Infiltrating the Heart and Kidney: A Rare Pediatric Case of Multisystem Langerhans Cell Histiocytosis from Pakistan

Abstract

Langerhans cell histiocytosis (LCH) is a rare, clonal disease of the monocyte-macrophage system, varying in its clinical presentation from mere self-healing skin and bone lesions to life-threatening multi-system disease. In descending order of frequency, the disease is known to involve the skeleton, skin, lymph nodes and lesser often, the liver, spleen, lungs, hematopoietic and central nervous systems. Here, we present a pediatric case of multi-system LCH in a five-year-old child, unique in its evident cardiac and renal involvement alongside other organ systems and important in how the diagnosis was aided by a fine needle aspiration cytology instead of the costlier histopathological procedures, in a setting with limited resources.

Pericardial Effusion in a Pediatric Patient with Langerhans-Cell Histiocytosis (LCH): A Case Report and Review of an Atypical Presentation of Multisystemic LCH

“…It is important to mention two other case reports of patients with LCH which had cardiac involvement but didn't develop pericardial effusion. 14,15 All patients that had treatment responded to chemotherapy without relapses of pericardial effusion. All patients that had followup survived.…”