Grand rounds and Teaching Files - Pediatric Oncall
Fever, jaundice with emerging pancytopenia

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Clinical Problem:

Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai, India.

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056, India.

A 7 year old boy born of non-consanguineous marriage presented with fever with chills for 10 days. He had left flank pain and bilateral painful knees along with fever. There was one episode of uprolling of eyes 7 days ago which lasted for 2 minutes. He had received anti-malarials and cotrimoxazole for these complaints. There is no contact with any patient having TB. He had fungal urinary tract infection at 5 years of age and was treated with IV antibiotics, ionotropic support and antifungal agents. His milestones and immunization are normal. On examination, he had tachycardia, bilateral knee effusions with restriction of movement, pallor and insignificant cervical lymphadenopathy with no organomegaly.
His investigations initially revealed leucocytosis which then subsequently developed into pancytopenia. He developed jaundice and elevated transaminases on Day 20 of illness which then subsided by Day 25 of illness. His serial hemograms and liver function tests are depicted in Table 1. His other biochemistries showed elevated LDH and uric acid. Renal tests were normal. Other investigations in form of Widal test, Leptospira tridot, Peripheral smear for malarial parasites, OptiMAL test, Hepatitis A IgM, HBsAg, Anti Hepatitis C, Dengue IgM, HIV ELISA, Parvovirus IgM, Brucella IgM and IgG, Weil Felix test were negative. Blood bactec culture and urine culture did not show any growth. Ultrasound of abdomen and electroencephalogram were normal. Prothrombin time was 17.3 {control = 11.0 sec} and Partial Thromboplastin time = 42.5 {control = 23.6 sec}. Serum immunoglobulins, ANA and dsDNA were normal. Echocardiography did not show any vegetations. Bone marrow examination did not show malignant cells but was diagnostic of the disease.

What is the diagnosis_?

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