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

Fingerprint

Juvenile Xanthogranuloma
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Bone Marrow
Bone and Bones
Skin
Maintenance Chemotherapy
Non-Langerhans-Cell Histiocytosis
Therapeutics
6-Mercaptopurine
Histiocytes
S100 Proteins
Hip Joint
Arthralgia
Vincristine
Giant Cells
Knee Joint
Scalp
Methotrexate
Dexamethasone
Leukemia

Keywords

  • bone marrow
  • juvenile xanthogranuloma
  • lymphoblastic leukemia
  • systemic

ASJC Scopus subject areas

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

Cite this

Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State. / Cheon, Eunjae; Yang, Saemi; Han, Jae Ho; Lee, Kwang Chul; Park, Jun Eun.

In: Pediatric and Developmental Pathology, Vol. 21, No. 5, 01.09.2018, p. 489-493.

Research output: Contribution to journalArticle

@article{8453ea903dc84a14b609a86b1ac90e32,
title = "Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State",
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.",
keywords = "bone marrow, juvenile xanthogranuloma, lymphoblastic leukemia, systemic",
author = "Eunjae Cheon and Saemi Yang and Han, {Jae Ho} and Lee, {Kwang Chul} and Park, {Jun Eun}",
year = "2018",
month = "9",
day = "1",
doi = "10.1177/1093526617721775",
language = "English",
volume = "21",
pages = "489--493",
journal = "Pediatric and Developmental Pathology",
issn = "1093-5266",
publisher = "Society for Pediatric Pathology",
number = "5",

}

TY - JOUR

T1 - Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State

AU - Cheon, Eunjae

AU - Yang, Saemi

AU - Han, Jae Ho

AU - Lee, Kwang Chul

AU - Park, Jun Eun

PY - 2018/9/1

Y1 - 2018/9/1

N2 - 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.

AB - 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.

KW - bone marrow

KW - juvenile xanthogranuloma

KW - lymphoblastic leukemia

KW - systemic

UR - http://www.scopus.com/inward/record.url?scp=85053913389&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85053913389&partnerID=8YFLogxK

U2 - 10.1177/1093526617721775

DO - 10.1177/1093526617721775

M3 - Article

C2 - 28836893

AN - SCOPUS:85053913389

VL - 21

SP - 489

EP - 493

JO - Pediatric and Developmental Pathology

JF - Pediatric and Developmental Pathology

SN - 1093-5266

IS - 5

ER -