Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies

Larissa S. Higgins, Ronald S. Go, David M Dingli, Shaji K Kumar, S Vincent Rajkumar, Angela Dispenzieri, Francis K. Buadi, Martha Lacy, John A. Lust, Prashant Kapoor, Nelson Leung, Yi Lin, Taxiarchis Kourelis, Morie Gertz, Robert A. Kyle, Wilson Gonsalves

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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.

Original languageEnglish (US)
JournalClinical Lymphoma, Myeloma and Leukemia
DOIs
StateAccepted/In press - Feb 25 2016

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Necrobiotic Xanthogranuloma
Paraproteinemias
Multiple Myeloma
Skin
Intralesional Injections
Monoclonal Gammopathy of Undetermined Significance
B-Cell Chronic Lymphocytic Leukemia
Skin Diseases
Disease Progression
Radiotherapy
Therapeutics
Retrospective Studies

Keywords

  • Dermatologic manifestations
  • Monoclonal gammopathies
  • Novel agent therapies
  • NXG
  • Progression

ASJC Scopus subject areas

  • Cancer Research
  • Hematology
  • Oncology

Cite this

@article{c27b9edcad0645b1916e11221c6634d8,
title = "Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies",
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.",
keywords = "Dermatologic manifestations, Monoclonal gammopathies, Novel agent therapies, NXG, Progression",
author = "Higgins, {Larissa S.} and Go, {Ronald S.} and Dingli, {David M} and Kumar, {Shaji K} and Rajkumar, {S Vincent} and Angela Dispenzieri and Buadi, {Francis K.} and Martha Lacy and Lust, {John A.} and Prashant Kapoor and Nelson Leung and Yi Lin and Taxiarchis Kourelis and Morie Gertz and Kyle, {Robert A.} and Wilson Gonsalves",
year = "2016",
month = "2",
day = "25",
doi = "10.1016/j.clml.2016.04.009",
language = "English (US)",
journal = "Clinical Lymphoma, Myeloma and Leukemia",
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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 M

AU - Kumar, Shaji K

AU - Rajkumar, S Vincent

AU - Dispenzieri, Angela

AU - Buadi, Francis K.

AU - Lacy, Martha

AU - Lust, John A.

AU - Kapoor, Prashant

AU - Leung, Nelson

AU - Lin, Yi

AU - Kourelis, Taxiarchis

AU - Gertz, Morie

AU - Kyle, Robert A.

AU - Gonsalves, Wilson

PY - 2016/2/25

Y1 - 2016/2/25

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.

AB - 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.

KW - Dermatologic manifestations

KW - Monoclonal gammopathies

KW - Novel agent therapies

KW - NXG

KW - Progression

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U2 - 10.1016/j.clml.2016.04.009

DO - 10.1016/j.clml.2016.04.009

M3 - Article

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JO - Clinical Lymphoma, Myeloma and Leukemia

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SN - 2152-2669

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