Hematology Practice Questions

Q: In infants, from which bone is a bone marrow biopsy typically performed?

Tibia. **Explanation:** The site for bone marrow aspiration and biopsy varies significantly with age due to the conversion of red (hematopoietic) marrow to yellow (fatty) marrow. **1. Why Tibia is Correct:** In **infants (children <1 year of age)**, the **upper third of the tibia** (anteromedial surface, just below the tibial tuberosity) is the preferred site. This is because the tibia contains active red marrow in infancy and is easily accessible, superficial, and provides a stable surface for the procedure. **2. Analysis of Incorrect Options:** * **Posterior Superior Iliac Spine (PSIS):** This is the **most common overall site** for bone marrow biopsy in adults and children older than 1–2 years. In infants, however, the iliac bone is thin and not yet fully ossified, making it less ideal than the tibia. * **Sternum:** This is a common site in adults but is **strictly contraindicated in children**. The sternal plate in infants is very thin and the underlying mediastinal structures (heart and great vessels) are at high risk of fatal puncture. * **Iliac Crest (Anterior):** While it can be used if the patient cannot be turned prone, it is generally less preferred than the PSIS due to a thicker cortical bone layer. **3. NEET-PG High-Yield Pearls:** * **Preferred site 1–2 years & Adults:** Posterior Superior Iliac Spine (PSIS). * **Site for Intraosseous (IO) access in emergencies:** Proximal Tibia (same as biopsy site in infants). * **Marrow Conversion:** Red marrow is replaced by yellow marrow in a centripetal direction (from distal limbs toward the axial skeleton) as a child grows.

Q: An otherwise healthy 1-year-old child has a routine CBC revealing polymorphonuclear neutrophils as shown below. Which of the following is the most appropriate next step?

Begin folic acid supplementation. ***Begin folic acid supplementation*** - **Hypersegmented neutrophils** (PMNs with ≥5 lobes) are the hallmark finding of **megaloblastic anemia**, indicating folate or B12 deficiency. - In a **1-year-old infant**, **folate deficiency** is the most common cause of megaloblastic anemia, making folic acid supplementation the appropriate first-line treatment. *Bone marrow aspiration* - This invasive procedure is unnecessary when **hypersegmented neutrophils** already provide clear evidence of megaloblastic anemia. - Bone marrow aspiration would only be considered if there were concerns about **malignancy** or other hematologic disorders, which is not indicated here. *Begin oral iron therapy* - **Iron deficiency anemia** presents with **microcytic hypochromic** red blood cells, not hypersegmented neutrophils. - Iron therapy would not address the underlying **folate deficiency** causing the megaloblastic changes observed. *Hemoglobin electrophoresis* - This test is used to diagnose **hemoglobinopathies** like sickle cell disease or thalassemia, not megaloblastic anemia. - **Hypersegmented neutrophils** are not associated with hemoglobin variants and would not be corrected by identifying hemoglobinopathies.

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.

Question 1

A nine-month-old boy presented with progressive lethargy, irritability, and pallor since 6 months of age. Examination revealed severe pallor. Investigations showed Hb 3.8 mg/dL, MCV 58 fL, MCH 19.4 pg/cell. Blood film shows normal osmotic fragility, target cells, and normoblasts. X-ray skull shows expansion of the erythroid marrow. Which of the following is the most likely diagnosis?

Accepted Answer

Iron deficiency anemia

Answer

Acute lymphoblastic leukemia

Answer

Hemoglobin D disease

Answer

Hereditary spherocytosis

Question 2

In infants, from which bone is a bone marrow biopsy typically performed?

Accepted Answer

Tibia

Answer

Sternum

Answer

Posterior superior iliac spine

Answer

Iliac crest

Question 3

An 18-month-old boy presented with a tender, swollen, warm right knee with significant hemarthrosis after an injury. His PT is 12 seconds (normal, 13 seconds), PTT is over 100 seconds (normal, 25 seconds), platelet count is 300,000/mm
3, and bleeding time is normal. What should be the initial management?

Accepted Answer

Factor VIII concentrate

Answer

Fresh-frozen plasma

Answer

Aspiration of knee

Answer

Passive exercise

Question 4

An otherwise healthy 1-year-old child has a routine CBC revealing polymorphonuclear neutrophils as shown below. Which of the following is the most appropriate next step?

Accepted Answer

Begin folic acid supplementation

Answer

Bone marrow aspiration

Answer

Begin oral iron therapy

Answer

Hemoglobin electrophoresis

Question 5

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 6

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 7

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 8

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 9

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 10

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

Hematology — MCQs

Hematology — MCQs

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