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Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies.
Clin Lymphoma Myeloma Leuk 2016; 16(8):447-52CL

Abstract

INTRODUCTION

Necrobiotic xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with a monoclonal gammopathy.

PATIENTS AND METHODS

The present report describes the findings from a single tertiary medical center retrospective study, including the clinical features of 35 patients with NXG and monoclonal gammopathy from 2000 to 2015 and their subsequent disease course and treatment response. The median age at diagnosis was 56 years (range, 26-88 years).

RESULTS

Most patients had a plasma cell dyscrasia consisting of monoclonal gammopathy of undetermined significance in 28 patients and smoldering multiple myeloma in 5 patients; the remaining 2 patients had chronic lymphocytic leukemia. An IgG isotype of monoclonal gammopathy was present in almost all the patients (97%). The most common site of cutaneous involvement of NXG was periorbital (66%). The treatments were heterogeneous and included excision, intralesional injection, radiotherapy, and systemic chemotherapy. The median follow-up period was 46 months (range, 4 to 234 months). The median overall survival had not been reached at the analysis, and 80% of the patients were still alive. Eight patients (23%) had disease progression to multiple myeloma at a median of 67 months (range, 21 to 107 months), demonstrating that although the clinical course is generally indolent, malignant transformation is not uncommon. At the last follow-up visit, 80% had signs of either clinical improvement or stable skin disease.

CONCLUSION

Cutaneous objective responses can be achieved with treatment of lymphoplasmacytic malignancies.

Authors+Show Affiliations

Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN.Divisions of Hematology and Blood and Marrow Transplantation, Mayo Clinic, Rochester, MN. Electronic address: gonsalves.wilson@mayo.edu.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

27238425

Citation

Higgins, Larissa S., et al. "Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies." Clinical Lymphoma, Myeloma & Leukemia, vol. 16, no. 8, 2016, pp. 447-52.
Higgins LS, Go RS, Dingli D, et al. Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies. Clin Lymphoma Myeloma Leuk. 2016;16(8):447-52.
Higgins, L. S., Go, R. S., Dingli, D., Kumar, S. K., Rajkumar, S. V., Dispenzieri, A., ... Gonsalves, W. I. (2016). Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies. Clinical Lymphoma, Myeloma & Leukemia, 16(8), pp. 447-52. doi:10.1016/j.clml.2016.04.009.
Higgins LS, et al. Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies. Clin Lymphoma Myeloma Leuk. 2016;16(8):447-52. PubMed PMID: 27238425.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies. AU - Higgins,Larissa S, AU - Go,Ronald S, AU - Dingli,David, AU - Kumar,Shaji K, AU - Rajkumar,S Vincent, AU - Dispenzieri,Angela, AU - Buadi,Francis K, AU - Lacy,Martha Q, AU - Lust,John A, AU - Kapoor,Prashant, AU - Leung,Nelson, AU - Lin,Yi, AU - Kourelis,Taxiarchis V, AU - Gertz,Morie A, AU - Kyle,Robert A, AU - Gonsalves,Wilson I, Y1 - 2016/05/05/ PY - 2016/02/25/received PY - 2016/03/29/revised PY - 2016/04/26/accepted PY - 2016/5/31/entrez PY - 2016/5/31/pubmed PY - 2017/11/4/medline KW - Dermatologic manifestations KW - Monoclonal gammopathies KW - NXG KW - Novel agent therapies KW - Progression SP - 447 EP - 52 JF - Clinical lymphoma, myeloma & leukemia JO - Clin Lymphoma Myeloma Leuk VL - 16 IS - 8 N2 - INTRODUCTION: Necrobiotic xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with a monoclonal gammopathy. PATIENTS AND METHODS: The present report describes the findings from a single tertiary medical center retrospective study, including the clinical features of 35 patients with NXG and monoclonal gammopathy from 2000 to 2015 and their subsequent disease course and treatment response. The median age at diagnosis was 56 years (range, 26-88 years). RESULTS: Most patients had a plasma cell dyscrasia consisting of monoclonal gammopathy of undetermined significance in 28 patients and smoldering multiple myeloma in 5 patients; the remaining 2 patients had chronic lymphocytic leukemia. An IgG isotype of monoclonal gammopathy was present in almost all the patients (97%). The most common site of cutaneous involvement of NXG was periorbital (66%). The treatments were heterogeneous and included excision, intralesional injection, radiotherapy, and systemic chemotherapy. The median follow-up period was 46 months (range, 4 to 234 months). The median overall survival had not been reached at the analysis, and 80% of the patients were still alive. Eight patients (23%) had disease progression to multiple myeloma at a median of 67 months (range, 21 to 107 months), demonstrating that although the clinical course is generally indolent, malignant transformation is not uncommon. At the last follow-up visit, 80% had signs of either clinical improvement or stable skin disease. CONCLUSION: Cutaneous objective responses can be achieved with treatment of lymphoplasmacytic malignancies. SN - 2152-2669 UR - https://www.unboundmedicine.com/medline/citation/27238425/Clinical_Features_and_Treatment_Outcomes_of_Patients_With_Necrobiotic_Xanthogranuloma_Associated_With_Monoclonal_Gammopathies_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2152-2650(16)30052-0 DB - PRIME DP - Unbound Medicine ER -