Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation.

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy.

Threshold of galactomannan antigenemia positivity for early diagnosis of invasive aspergillosis in neutropenic children.

Purpose: Invasive aspergillosis (IA) is an important cause of morbidity and mortality in immunocompromised patients. Pediatric data on the accuracy and optimal cutoff of galactomannan antigen detection to diagnose IA is sparse and controversial. We evaluated the utility and optimal serum galactomannan assay (GA) cutoff in children.

Recombinant activated factor VII usage in life threatening hemorrhage: a pediatric experience.

Objective: To determine the safety and efficacy of off label usage of Recombinant activated factor VII (rFVIIa) in children with severe bleeding in non-hemophiliac children with diverse etiologies like leukemia, post hematopoietic stem cell transplantation, dengue shock syndrome and Glanzmann thrombasthenia.

Method: Medical records of 16 non-hemophiliac children with 20 bleeding episodes where rFVIIa was administered only after failure of standard measures to control bleeding were retrospectively reviewed and data collected regarding patient demographics, diagnosis and location of bleeding. Blood counts, coagulation and other lab parameters, both pre and post rFVIIa, were also noted.