Acute lymphoblastic leukemia masquerading as hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a condition characterized by immunological imbalance due to inappropriate activation of macrophage, T/NK cells resulting in hypercytokinemia and subsequent tissue damage. We present an interesting case of acute lymphoblastic leukemia (ALL) who presented to us with clinical and laboratory features of HLH. High index of suspicion for malignancy based on clinical history and bone marrow examination led us to reach at definitive diagnosis of ALL.

Gut and liver involvement in pediatric hematolymphoid malignancies.

Hematolymphoid malignancies are common neoplasms in childhood. The involvement of the gastrointestinal (GI) tract, liver, biliary system, pancreas, and peritoneum are closely interlinked and commonly encountered. In leukemias, lymphomas, and Langerhans cell histiocytosis (LCH), the manifestations result from infiltration, compression, overwhelmed immune system, and chemotherapy-induced drug toxicities. In acute leukemias, major manifestations are infiltrative hepatitis, drug induced gastritis, neutropenic typhlitis and chemotherapy related pancreatitis. Chronic leukemias are rare. Additional presentation in lymphomas is cholestasis due to infiltration or biliary obstruction by lymph nodal masses. Presence of ascites needs a thorough workup for the underlying pathophysiology that may modify the therapy and affect the outcome. Uncommon hematolymphoid malignancies are primary hepatic, hepatosplenic, and GI lymphomas which have strict definitions. In advanced diseases with extensive spread, it may be impossible to distinguish these diseases from the primary site of origin. LCH produces biliary strictures that mimic as sclerosing cholangitis. Liver infiltration is associated with poor liver recovery even after chemotherapy. The heterogeneity of gut and liver manifestations in hematolymphoid malignancies has a clinical impact on their management. Though chemotherapy is the mainstay of therapy in all hematolymphoid malignancies, debulking surgery and radiotherapy have an adjuvant role in specific clinical scenarios. Rare situations presenting as liver failure or end-stage liver disease require liver transplantation. At their initial presentation to a primary care physician, given the ambiguity in clinical manifestations and the prognostic difference with time-bound management, it is vital to recognize them early for optimal outcomes. Pooled data from robust registries across the world is required for better understanding of these complications.

Basidiobolomycosis complicated by hydronephrosis and a perinephric abscess presenting as a hypertensive emergency in a 7-year-old boy.

A 7-year-old boy presented with a chronic, indurated, tender left thigh swelling in association with a hypertensive emergency. He had a bilateral moderate degree of hydronephrosis and a left perinephric abscess, and MRI features of posterior reversible encephalopathy syndrome. Histopathological examination of the biopsy specimen demonstrated eosinophilic fasciitis with filamentous fungi. Basidiobolus ranarum was isolated from the culture. The fungus was also isolated from a perinephric fluid aspirate. Computerised tomography of the abdomen demonstrated features consistent with fungal invasion of the pelvic floor muscles and urinary bladder, leading to bilateral hydronephrosis. He required multiple antihypertensive drug therapy and was treated with intravenous amphotericin B, oral itraconazole and potassium iodide. Antihypertensive agents were discontinued after 2 weeks of antifungal therapy. At 6-months follow-up, the hydronephrosis had resolved completely. Perinephric abscess associated with basidiobolomycosis has not been reported previously.

Referral Patterns and Factors Influencing Age at Admission of Infants with Cholestasis in India.

OBJECTIVES: To define the recognition, age at admission, referral time and referral pattern of neonatal cholestasis in India. METHODS: This prospective, observational study was conducted from February 2015 through March 2016 in the Pediatric gastroenterology unit of JIPMER, Pondicherry in infants with cholestasis < 6 mo of age. RESULTS: Among 64 infants, median age of admission was 52 d (IQR 28-63 d). Fifty of sixty four infants (78.1%) came with parent-reported cholestasis-related symptoms of either jaundice alone (57.8%) or bleeding manifestations (20.3%). In 21.9% infants, jaundice was detected by physicians at a median age of 45 d (IQR 38.5-53.2 d). Two infants had intracranial bleed. Only 34% infants with pale stools were identified by the mother. The median healthcare-seeking time was 5.5 d (IQR 2.5-12 d). Among infants presenting to primary healthcare physicians (PHPs) with cholestasis-related symptoms, median time to referral was 5 d (IQR 2.5-12 d). The first point of healthcare contact in 54.7% was a PHP; 17.1% PHPs had reassured the parents. Herbal preparations were prescribed by 14.3%. Only 11.8% of those with jaundice as the only problem were given vitamin K before referral. Biliary atresia (BA) was missed in neonatal intensive care units in 9 cases. CONCLUSIONS: The above issues need to be accounted for before evaluating or implementing screening strategies in India.