Hematology Practice Questions

Q: What is true about Immune Thrombocytopenic purpura (ITP)?

Intracranial bleed is a rare complication.. **Explanation:** Immune Thrombocytopenic Purpura (ITP) is an acquired hemorrhagic disorder caused by the immune-mediated destruction of platelets. **1. Why Option A is correct:** Intracranial hemorrhage (ICH) is the most dreaded but **rare** complication of ITP, occurring in less than **0.1% to 0.5%** of cases. It typically occurs when the platelet count falls below 10,000/mm³. Because the primary defect is in platelet number (not function) and the remaining platelets are often young and hyperfunctional, spontaneous major bleeding is uncommon despite very low counts. **2. Why the other options are incorrect:** * **Option B:** Splenomegaly is **not** a feature of ITP. The spleen is the site of platelet destruction, but it does not typically enlarge. If significant splenomegaly is present, clinicians must investigate alternative diagnoses like leukemia or hypersplenism. * **Option C:** In children (Acute ITP), there is **no gender predilection** (Male = Female). A female preponderance is only seen in the chronic form, typically in adolescents and adults. * **Option D:** While Acute ITP is indeed self-limiting (resolving within 6 months in 80% of children), **Option A is the more definitive "classic" teaching point** regarding complications in standard pediatric textbooks (like Nelson). *Note: In many exam formats, D is also considered a true statement, but A is the high-yield clinical fact prioritized in NEET-PG.* **Clinical Pearls for NEET-PG:** * **Trigger:** Often follows a viral infection (e.g., URTI) by 1–4 weeks. * **Bone Marrow:** Shows increased or normal megakaryocytes (compensatory). * **Treatment:** Observation is preferred if bleeding is minimal. If treatment is needed (platelets 12 months.

Q: A male baby was born in a hospital. After 12 hours of birth, the baby was found to be pale. His serum total bilirubin level was 20 mg/dl and unconjugated bilirubin was 15 mg/dl. After 36 hours of birth, Hemoglobin was 14 g/dl, and the reticulocyte count was high. On peripheral smear, nucleated RBCs and spherocytes are seen. What is the best possible diagnosis?

Hereditary spherocytosis. **Explanation:** The clinical presentation points towards a case of **early-onset neonatal jaundice** with evidence of **hemolysis**. **1. Why Hereditary Spherocytosis (HS) is the correct answer:** * **Early Jaundice:** HS often presents within the first 24 hours of life. The high unconjugated bilirubin (15 mg/dl) and high reticulocyte count indicate active hemolysis. * **Peripheral Smear:** The presence of **spherocytes** and **nucleated RBCs** (erythroblasts) is the hallmark of HS. Spherocytes are formed due to defects in RBC membrane proteins (like Spectrin or Ankyrin), leading to membrane loss and a spherical shape. * **Hemoglobin:** A Hb of 14 g/dl in a neonate is relatively low (normal is 17–20 g/dl), supporting a hemolytic process. **2. Why other options are incorrect:** * **Rh Incompatibility:** While it causes severe early jaundice and nucleated RBCs (Erythroblastosis fetalis), the peripheral smear typically shows **polychromasia**, not spherocytes. Spherocytes are more characteristic of ABO incompatibility, but HS is the classic diagnosis when spherocytes dominate the smear. * **Pyruvate Kinase Deficiency:** This causes non-spherocytic hemolytic anemia. The smear would show **echinocytes** (burr cells) rather than spherocytes. * **Sickle Cell Anemia:** This rarely presents in the neonatal period because **HbF (Fetal Hemoglobin)** is predominant, which inhibits the sickling of RBCs. Symptoms usually appear after 6 months when HbF levels drop. **Clinical Pearls for NEET-PG:** * **Most common cause of HS:** Mutation in the **Ankyrin** gene (Autosomal Dominant). * **Screening Test:** Osmotic Fragility Test (increased fragility). * **Confirmatory Test:** EMA (Eosin-5-maleimide) binding test via flow cytometry (Gold Standard). * **Management:** Phototherapy/Exchange transfusion for neonatal jaundice; Splenectomy (usually deferred until after 5 years of age).

Q: A child presents with bleeding gums and a swollen knee. What is the most likely underlying cause?

Hemophilia. **Explanation:** The clinical presentation of **bleeding gums** combined with a **swollen knee** (suggestive of hemarthrosis) is a classic indicator of a coagulation factor deficiency, most commonly **Hemophilia**. **1. Why Hemophilia is Correct:** Hemophilia (A or B) is characterized by a deficiency in clotting factors (VIII or IX). The hallmark of secondary hemostasis defects is bleeding into deep tissues, specifically **hemarthrosis** (bleeding into joints like the knee) and intramuscular hematomas. While mucosal bleeding like bleeding gums is more common in primary hemostasis defects, it can occur in hemophilia following minor trauma or dental procedures. The presence of a swollen joint is the "clue" that points toward a factor deficiency rather than a platelet disorder. **2. Why Other Options are Incorrect:** * **ITP:** This is a disorder of primary hemostasis (platelets). It typically presents with superficial bleeding such as petechiae, purpura, and epistaxis. It rarely, if ever, causes hemarthrosis. * **Scurvy (Vitamin C deficiency):** While Scurvy causes bleeding gums and subperiosteal hemorrhages (which can cause limb pain/pseudoparalysis), it does not typically cause true intra-articular joint swelling (hemarthrosis). * **Trauma:** While trauma can cause a swollen knee, it would not explain spontaneous or recurrent bleeding gums unless there was significant facial injury. **NEET-PG High-Yield Pearls:** * **Hemarthrosis** is the most common clinical manifestation of Hemophilia. The **knee** is the most frequently involved joint. * **Lab Profile:** Prolonged aPTT, normal PT, and normal Bleeding Time (BT). * **Target Joints:** Repeated bleeding into the same joint leads to "Hemophilic Arthropathy," resulting in joint destruction. * **Differential:** If a question mentions bleeding gums + skin petechiae, think **Platelet disorders**. If it mentions deep muscle/joint bleeds, think **Coagulation disorders**.

Q: A 7-year-old child presented with high-grade fever with chills and rigor. Peripheral smear examination revealed Plasmodium vivax. The child was treated for malaria. Subsequently, the child presented with jaundice, and a repeat peripheral smear showed abnormalities. What is your diagnosis?

Glucose 6 Phosphate dehydrogenase deficiency. ### Explanation **Correct Option: D (Glucose-6-Phosphate Dehydrogenase Deficiency)** The clinical presentation describes a classic case of **drug-induced oxidative hemolysis**. *Plasmodium vivax* malaria requires treatment with **Primaquine** to eliminate the hypnozoite stage (preventing relapse). However, Primaquine is a potent oxidizing agent. In patients with G6PD deficiency, the red blood cells cannot generate sufficient NADPH to maintain reduced glutathione levels. This leads to oxidative damage, hemoglobin denaturation (forming **Heinz bodies**), and subsequent intravascular hemolysis, which manifests clinically as **jaundice** and dark urine shortly after starting treatment. **Why Incorrect Options are Wrong:** * **A. Viral Hepatitis:** While it causes jaundice, it does not typically follow malaria treatment or show specific "abnormalities" on a peripheral smear related to acute hemolysis. * **B. Sickle Cell Anemia:** This is a chronic hemolytic condition. While malaria can trigger a crisis, the temporal relationship with *P. vivax* treatment specifically points toward G6PD deficiency. * **C. Hemolytic Uremic Syndrome (HUS):** HUS typically presents with a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, usually following a diarrheal prodrome (e.g., Shiga toxin-producing *E. coli*), not standard malaria therapy. **NEET-PG High-Yield Pearls:** * **Screening Rule:** Always screen for G6PD levels before prescribing **Primaquine** or **Tafenoquine**. * **Peripheral Smear Findings:** Look for **Heinz bodies** (supravital stain) and **"Bite cells"** (degmacytes) resulting from splenic macrophages removing denatured hemoglobin. * **Inheritance:** G6PD deficiency is an **X-linked recessive** disorder, making it more common in males. * **Protective Effect:** G6PD deficiency provides a selective advantage against *Plasmodium falciparum* malaria.

Q: A 7-year-old girl with sickle cell anemia presents with extreme fatigue and pallor. Her parents report that several classmates had recent rashes and bright red cheeks. On examination, the conjunctiva, gums, and nail beds are pale, and tachycardia is noted. A CBC reveals a 2 g/dL drop in hemoglobin from her last result 3 months ago, with a reticulocyte count of 0.05%. What is the most likely diagnosis?

Aplastic crisis from parvovirus B19 infection. **Explanation:** The clinical presentation is a classic case of **Transient Aplastic Crisis (TAC)** triggered by **Parvovirus B19**. In patients with high red cell turnover (like Sickle Cell Anemia), Parvovirus B19 is particularly dangerous because it infects and lyses **erythroid progenitor cells** by binding to the **P-antigen** (globoside). The hallmark of an aplastic crisis is a sudden drop in hemoglobin accompanied by an **inappropriately low reticulocyte count** (<1%), indicating bone marrow failure rather than hemolysis. The mention of "bright red cheeks" in classmates refers to **Erythema Infectiosum (Fifth Disease)**, the common pediatric presentation of Parvovirus B19. **Analysis of Incorrect Options:** * **B. Coxsackievirus B:** While a common cause of viral myocarditis and pericarditis, it does not typically cause bone marrow suppression or aplastic crises. * **C. Norovirus:** This is a leading cause of viral gastroenteritis. While it causes vomiting and diarrhea, it does not target erythroid precursors or cause acute drops in hemoglobin. * **D. Coltivirus:** This is the causative agent of Colorado Tick Fever. While it can cause a mild leukopenia, it is not associated with aplastic crises in sickle cell patients. **NEET-PG High-Yield Pearls:** * **Receptor:** Parvovirus B19 uses the **P-antigen** on RBCs. * **Morphology:** Look for **"Giant Pronormoblasts"** with viral inclusions in the bone marrow. * **Other Manifestations:** Hydrops fetalis (in pregnancy), symmetric polyarthritis (in adults), and "Slapped Cheek" rash. * **Key Lab Finding:** The **reticulocytopenia** is the "clincher" to differentiate aplastic crisis from a sequestration or hemolytic crisis.

Q: Which of the following is NOT a feature of TAR syndrome?

Ventricular septal defect. **Explanation:** **TAR Syndrome (Thrombocytopenia-Absent Radius)** is a rare genetic disorder characterized by the hallmark association of bilateral radial aplasia and a low platelet count. 1. **Why Option C is the correct answer:** While approximately 33% of patients with TAR syndrome have congenital heart defects, the most common cardiac anomaly is **Tetralogy of Fallot (TOF)** or **Atrial Septal Defect (ASD)**. Ventricular Septal Defect (VSD) is not a classic or defining feature of this syndrome, making it the "least likely" or "not a feature" in a comparative MCQ context. 2. **Analysis of Incorrect Options:** * **A. Thrombocytopenia:** This is a mandatory feature. It is usually most severe in the first year of life and may improve over time. * **B. Multiple skeletal anomalies:** The defining feature is **bilateral absence of the radius**. Crucially, unlike Fanconi Anemia, the **thumbs are always present** in TAR syndrome. Other anomalies can include ulnar or humeral hypoplasia and lower limb defects (e.g., dislocated hips). * **D. Autosomal recessive inheritance:** TAR syndrome follows an autosomal recessive pattern, typically involving a microdeletion on chromosome **1q21.1** combined with a specific mutation in the **RBM8A gene**. **High-Yield Clinical Pearls for NEET-PG:** * **The "Thumb" Rule:** In TAR syndrome, the **thumb is present**. In Fanconi Anemia and Holt-Oram Syndrome, the thumb is typically absent or hypoplastic. * **Hematology:** Platelet counts usually stabilize after the first year of life. * **Leukemoid Reaction:** Patients may show an extremely high WBC count (mimicking leukemia) during infancy. * **Cow’s Milk Allergy:** There is a known high association between TAR syndrome and cow's milk intolerance.

Q: Which condition has an autosomal dominant inheritance pattern?

Diamond blackfan anaemia. The correct answer is **Diamond-Blackfan Anaemia (DBA)**. This condition is a congenital pure red cell aplasia characterized by macrocytic anemia, reticulocytopenia, and a selective decrease in erythroid precursors in the bone marrow. **1. Why Diamond-Blackfan Anaemia is correct:** DBA is primarily inherited in an **Autosomal Dominant (AD)** pattern. It is caused by mutations in genes encoding ribosomal proteins (most commonly **RPS19**), leading to "ribosomal stress" and p53-mediated apoptosis of erythroid progenitor cells. Approximately 25% of patients also present with physical anomalies (triphalangeal thumbs, craniofacial defects). **2. Why the other options are incorrect:** * **Schwachman-Diamond Syndrome (SDS):** This is an **Autosomal Recessive (AR)** disorder characterized by exocrine pancreatic insufficiency and bone marrow failure (neutropenia). * **Dyskeratosis Congenita:** This is genetically heterogeneous but most commonly follows an **X-linked Recessive** pattern (DKC1 gene). While AD and AR forms exist, it is classically taught as X-linked in the context of the "triad" (nail dystrophy, leukoplakia, hyperpigmentation). * **Congenital Amegakaryocytic Thrombocytopenia (CAMT):** This is an **Autosomal Recessive (AR)** disorder caused by a mutation in the *MPL* gene (thrombopoietin receptor), leading to severe isolated thrombocytopenia at birth. **Clinical Pearls for NEET-PG:** * **DBA vs. TEC:** Transient Erythroblastopenia of Childhood (TEC) is an acquired mimic of DBA but occurs in older children (>1 year) and has no physical anomalies. * **Thumb Anomalies:** Both DBA and Fanconi Anemia (AR) feature thumb deformities; however, DBA is a pure red cell aplasia, while Fanconi is a pancytopenia. * **Biochemical Marker:** Elevated **Erythrocyte Adenosine Deaminase (eADA)** levels are a high-yield diagnostic marker for DBA.

Q: An 8-month-old male infant is brought in for routine checkup. The child is pale, with a hemoglobin level of 9 g/dL, mean corpuscular volume of 65 fL, increased total iron binding capacity, and low serum iron. The infant was breastfed for three months, followed by cow's milk substitute. For the last two months, fruits and vegetables have been introduced, but not eggs or milk. What is the best treatment?

Dietary modification and iron supplementation. **Explanation:** The infant presents with classic features of **Iron Deficiency Anemia (IDA)**: microcytic (MCV 65 fL), hypochromic anemia with low serum iron and elevated Total Iron Binding Capacity (TIBC). The primary etiology here is nutritional, specifically the early introduction of cow’s milk (before 12 months) and a lack of iron-rich complementary foods. **1. Why Option D is Correct:** The management of nutritional IDA requires a two-pronged approach. **Iron supplementation** (typically 3–6 mg/kg/day of elemental iron) is essential to replenish depleted iron stores and correct the hemoglobin level. Simultaneously, **dietary modification** is crucial to prevent recurrence. This includes limiting cow's milk intake (which is low in iron and can cause occult GI blood loss) and introducing iron-fortified cereals, meats, or legumes. **2. Why Other Options are Wrong:** * **Option A:** Multivitamins do not contain sufficient elemental iron to treat established anemia. * **Option B:** While diet is important, dietary changes alone are too slow to correct significant anemia (Hb 9 g/dL) and cannot efficiently replenish bone marrow iron stores. * **Option C:** Iron supplementation alone treats the symptom but fails to address the underlying cause (poor diet/excessive cow's milk), leading to a high risk of relapse once the supplement is stopped. **Clinical Pearls for NEET-PG:** * **Cow’s Milk:** Should not be started before **1 year of age** due to low iron bioavailability and risk of microscopic intestinal bleeding. * **Screening:** AAP recommends universal screening for anemia at **12 months** of age. * **Response to Iron:** The earliest sign of response to iron therapy is an increase in **reticulocyte count** (peaks in 5–10 days), followed by a rise in hemoglobin. * **Duration:** Iron therapy should continue for **2–3 months after** hemoglobin normalizes to replenish ferritin stores.

Question 1

What is true about Immune Thrombocytopenic purpura (ITP)?

Accepted Answer

Intracranial bleed is a rare complication.

Answer

Splenomegaly is prominent.

Answer

It is more common in females.

Answer

Acute ITP is a self-limiting condition.

Question 2

A male baby was born in a hospital. After 12 hours of birth, the baby was found to be pale. His serum total bilirubin level was 20 mg/dl and unconjugated bilirubin was 15 mg/dl. After 36 hours of birth, Hemoglobin was 14 g/dl, and the reticulocyte count was high. On peripheral smear, nucleated RBCs and spherocytes are seen. What is the best possible diagnosis?

Accepted Answer

Hereditary spherocytosis

Answer

Pyruvate kinase deficiency

Answer

Sickle cell anemia

Answer

Rh incompatibility

Question 3

A child presents with bleeding gums and a swollen knee. What is the most likely underlying cause?

Accepted Answer

Hemophilia

Answer

Idiopathic Thrombocytopenic Purpura (ITP)

Answer

Scurvy

Answer

Trauma

Question 4

Constitutional pancytopenia can be seen in which of the following conditions, except?

Accepted Answer

Diamond–Blackfan syndrome

Answer

Fanconi's anemia

Answer

Dyskeratosis congenita

Answer

Shwachman Diamond syndrome

Question 5

Diamond Blackfan anemia is another name for which of the following conditions?

Accepted Answer

Inherited erythroid aplasia

Answer

Inherited thrombocytopenia

Answer

Inherited leucopenia

Answer

All of the above

Question 6

A 7-year-old child presented with high-grade fever with chills and rigor. Peripheral smear examination revealed Plasmodium vivax. The child was treated for malaria. Subsequently, the child presented with jaundice, and a repeat peripheral smear showed abnormalities. What is your diagnosis?

Accepted Answer

Glucose 6 Phosphate dehydrogenase deficiency

Answer

Viral Hepatitis

Answer

Sickle cell Anemia

Answer

Hemolytic uremic syndrome

Question 7

A 7-year-old girl with sickle cell anemia presents with extreme fatigue and pallor. Her parents report that several classmates had recent rashes and bright red cheeks. On examination, the conjunctiva, gums, and nail beds are pale, and tachycardia is noted. A CBC reveals a 2 g/dL drop in hemoglobin from her last result 3 months ago, with a reticulocyte count of 0.05%. What is the most likely diagnosis?

Accepted Answer

Aplastic crisis from parvovirus B19 infection

Answer

Pericarditis caused by Coxsackievirus B

Answer

Gastroenteritis with bleeding caused by Norovirus

Answer

Exacerbated anemia from Colti virus infection

Question 8

Which of the following is NOT a feature of TAR syndrome?

Accepted Answer

Ventricular septal defect

Answer

Thrombocytopenia

Answer

Multiple skeletal anomalies

Answer

Autosomal recessive type of inheritance

Question 9

Which condition has an autosomal dominant inheritance pattern?

Accepted Answer

Diamond blackfan anaemia

Answer

Schwachman diamond syndrome

Answer

Dyskeratosis congenita

Answer

Congenital amegakaryocytic thrombocytopenia

Question 10

An 8-month-old male infant is brought in for routine checkup. The child is pale, with a hemoglobin level of 9 g/dL, mean corpuscular volume of 65 fL, increased total iron binding capacity, and low serum iron. The infant was breastfed for three months, followed by cow's milk substitute. For the last two months, fruits and vegetables have been introduced, but not eggs or milk. What is the best treatment?

Accepted Answer

Dietary modification and iron supplementation

Answer

Multivitamins

Answer

Dietary modification alone

Answer

Iron supplementation alone

Hematology — MCQs

Hematology — MCQs

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