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Hemophagocytic lymphohistiocytosis (HLH) is a rare, multisystem, potentially fatal clinicopathologic syndrome. HLH presenting predominantly as pediatric acute liver failure (PALF) has been rarely reported. Early recognition is imperative to initiate life-saving treatment but is often hampered due to the rarity of this syndrome, variable clinical presentations, and nonspecific clinical and laboratory findings. In this article, we reported a case of secondary HLH (H1N1 and RSV positive) presenting as PALF from India. A previously healthy 22-month-old boy presented with fever, vomiting, and altered sensorium for 10 days. He had coagulopathy and deranged liver functions. He was evaluated for underlying etiology and managed on lines of PALF. Due to persistent bicytopenia and excessively high ferritin levels, HLH was strongly suspected though he did not fulfill all clinical criteria for the diagnosis of HLH. Presence of seizures and cerebrospinal fluid analysis was suggestive of central nervous system involvement. There was no evidence of primary HLH on genetic evaluation. Real-time polymerase chain reaction amplifications were positive for RSV and influenza A H1N1, confirming the causative triggers. After the administration of immunosuppressants and oseltamivir, the patient's symptoms improved dramatically and he recovered completely. To the best of our knowledge, this is the fourth case reported worldwide till date of successful rescue of ALF in a child associated with HLH completely without resorting to liver transplantation. Clinical vigilance is crucial for possible presence of HLH with varied initial presentations in PALF despite incomplete diagnostic criteria, with detailed etiological workup for commencing life-saving therapy in time.

Medulloblastoma (MB) is the most common malignant tumor of the central nervous system in children with up to a third of these tumors presenting in children under 3 years of age. Its exquisite radio and chemosensitivity renders high cure rates in children in whom optimal resection has been achieved. Optimal surgery followed by radiation alone can cure about half of these children. The addition of chemotherapy has improved the outcomes dramatically and over 70% of children over 3 years of age with optimal resection and no metastasis can expect to be cured. Increasingly, the focus is on limiting the long-term sequelae of treatment. Precise molecular characterization can enable us to identify patients who can achieve optimal outcomes even in the absence of radiation. Insights into disease biology and molecular characterization have led to dramatic changes in our understanding, risk stratification, prognostication, and treatment approach in these children. In India, there is limited access to molecular profiling, making it challenging to apply biology driven approach to treatment in each child with MB. The Indian Society of Neuro-Oncology guidelines and the SIOP PODC adapted treatment recommendations for standard-risk MB based on the current evidence and logistic realities of low-middle income countries are a useful adjunct to guide clinical practice on a day-to-day basis in our setting.

Introduction: The management of patients with aplastic anemia, to an extent, depends on the etiology i.e., inherited or acquired. The classical Chromosomal breakage study involves detection of chromosomal breakage or aberrations (breaks, gaps, rearrangements, radials, exchanges, endoreduplications) in peripheral blood cells after culture with a T-cell mitogen and a DNA clastogenic (cross-linking) agent, such as diepoxybutane (DEB) or mitomycin C (MMC). The testing needs to be performed in laboratory with appropriate expertise in Fanconi Anemia testing. The present study was undertaken to find out the frequency of inherited aplastic anemia in North India. Materials and Methods: This study was carried out at the Department of Molecular Biology and Transplant Immunology, Indraprastha Apollo hospital, New Delhi. The study includes retrospective analysis of 528 aplastic anemia patients whose samples were tested at our department for Chromosomal breakage study during the period 2007 to 2011. Respective age and sex matched healthy controls were also processed for chromosomal breakage study. Patient′s habitat, clinical symptoms, differential blood count and history of drug exposure were documented for all patients referred to us, whereever available. Relative risk was estimated by odds ratio (OR) with 95% confidence interval (CI) in matched cases and controls. Conclusion: A significant increase in chromosomal breakages was seen in 13.1% patients. The survival data documented for 100 patients suggested 60% mortality.