Clinical Profile, Treatment and Outcome of Pediatric ''Coffee Bean'' Histiocytosis: A Single Center Experience

ABSTRACT

Introduction: Langerhans Cell Histiocytosis is a group of diseases with a myriad of clinical manifestations and biological behavior, characterized by proliferation and accumulation of Langerhans cells in different organs. Aims & Objectives: This study is to demonstrate the varied clinical presentations and the treatment outcome of the children in one of the few pediatriccenters in North India that cater to various benign and malignant blood disorders of children. Material(s) and Method(s) The study describes the 4-year experience of our center in managing children with LCH. The clinical presentations, relevant laboratory, and radiological findings, treatment, and outcome of all the 14 children presenting between December 2018 and August 2022 were retrieved from our Hospital Information system. The LCH IV protocol was taken as the backbone for classification, risk organ involvement, stratum allocation, for deciding on treatment strategy, treatment response, and outcome. Result(s) A total of 14 children were diagnosed with LCH during the study period. The mean age at diagnosis was 36 months(range 10-110 months, median 34 months). The male female ratio was 95. 12 children had multisystem LCH while 2 children had single system LCH. Lymphadenopathy was the most common clinical presentation while the skeletal system was the most common system involved, affecting 64% of the children. There were a total of 8(57%) children who were in complete remission, of which 1 child expired and one relapsed, though went into the second remission. One child had a progressive disease. Three(21%) children left against medical advice while 1 child abandoned treatment 2 weeks into therapy. One of the children became covid positive after diagnosis and expired before the treatment could be initiated. Conclusion(s) The wide array of clinical manifestations warrants the need for a high index of suspicion for an earlier diagnosis of LCH. The morphological identification of Langerhan's cells and positive IHC for CD1a, S100, and/or CD207 are necessary for definitive diagnosis. All patients with LCH should undergo BRAF-V600E mutational testing to aid in diagnosis and treatment.