrow's compensatory response. To make up for the anemia, the marrow increases erythropoiesis, releasing immature RBCs (reticulocytes) into the peripheral blood. **NEET-PG High-Yield Pearls:** * **Haptoglobin:** The most sensitive laboratory marker for hemolysis is **decreased serum haptoglobin** (it binds to free hemoglobin). * **LDH:** Serum Lactate Dehydrogenase is typically **elevated** due to release from ruptured RBCs. * **Urine:** In intravascular hemolysis, look for **hemosiderinuria** (chronic) and **hemoglobinuria** (acute). Note that bilirubin is *not* found in the urine in hemolytic jaundice because unconjugated bilirubin is water-insoluble [3].

Question 102: A 23-year-old African-American man with a history since early childhood of severe anemia requiring many transfusions presents with non-healing leg ulcers and recurrent episodes of abdominal and chest pain. Which laboratory abnormality is most likely associated with these signs and symptoms?

A. Decreased erythropoietin
B. Increased erythrocyte osmotic fragility
C. Decreased erythropoiesis
D. Sickle cells on peripheral blood smear (Correct Answer)

Explanation: **Explanation:** The clinical presentation of severe anemia requiring transfusions since childhood, recurrent episodes of abdominal and chest pain (vaso-occlusive crises), and non-healing leg ulcers in an African-American patient is classic for **Sickle Cell Anemia (SCA)**. **1. Why the Correct Answer is Right:** Sickle Cell Anemia is caused by a point mutation in the $\beta$-globin gene (glutamic acid replaced by valine at the 6th position). Under conditions of hypoxia, acidosis, or dehydration, the abnormal Hemoglobin S (HbS) polymerizes [1], causing RBCs to assume a "sickle" shape. These rigid cells lead to microvascular occlusion (causing pain and ulcers) and premature hemolysis. The presence of **sickle cells on a peripheral blood smear** is a hallmark diagnostic finding [2]. **2. Why Incorrect Options are Wrong:** * **A. Decreased erythropoietin:** In chronic hemolytic anemias like SCA, the body compensates for low oxygen-carrying capacity by *increasing* erythropoietin production to stimulate the bone marrow. * **B. Increased erythrocyte osmotic fragility:** This is the characteristic finding in **Hereditary Spherocytosis**, not SCA. In fact, sickle cells often show *decreased* osmotic fragility due to a higher surface-area-to-volume ratio. * **C. Decreased erythropoiesis:** Patients with SCA typically have *increased* erythropoiesis (erythroid hyperplasia) to compensate for hemolysis, unless they experience an aplastic crisis (e.g., Parvovirus B19 infection) [2]. **Clinical Pearls for NEET-PG:** * **Leg Ulcers:** Occur in ~10% of SCA patients, usually over the medial/lateral malleoli due to ischemia. * **Autosplenectomy:** Recurrent splenic infarcts lead to a shrunken, fibrotic spleen by adulthood [2], increasing risk for encapsulated organisms (*S. pneumoniae, H. influenzae*). * **Salmonella Osteomyelitis:** SCA patients have a unique predisposition to *Salmonella* bone infections. * **Screening/Diagnosis:** Solubility test (screening) and Hb Electrophoresis (confirmatory) [1].

Question 103: Which of the following is a poor prognostic factor associated with acute lymphoblastic leukemia (ALL) in children?

A. Total leukocyte count of 4,000-10,000/µL
B. Age at diagnosis more than 2 years
C. Testicular involvement (Correct Answer)
D. Female gender

Explanation: **Explanation:** Prognostic factors in Acute Lymphoblastic Leukemia (ALL) are critical for risk stratification and determining treatment intensity. **Why Testicular Involvement is Correct:** Testicular involvement at the time of diagnosis is considered a **poor prognostic factor**. The testes (along with the CNS) act as "pharmacological sanctuaries" where the blood-testis barrier prevents many standard chemotherapeutic agents from reaching effective concentrations. This increases the risk of relapse and necessitates more intensive systemic therapy or local radiation. **Analysis of Incorrect Options:** * **A. Total Leukocyte Count (TLC) of 4,000-10,000/µL:** This is a **favorable** prognostic factor. A low initial WBC count (<50,000/µL for B-ALL) is associated with better outcomes. High WBC counts (>50,000/µL in B-ALL or >100,000/µL in T-ALL) indicate high tumor burden and poor prognosis [1]. * **B. Age at diagnosis >2 years:** The "age peak" for a good prognosis is between **1 and 10 years**. Infants (<1 year) and adolescents/adults (>10 years) have a significantly poorer prognosis. Therefore, being >2 years old (within the 1-10 range) is generally favorable. * **D. Female Gender:** Statistically, **females** have a slightly better prognosis than males. Males have a higher risk of late relapse, partly due to the potential for occult testicular involvement. **High-Yield Clinical Pearls for NEET-PG:** * **Best Prognostic Age:** 1–9 years. * **Cytogenetics:** **t(12;21)** (ETV6-RUNX1) has the **best** prognosis; **t(9;22)** (Philadelphia chromosome) and **t(4;11)** have the **worst** prognosis [1]. * **Ploidy:** Hyperdiploidy (>50 chromosomes) is a favorable factor. * **Early Response:** The most important independent prognostic factor is the **minimal residual disease (MRD)** status after the induction phase of chemotherapy [1].

Question 104: Which of the following is an example of a chronic myeloproliferative disorder?

A. Neutrophilic leukemoid reaction
B. Essential thrombocythemia (Correct Answer)
C. Plasmacytosis
D. Hairy cell leukemia

Explanation: Explanation: Chronic Myeloproliferative Neoplasms (MPNs) are a group of clonal hematopoietic stem cell disorders characterized by the autonomous overproduction of one or more myeloid lineages (erythroid, granulocytic, or megakaryocytic) [2] in the bone marrow. **Why Essential Thrombocythemia (ET) is correct:** ET is a classic MPN characterized by persistent thrombocytosis (platelet count >450,000/µL) and megakaryocytic hyperplasia in the bone marrow. It is driven by mutations in **JAK2 (50-60%)**, **CALR**, or **MPL** genes, which lead to constitutive signaling of the thrombopoietin receptor. **Why the other options are incorrect:** * **Neutrophilic leukemoid reaction:** This is a **reactive** increase in white blood cell count (usually >50,000/µL) in response to infection, inflammation, or malignancy. It is not a clonal neoplastic process. * **Plasmacytosis:** This refers to an increased number of plasma cells. While it can be seen in neoplastic conditions like Multiple Myeloma, it is a **lymphoid** lineage disorder, not a myeloid one [2]. * **Hairy cell leukemia:** This is a chronic **B-cell lymphoproliferative disorder** (mature B-cell neoplasm) characterized by "hairy" cytoplasmic projections and BRAF V600E mutations. **High-Yield Clinical Pearls for NEET-PG:** * **Classic MPNs include:** Chronic Myeloid Leukemia (CML), Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Primary Myelofibrosis (PMF). * **CML** is distinguished by the presence of the **Philadelphia chromosome [t(9;22)]** and the BCR-ABL1 fusion gene. * **JAK2 V617F mutation** is present in >95% of PV cases [1] and ~50-60% of ET and PMF cases. * **Erythromelalgia** (burning pain and redness in extremities) is a characteristic clinical feature of ET and PV due to microvascular occlusion.

Question 105: 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?

A. Reticulocyte count
B. Osmotic fragility test
C. Coombs test (Correct Answer)
D. Bone marrow aspiration

Explanation: ### 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.

Question 106: Which of the following is not a type of hypoproliferative anemia?

A. Anemia caused by Parvovirus B19 infection
B. Anemia of chronic disease/inflammation
C. Fanconi anemia
D. G6PD deficiency (Correct Answer)

Explanation: 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 < 2% indicates hypoproliferative anemia; 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.

Question 107: 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?

A. Phenytoin toxicity
B. Fish tapeworm infection
C. Hookworm infection (Correct Answer)
D. Blind loop syndrome

Explanation: ### 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 Thalassemia; >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.

Question 108: 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?

A. Disseminated intravascular coagulation
B. Glanzmann thrombasthenia (Correct Answer)
C. Immune thrombocytopenic purpura
D. Vitamin C deficiency

Explanation: 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 109: Which of the following is false about hemolytic anemias?

A. Jaundice is the main clinical sign
B. Shortened red cell life span
C. Increased reticulocyte count
D. Decreased erythropoietin levels (Correct Answer)

Explanation: **Explanation:** Hemolytic anemia is characterized by the premature destruction of red blood cells (RBCs) [1]. To understand this question, one must differentiate between the body’s compensatory mechanisms and the pathological consequences of hemolysis. **Why Option D is the Correct (False) Statement:** In hemolytic anemia, the rapid destruction of RBCs leads to a decrease in the oxygen-carrying capacity of the blood (anemia). This tissue hypoxia is sensed by the peritubular interstitial cells of the kidney, which respond by **increasing the production of Erythropoietin (EPO)** [3]. Elevated EPO levels stimulate the bone marrow to increase erythropoiesis to compensate for the loss. Therefore, erythropoietin levels are **increased**, not decreased. **Analysis of Other Options:** * **Option A (Jaundice):** This is a hallmark sign. The breakdown of hemoglobin releases heme, which is converted into unconjugated bilirubin [2]. When the liver's conjugating capacity is exceeded, **unconjugated hyperbilirubinemia** and clinical jaundice occur. * **Option B (Shortened RBC life span):** This is the fundamental definition of hemolysis [1]. Normal RBCs live for approximately 120 days; in hemolytic states, this is significantly reduced. * **Option C (Increased reticulocyte count):** As the bone marrow responds to elevated EPO, it releases immature RBCs (reticulocytes) into the peripheral blood. A high **Reticulocyte Production Index (RPI > 2%)** is a key diagnostic feature of hemolysis. **NEET-PG High-Yield Pearls:** * **Haptoglobin:** The most sensitive laboratory marker for hemolysis is **decreased serum haptoglobin** (it binds to free hemoglobin). * **LDH:** Serum Lactate Dehydrogenase is typically **elevated** due to release from ruptured RBCs. * **Urine:** In intravascular hemolysis, look for **hemosiderinuria** (chronic) and **hemoglobinuria** (acute), but notably, there is **no bilirubin in the urine** (acholuric jaundice) because unconjugated bilirubin is not water-soluble.

Question 110: The TRUE statement about B cell Prolymphocytic leukemia is:

A. The median age is 40
B. Generalised lymphadenopathy is the presenting symptom
C. The cells express surface IgM and CD19, 20, 22 (Correct Answer)
D. Splenectomy improves survival

Explanation: **Explanation:** **B-cell Prolymphocytic Leukemia (B-PLL)** is a rare, aggressive mature B-cell neoplasm characterized by a high count of circulating prolymphocytes (usually >55%). **Why Option C is Correct:** B-PLL cells represent a mature B-cell phenotype. They characteristically show **strong expression of surface IgM** (with or without IgD) and pan-B-cell markers including **CD19, CD20, and CD22**. Unlike Chronic Lymphocytic Leukemia (CLL), B-PLL cells typically express high density surface immunoglobulins and are usually **CD5 negative** and **CD23 negative** (though CD5 can be positive in 20-30% of cases). **Why Other Options are Incorrect:** * **Option A:** The median age at diagnosis is **65–70 years**. It is a disease of the elderly and is extremely rare in patients under 50. * **Option B:** The hallmark clinical feature is **massive splenomegaly** with a rapidly rising white blood cell count (often >100,000/µL). In contrast to CLL, **lymphadenopathy is typically minimal or absent**. * **Option D:** While splenectomy or splenic irradiation may provide symptomatic relief for massive, painful splenomegaly or hypersplenism, it is a palliative measure and **does not improve overall survival**. **High-Yield Clinical Pearls for NEET-PG:** * **Morphology:** Prolymphocytes are larger than mature lymphocytes, featuring a vesicular nucleus and a **single, prominent, central nucleolus**. * **Cytogenetics:** The most common abnormality is **17p deletion (TP53 mutation)**, which contributes to its poor prognosis and resistance to conventional chemotherapy. * **Differential Diagnosis:** Must be distinguished from "CLL in prolymphocytoid transformation," where prolymphocytes exceed 10% but are less than 55%.

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Hematology — MCQs

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