Page 32 of 34
PE26.1-17 | Hemato-Oncology — Graded Quiz
Click any question card to reveal the correct answer.
A 14-month-old boy presents with pallor. Hb 8.1 g/dL, MCV 66 fL. Serum ferritin 6 ng/mL, serum iron 38 µg/dL, TIBC 540 µg/dL. Mentzer index is 14. A trial of oral iron is started. After 4 weeks, Hb rises to 9.8 g/dL. What does this response CONFIRM?
A therapeutic response to oral iron (Hb rise >1 g/dL per week or >2 g/dL in 4 weeks) confirms the diagnosis of IDA. Low ferritin, low serum iron, elevated TIBC, and Mentzer index >13 all support IDA. Continue oral iron for 3 months after Hb normalisation to replenish stores.
Diagnosis of IDA is confirmed by a therapeutic trial response: Hb rise >1 g/dL per week. Continue iron for 3 months post-correction. If no response, exclude thalassaemia trait, occult blood loss, or malabsorption.
Mentzer index >13 favours IDA over thalassaemia trait — not thalassaemia (A). Electrophoresis (C) is needed if the response to iron is absent/poor or if risk factors for thalassaemia trait are present. Parenteral iron (D) is reserved for genuine oral iron intolerance or malabsorption.
Click to reveal answer
A 9-month-old infant weighing 9 kg, attending an ICDS centre, is found to have Hb 9.4 g/dL with microcytic hypochromic anaemia. She is otherwise well and consuming mixed diet. Under the National Anaemia Control Programme, what is the recommended management?
The National Anaemia Control Programme (NACP) under the National Nutritional Anaemia Prophylaxis Programme recommends iron prophylaxis for children 6–60 months. For mild-moderate anaemia (Hb 8–11 g/dL), the programme provides iron supplementation through ICDS and school health programmes. The therapeutic vs prophylactic dose depends on severity.
National Anaemia Control Programme: weekly IFA supplementation for children 1–5 years (45 mg elemental iron + 400 mcg folic acid weekly) through ICDS. Aims to reduce nutritional anaemia prevalence. Screen Hb at ICDS visits; treat overt IDA with therapeutic doses.
Therapeutic dose (A) would be used for confirmed IDA, but under NACP, programme-based supplementation at ICDS is the system response for mild anaemia in well children. Transfusion (C) is not indicated for Hb 9.4 g/dL in a well child. Anaemia is not 'normal' and requires intervention (D).
Click to reveal answer
A 10-year-old boy with hereditary spherocytosis presents with increasing pallor, splenomegaly, and episodic jaundice. His sibling also has HS. Which investigation MOST specifically confirms the diagnosis of hereditary spherocytosis?
Hereditary spherocytosis is caused by defects in red cell membrane proteins (spectrin, ankyrin, band 3). Osmotic fragility test (OFT) demonstrates increased lysis in hypotonic solutions. The EMA binding test (eosin-5-maleimide) is more sensitive and specific, detecting reduced band 3 by flow cytometry.
Hereditary spherocytosis: autosomal dominant membrane protein defect (spectrin, ankyrin, band 3). Spherocytes on smear, increased osmotic fragility, DAT negative. Treat severe cases with splenectomy (but vaccinate against encapsulated organisms first).
HS shows spherocytes (not hypochromic microcytes) on smear (A). DAT is positive in autoimmune haemolytic anaemia, not HS (B). HbF elevation occurs in thalassaemia major (D).
Click to reveal answer
A 12-year-old boy with severe haemophilia A (Factor VIII < 1%) sustains a fall and develops a right knee haemarthrosis. He is in significant pain. What is the MOST appropriate immediate management?
Haemarthrosis in haemophilia A requires immediate Factor VIII replacement to halt bleeding. Target: raise factor level to 40–50% for joint bleeds (use weight-based dosing: units of FVIII = % rise desired × weight [kg] ÷ 2). Analgesics: paracetamol or codeine. Strictly avoid NSAIDs, aspirin, and IM injections.
Haemophilia A: haemarthrosis requires immediate Factor VIII (target 40–50%). NEVER give IM injections or aspirin/NSAIDs. RICE + factor + safe analgesia (paracetamol/codeine). Early treatment prevents target joint formation.
IM injections (A) are absolutely contraindicated in haemophilia — risk of severe haematoma. Aspirin (B) inhibits platelet function and worsens bleeding. Fresh frozen plasma (B) is suboptimal — factor concentrate is preferred. Delaying factor infusion (D) allows joint damage to progress — treat immediately.
Click to reveal answer
A 3-year-old girl presents with pallor, fatigue, and gum hypertrophy. Peripheral smear shows 40% blast cells. Flow cytometry confirms B-cell lineage ALL. Initial WBC is 85,000/µL. Which monitoring parameter is MOST critical in the first 72 hours of chemotherapy?
High WBC ALL (>50,000/µL) is high risk for tumour lysis syndrome when chemotherapy is initiated. Massive blast lysis releases: hyperkalaemia, hyperphosphataemia, hyperuricaemia, and secondary hypocalcaemia → acute kidney injury, cardiac arrhythmia, and death. Monitor electrolytes 4–6 hourly for 72 hours with aggressive hydration and allopurinol/rasburicase prophylaxis.
TLS risk in ALL is highest with WBC >50,000/µL. Prophylaxis: hyperhydration (3 L/m²/day), allopurinol; rasburicase for very high risk. Monitor electrolytes 4–6 hourly in first 72 hours. Avoid nephrotoxins. TLS = hyperkalaemia + hyperphosphataemia + hyperuricaemia + hypocalcaemia.
Chest X-rays (A) detect pneumonia or mediastinal mass but are not the most critical TLS monitor. Daily bone marrow aspirates (C) are not performed during induction — response is assessed at Day 29. Platelet transfusion (D) is given for active bleeding or platelets <10,000, not prophylactically every 8 hours.
Click to reveal answer
A 15-year-old girl presents with left upper quadrant discomfort and splenomegaly. Peripheral smear shows target cells, HbS 80%, HbA2 3%, HbF 15% on HPLC. She has had one previous episode of painful crisis. She is now asymptomatic and stable. Which long-term prophylactic medication is indicated?
All children with sickle cell disease (HbSS) require penicillin V prophylaxis (125 mg twice daily until age 5, then 250 mg twice daily) and pneumococcal vaccination because functional asplenia develops early due to autoinfarction of the spleen. This dramatically reduces pneumococcal sepsis mortality.
Sickle cell disease: functional asplenia → overwhelming sepsis risk (Streptococcus pneumoniae, H. influenzae, Salmonella). Prophylaxis: penicillin V from diagnosis, pneumococcal + meningococcal + Hib vaccines. Iron NOT indicated — haemolysis causes iron recycling not depletion.
Monthly transfusions (A) are indicated for primary stroke prevention (TCD-confirmed high risk) or recurrent ACS, not routine prophylaxis. Iron supplementation (C) is contraindicated in sickle cell disease as iron overload worsens with haemolysis. Aspirin (D) is not used routinely in sickle cell; antiplatelet therapy in children carries Reye's syndrome risk.
Click to reveal answer
A 14-year-old boy presents with cervical lymphadenopathy, fever, and weight loss. A lymph node biopsy shows a polymorphous infiltrate with Reed-Sternberg cells. CT scan shows disease limited to cervical and mediastinal nodes with no infradiaphragmatic involvement. Which staging is correct and which treatment approach applies?
Ann Arbor staging: Stage I = one lymph node region; Stage II = two or more regions on SAME side of diaphragm; Stage III = both sides of diaphragm; Stage IV = diffuse extranodal involvement. This patient has cervical + mediastinal nodes (two supra-diaphragmatic regions) = Stage II. Standard treatment: ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) ± involved-field radiotherapy.
Hodgkin lymphoma staging (Ann Arbor): I=single region, II=≥2 regions same side of diaphragm, III=both sides, IV=diffuse extranodal. Treatment: ABVD-based chemotherapy ± involved-field RT. Cure rates >85% overall, >90% for early stages.
Stage IV (A) requires diffuse extranodal spread. Stage I (C) is a single nodal region — this patient has two regions. CHOP (D) is used for B-cell NHL (lymphomas), not Hodgkin lymphoma.
Click to reveal answer
A 6-year-old boy is investigated for pallor. His peripheral smear shows target cells, hypochromic microcytes, and nucleated RBCs. HPLC reveals HbA 0%, HbA2 6.5%, HbF 92%. What is the MOST important indication for splenectomy in this child?
In thalassaemia major, splenectomy is indicated when blood transfusion requirements increase substantially (>200–250 mL/kg/year of packed cells) due to hypersplenism — i.e., the spleen is destroying transfused blood. Splenectomy must follow complete vaccination against encapsulated organisms (pneumococcus, meningococcus, Hib) and is generally deferred until age >5 years to minimise post-splenectomy sepsis risk.
Splenectomy in thalassaemia major: indicated for increasing transfusion requirements (>200–250 mL/kg/year). Defer until age >5. Pre-op: pneumococcal, meningococcal, Hib vaccines. Post-op: lifelong penicillin prophylaxis. Not indicated for isolated elevated ferritin.
Elevated ferritin (B) is an indication for chelation, not splenectomy. HbA2 >3.5% (C) is diagnostic of beta-thalassaemia trait — not an indication for splenectomy. Asymptomatic massive splenomegaly at age 3 (D) alone is not an indication — splenectomy is deferred until >5 years.
Click to reveal answer
A 9-year-old girl with chronic ITP presents with platelet count persistently at 18,000–25,000/µL for 8 months. She has minor petechiae but no mucosal or significant bleeding. What is the MOST appropriate management at this stage?
Chronic ITP (> 12 months in adults; by convention >6 months in children) with platelet count 18,000–25,000/µL and ONLY petechiae (no mucous membrane, internal, or severe bleeding) is managed by watchful waiting. Activity restriction (avoid contact sports, IM injections) reduces major bleeding risk. Most children with chronic ITP have spontaneous remission over time.
Chronic ITP (>6 months) with dry purpura (petechiae only) and platelets >10,000: watchful waiting. Avoid contact sports and NSAIDs. Treat acute bleeding episodes with IVIG or steroids. Splenectomy and TPO agonists (eltrombopag) reserved for refractory cases in older children/adolescents.
Platelet transfusions (A) are used only for life-threatening or severe mucosal bleeding — they are rapidly destroyed in ITP. Splenectomy (C) is a later option for true chronic refractory ITP, typically deferred until puberty/adulthood in children. Long-term steroids (D) cause growth suppression, osteoporosis, Cushingoid effects — not appropriate for chronic management at this platelet level with no bleeding.
Click to reveal answer
A parent whose 2-year-old child has been diagnosed with IDA asks about prevention of anaemia in the family's new baby currently aged 4 months. The baby is exclusively breastfed. Which MOST appropriate prevention advice should be given?
Exclusively breastfed term infants require iron supplementation from 4 months because breast milk iron (0.5 mg/L), while well absorbed, is insufficient beyond 4 months as fetal stores deplete. WHO/IAP recommends iron drops 1 mg/kg/day from 4 months for breastfed infants, followed by iron-rich complementary foods from 6 months.
IDA prevention in breastfed infants: iron drops 1 mg/kg/day from 4 months of age (term infants); iron-rich complementary foods from 6 months. Preterm/LBW infants need earlier supplementation (2 mg/kg/day from 2 months). Counsel families about dietary iron sources and cooking in iron utensils.
Replacing breast milk with formula (A) is contraindicated — breast milk is superior and WHO recommends exclusive breastfeeding until 6 months. Breast milk iron is inadequate beyond 4 months (C) — term iron stores deplete around 4–6 months. Delaying complementary foods until 12 months (D) is incorrect — introduce at 6 months.
Click to reveal answer