Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State

Eunjae Cheon, Saemi Yang, Jae Ho Han, Kwang Chul Lee, Jun Eun Park

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. We encountered a systemic JXG case involving the bone marrow, multiple bones, and the skin during acute lymphoblastic leukemia (ALL) treatment. A 16-year-old boy undergoing ALL treatment experienced unexplained prolonged fever and scalp, hip joint, and right knee joint pain for 2 weeks during interim maintenance chemotherapy. Bone marrow pathologic findings revealed no evidence of leukemia relapse but showed diffuse infiltration of histiocytes with several Touton-type giant cells; the stains were positive for CD68 and negative for CD1a and S100 protein. Bone and skin biopsies confirmed the findings. Symptoms have resolved since maintenance chemotherapy, which included vincristine, dexamethasone, 6-mercaptopurine, and methotrexate. Bone marrow involvement of JXG is very rare, occurring only in patients less than 1 year of age; however, this case was reported in an adolescent during ALL treatment.

Original languageEnglish
Pages (from-to)489-493
Number of pages5
JournalPediatric and Developmental Pathology
Volume21
Issue number5
DOIs
Publication statusPublished - 2018 Sep 1

Keywords

  • bone marrow
  • juvenile xanthogranuloma
  • lymphoblastic leukemia
  • systemic

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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