Clinical Pediatric Hematology-Oncology  2018;25(1):66-70

doi:10.15264/cpho.2018.25.1.66

A Case of Erdheim-Chester Disease Developed during Treatment of Leukemia in a Child.

Joon Pyo HONG 1 ; Won Ki AHN ; Joo Yeon LIM ; Jo Eun JUNG ; Seung Min HAHN ; Jung Woo HAN ; Chuhl Joo LYU

Affiliations

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Keywords

Erdheim-Chester disease; Acute lymphoblastic leukemia; Child

Country

Republic of Korea

Language

English

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhan's cell histiocytosis disorder characterized by replacement of normal tissue by lipid-laden histiocytes affecting various organs. A few pediatric cases have been reported worldwide. Here we present a child with leukemia who was diagnosed as ECD. A 2-year and 11-month old boy diagnosed with high risk acute lymphoblastic leukemia (ALL) at the age of 17 months, received allogeneic hematopoietic stem cell transplantation (HSCT) at the age of 2 years old. Six months after the transplantation, the patient was admitted to the hospital with palpable left calf nodules. Bone marrow study suggested ECD without leukemia with complete chimerism status. Excisional biopsy of the left calf nodule showed ‘aggregation of non-Langerhan's cell type epitheloid histiocytes’; clinically suggestive of ECD. The patient was started on vinblastine and corticosteroid treatment.