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Necrobiotic xanthogranuloma: a 30-year single-center experience.
Ann Hematol. 2018 Aug; 97(8):1471-1479.AH

Abstract

To characterize the clinical features, associated disorders, and treatment of necrobiotic xanthogranuloma (NXG), a rare non-Langerhans cell histiocytosis, we conducted a retrospective review of pathologically confirmed NXG at Mayo Clinic Arizona from 1987 to June 2017. Data on clinical findings, laboratory findings, associated disorders, therapy, and response to therapy were extracted. Nineteen patients were identified. Mean age was 54 years (range, 17-84) with equal gender distribution. Median follow-up was 5.5 years (range, 1-18). Most patients had a detectable monoclonal protein (84%), and IgG kappa constituted 58%. The most common cutaneous lesions involved the periorbital region (53%). The majority of patients had extracutaneous manifestations, most commonly affecting the liver (32%) and the sinuses (21%). Hematologic malignancies were diagnosed in 26% of patients and included Hodgkin lymphoma, chronic lymphocytic leukemia (CLL), smoldering myeloma, and multiple myeloma. The most common treatment was chlorambucil with or without systemic corticosteroids. Response was seen in most patients (95%), and most patients received 1-3 lines of therapy (74%). NXG is a reactive histiocytic disorder that commonly involves multiple organ systems and requires a high degree of clinical suspicion for accurate diagnosis. Treatment decisions should be based on coexisting conditions and pattern of disease involvement.

Authors+Show Affiliations

Division of Hematology/Oncology, Mayo Clinic, 5777 E. Mayo Boulevard, Phoenix, AZ, 85054, USA. hilal.talal@mayo.edu.Department of Dermatology, Mayo Clinic, Phoenix, AZ, USA. Department of Laboratory Medicine and Pathology, Mayo Clinic, Phoenix, AZ, USA.Department of Dermatology, Mayo Clinic, Phoenix, AZ, USA.Division of Hematology/Oncology, Mayo Clinic, 5777 E. Mayo Boulevard, Phoenix, AZ, 85054, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29568990

Citation

Hilal, Talal, et al. "Necrobiotic Xanthogranuloma: a 30-year Single-center Experience." Annals of Hematology, vol. 97, no. 8, 2018, pp. 1471-1479.
Hilal T, DiCaudo DJ, Connolly SM, et al. Necrobiotic xanthogranuloma: a 30-year single-center experience. Ann Hematol. 2018;97(8):1471-1479.
Hilal, T., DiCaudo, D. J., Connolly, S. M., & Reeder, C. B. (2018). Necrobiotic xanthogranuloma: a 30-year single-center experience. Annals of Hematology, 97(8), 1471-1479. https://doi.org/10.1007/s00277-018-3301-1
Hilal T, et al. Necrobiotic Xanthogranuloma: a 30-year Single-center Experience. Ann Hematol. 2018;97(8):1471-1479. PubMed PMID: 29568990.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Necrobiotic xanthogranuloma: a 30-year single-center experience. AU - Hilal,Talal, AU - DiCaudo,David J, AU - Connolly,Suzanne M, AU - Reeder,Craig B, Y1 - 2018/03/22/ PY - 2017/11/30/received PY - 2018/03/12/accepted PY - 2018/3/24/pubmed PY - 2018/7/4/medline PY - 2018/3/24/entrez KW - Histiocytosis KW - Lymphoma KW - MGUS KW - Myeloma KW - NXG KW - Necrobiotic xanthogranuloma SP - 1471 EP - 1479 JF - Annals of hematology JO - Ann. Hematol. VL - 97 IS - 8 N2 - To characterize the clinical features, associated disorders, and treatment of necrobiotic xanthogranuloma (NXG), a rare non-Langerhans cell histiocytosis, we conducted a retrospective review of pathologically confirmed NXG at Mayo Clinic Arizona from 1987 to June 2017. Data on clinical findings, laboratory findings, associated disorders, therapy, and response to therapy were extracted. Nineteen patients were identified. Mean age was 54 years (range, 17-84) with equal gender distribution. Median follow-up was 5.5 years (range, 1-18). Most patients had a detectable monoclonal protein (84%), and IgG kappa constituted 58%. The most common cutaneous lesions involved the periorbital region (53%). The majority of patients had extracutaneous manifestations, most commonly affecting the liver (32%) and the sinuses (21%). Hematologic malignancies were diagnosed in 26% of patients and included Hodgkin lymphoma, chronic lymphocytic leukemia (CLL), smoldering myeloma, and multiple myeloma. The most common treatment was chlorambucil with or without systemic corticosteroids. Response was seen in most patients (95%), and most patients received 1-3 lines of therapy (74%). NXG is a reactive histiocytic disorder that commonly involves multiple organ systems and requires a high degree of clinical suspicion for accurate diagnosis. Treatment decisions should be based on coexisting conditions and pattern of disease involvement. SN - 1432-0584 UR - https://www.unboundmedicine.com/medline/citation/29568990/Necrobiotic_xanthogranuloma:_a_30_year_single_center_experience_ L2 - https://dx.doi.org/10.1007/s00277-018-3301-1 DB - PRIME DP - Unbound Medicine ER -