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Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia.
Am J Clin Dermatol. 2010; 11(1):67-71.AJ

Abstract

The development of xanthogranulomas has been linked to hematologic malignancies in children and adults, based on a number of reports in the literature. In children, a specific association between juvenile xanthogranuloma, neurofibromatosis 1, and juvenile myelomonocytic leukemia has been described. We report a case of a 9-month-old child, without a known diagnosis of neurofibromatosis 1, who presented with hepatosplenomegaly, anemia, thrombocytopenia, and multiple cutaneous nodules, which were confirmed to be juvenile xanthogranulomas upon biopsy. A concurrent work-up showed that the child had juvenile myelomonocytic leukemia. Although cutaneous juvenile xanthogranulomas are benign lesions, in several reported cases they have been shown to herald leukemia. This association between xanthogranulomas and hematologic malignancy is poorly understood. Juvenile xanthogranulomas have a number of morphologic variants and clinical presentations that can be confused with the cutaneous lesions of Langerhans cell histiocytosis and dermatofibroma. Recognition of the broad clinicopathologic spectrum of juvenile xanthogranulomas is critical for proper diagnosis.

Authors+Show Affiliations

Department of Pathology, Evanston Northwestern Healthcare, Evanston, Illinois, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

20000879

Citation

Cham, Elaine, et al. "Cutaneous Xanthogranulomas, Hepatosplenomegaly, Anemia, and Thrombocytopenia as Presenting Signs of Juvenile Myelomonocytic Leukemia." American Journal of Clinical Dermatology, vol. 11, no. 1, 2010, pp. 67-71.
Cham E, Siegel D, Ruben BS. Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia. Am J Clin Dermatol. 2010;11(1):67-71.
Cham, E., Siegel, D., & Ruben, B. S. (2010). Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia. American Journal of Clinical Dermatology, 11(1), 67-71. https://doi.org/10.2165/11311130-000000000-00000
Cham E, Siegel D, Ruben BS. Cutaneous Xanthogranulomas, Hepatosplenomegaly, Anemia, and Thrombocytopenia as Presenting Signs of Juvenile Myelomonocytic Leukemia. Am J Clin Dermatol. 2010;11(1):67-71. PubMed PMID: 20000879.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia. AU - Cham,Elaine, AU - Siegel,Dawn, AU - Ruben,Beth S, PY - 2009/12/17/entrez PY - 2009/12/17/pubmed PY - 2010/4/3/medline SP - 67 EP - 71 JF - American journal of clinical dermatology JO - Am J Clin Dermatol VL - 11 IS - 1 N2 - The development of xanthogranulomas has been linked to hematologic malignancies in children and adults, based on a number of reports in the literature. In children, a specific association between juvenile xanthogranuloma, neurofibromatosis 1, and juvenile myelomonocytic leukemia has been described. We report a case of a 9-month-old child, without a known diagnosis of neurofibromatosis 1, who presented with hepatosplenomegaly, anemia, thrombocytopenia, and multiple cutaneous nodules, which were confirmed to be juvenile xanthogranulomas upon biopsy. A concurrent work-up showed that the child had juvenile myelomonocytic leukemia. Although cutaneous juvenile xanthogranulomas are benign lesions, in several reported cases they have been shown to herald leukemia. This association between xanthogranulomas and hematologic malignancy is poorly understood. Juvenile xanthogranulomas have a number of morphologic variants and clinical presentations that can be confused with the cutaneous lesions of Langerhans cell histiocytosis and dermatofibroma. Recognition of the broad clinicopathologic spectrum of juvenile xanthogranulomas is critical for proper diagnosis. SN - 1175-0561 UR - https://www.unboundmedicine.com/medline/citation/20000879/Cutaneous_xanthogranulomas_hepatosplenomegaly_anemia_and_thrombocytopenia_as_presenting_signs_of_juvenile_myelomonocytic_leukemia_ L2 - https://dx.doi.org/10.2165/11311130-000000000-00000 DB - PRIME DP - Unbound Medicine ER -