A 10-year-old girl presents to the emergency with high grade fever of 2-weeks duration and rashes over lower limbs for the last 7 days. Examination reveals mild pallor, generalized petechiae and hepatosplenomegaly. Investigations reveal: Hemoglobin 9 g/dL, total leucocyte count 2100/mm3, platelet count 20,000/mm3, peripheral smear shows pancytopenia, SGOT 50 IU/dL, SGPT 64 IU/dL, serum ferritin 1200 microgram/L, serum fibrinogen 100 mg/dL and serum triglycerides of 350 mg/dL. The most likely diagnosis is:
Hemophagocytic lymphohistiocytosis
Prolonged fever, hepatosplenomegaly, pancytopenia, hyperferritinemia, hypofibrinogenemia and hypertriglyceridemia fulfil the HLH-2004 diagnostic criteria for Hemophagocytic lymphohistiocytosis (HLH). Aplastic anemia does not cause hepatosplenomegaly or hyperferritinemia to this degree, and ITP would not explain the pancytopenia and organomegaly.
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Important Questions from Paediatric Emergencies
- Which of the following manifestations is/are NOT seen in breath holding spells?
A 6-year old child needs to undergo a central Catheter placement for total parental nutrition.The procedure is likely to evoke pain and anxiety. Which of the following is the most appropriate drug for sedation and analgesia in this child?
Which one of the following is the drug of choice in a child presenting with supraventricular tachycardia?
Which one of the following drugs is used in cardiopulmonary resuscitation for pulseless ventricular fibrillation?
A 4-week old infant presents with repeated episodes of non-bilious vomiting since 7 days.The infant seems active and hungry after the vomiting. Examination reveals an olive-shaped mass in the epigartrium. Which metabolic abnormality is expected in this infant?
