Asunto(s)
Xantogranuloma Necrobiótico/diagnóstico , Mieloma Múltiple Quiescente/diagnóstico , Anciano , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Xantogranuloma Necrobiótico/tratamiento farmacológico , Xantogranuloma Necrobiótico/etiología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Mieloma Múltiple Quiescente/complicacionesAsunto(s)
Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Mieloma Múltiple/complicaciones , Enfermedades de la Piel/patología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Células de la Médula Ósea/patología , Femenino , Humanos , Inmunoglobulina G/análisis , Mieloma Múltiple/diagnóstico por imagen , Mieloma Múltiple/tratamiento farmacológico , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/etiología , Paraproteinemias , Células Plasmáticas/patología , Tomografía Computarizada por Rayos X/métodosAsunto(s)
Xantogranuloma Necrobiótico/diagnóstico , Paraproteinemias/diagnóstico , Administración Intravenosa , Adulto , Biopsia , Sedimentación Sanguínea , Médula Ósea/patología , Ciclofosfamida/administración & dosificación , Párpados , Femenino , Humanos , Cadenas gamma de Inmunoglobulina/sangre , Inmunoglobulinas Intravenosas/administración & dosificación , Xantogranuloma Necrobiótico/sangre , Xantogranuloma Necrobiótico/tratamiento farmacológico , Xantogranuloma Necrobiótico/etiología , Paraproteinemias/sangre , Paraproteinemias/complicaciones , Paraproteinemias/tratamiento farmacológico , Piel/patologíaAsunto(s)
Asma/sangre , Asma/complicaciones , Inmunoglobulina G/sangre , Metotrexato/uso terapéutico , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/tratamiento farmacológico , Xantogranuloma Necrobiótico/etiología , Anciano , Fármacos Dermatológicos/uso terapéutico , Oftalmopatías/diagnóstico por imagen , Humanos , Masculino , Xantogranuloma Necrobiótico/fisiopatología , Polonia , Enfermedades Raras/sangre , Enfermedades Raras/diagnóstico , Enfermedades Raras/tratamiento farmacológico , Resultado del TratamientoAsunto(s)
Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Xantogranuloma Necrobiótico/diagnóstico , Biopsia , Extremidades , Humanos , Masculino , Persona de Mediana Edad , Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Xantogranuloma Necrobiótico/etiología , Xantogranuloma Necrobiótico/patología , Piel/patología , TorsoAsunto(s)
Leucemia Mielomonocítica Crónica/complicaciones , Xantogranuloma Necrobiótico/etiología , Síndromes Paraneoplásicos/etiología , Paraproteinemias/complicaciones , Femenino , Arteritis de Células Gigantes/etiología , Humanos , Leucemia Mielomonocítica Crónica/patología , Persona de Mediana Edad , Paraproteinemias/patologíaRESUMEN
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.
Asunto(s)
Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/etiología , Xantogranuloma Necrobiótico/terapia , Paraproteinemias/complicaciones , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores , Terapia Combinada , Manejo de la Enfermedad , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Inmunoglobulina G/sangre , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Xantogranuloma Necrobiótico/mortalidad , Paraproteinemias/diagnóstico , Tomografía Computarizada por Tomografía de Emisión de Positrones , Estudios Retrospectivos , Piel/patología , Resultado del TratamientoRESUMEN
BACKGROUND: Head and neck cancer is often managed with a combination of surgery, radiation therapy, and chemotherapy, and skin toxicity is not uncommon. Xanthogranuloma is a pathological finding resulting from an inflammatory reaction that has not been previously reported following head and neck radiation therapy. CASE PRESENTATION: A patient with squamous cell carcinoma of the oropharynx, treated with definitive chemoradiation and hyperthermia, presented at eight-month follow-up with an in-field cutaneous lesion in the low neck, initially concerning for recurrent tumor. Biopsy showed xanthogranuloma and the patient underwent complete resection with congruent surgical pathology. The patient remained free of malignancy but continued to experience wound healing difficulties at the resection site which resolved with specialized wound care and hyperbaric oxygen. CONCLUSIONS: Skin toxicity is not uncommon in patients with head and neck cancer treated with radiation therapy. Awareness of unusual pathologic sequelae, such as xanthogranuloma, is needed to provide patient counseling while continuing appropriate surveillance for recurrent malignancy.
Asunto(s)
Carcinoma de Células Escamosas/radioterapia , Neoplasias de Cabeza y Cuello/radioterapia , Xantogranuloma Necrobiótico/etiología , Traumatismos por Radiación/complicaciones , Piel/patología , Biopsia , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Xantogranuloma Necrobiótico/diagnóstico , Traumatismos por Radiación/diagnóstico , Piel/efectos de la radiación , Carcinoma de Células Escamosas de Cabeza y Cuello , Factores de TiempoRESUMEN
Necrobiotic xanthogranuloma (NXG) is an uncommon granulomatous disorder of unknown pathogenesis that often presents with yellowish plaques in a periorbital distribution. While a majority of cases are associated with an underlying paraproteinemia of the IgG kappa type, a much smaller number are found to be associated with an underlying multiple myeloma. We present a case of a 78-year-old male with an isolated lesion of NXG on his right upper extremity. Following his diagnosis of NXG, further investigation for underlying systemic disorders with serum immunofixation revealed a monoclonal IgG kappa immunoglobulin with an M-spike of 1.2 g/dL. A PET-CT demonstrated bone destruction in the left proximal fifth rib, left scapula, the anterior lumbar I (L1) vertebrae, the left lumbar III (L3) vertebrae posterior elements and possibly left sacrum. A bone marrow biopsy revealed 18 % plasma cells. With these findings he was diagnosed with stage I multiple myeloma. Though clinically unimpressive and atypical in location for NXG, early biopsy and diagnosis of this solitary lesion led to the discovery of his hematopoietic disorder.
Asunto(s)
Inmunoglobulina G/inmunología , Mieloma Múltiple/diagnóstico , Xantogranuloma Necrobiótico/etiología , Anciano , Biopsia , Humanos , Cadenas kappa de Inmunoglobulina/inmunología , Masculino , Mieloma Múltiple/complicaciones , Mieloma Múltiple/patología , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/patología , Estadificación de Neoplasias , Tomografía de Emisión de Positrones/métodos , Tomografía Computarizada por Rayos X/métodos , Extremidad SuperiorRESUMEN
RATIONALE: Xanthomatosis associated with monoclonal gammopathy includes hyperlipidaemic xanthoma (HX), normolipidaemic xanthoma (NX) and necrobiotic xanthogranuloma (NXG). All three pathologies are characterized by skin or visceral lesions related to cholesterol accumulation, monoclonal immunoglobulin (MIg) and hypocomplementemia. The pathophysiology underlying NXG remains unknown although the involvement of MIg is suspected. OBJECTIVE: To provide further insights into the pathophysiology of NXG, we evaluated the plasma lipid phenotype, mechanisms involved in cellular cholesterol accumulation and role of MIg in an analysis of blood and plasma markers of inflammation in 16 patients with xanthomatosis [NXG (n = 8) and NX (n = 8)] associated with monoclonal IgG relative to the relevant controls. RESULTS: The lipid profile of patients with NXG was characterized by a low HDL-C phenotype and an abnormal distribution of HDL particles. Sera from patients with NXG induced cholesterol accumulation in human macrophages. This accumulation was due in part to a significant reduction in the HDL capacity to promote cholesterol efflux from macrophages, which was not found in the case of NX. The MIg of NXG and NX patients was tested positively by ELISA to recognize a large spectrum of lipoproteins. High plasma levels of pro-inflammatory cytokines (TNFα and IL-6), soluble cytokine receptors (sIL-6R, sTNFRI and sTNFRII), adhesion molecules (VCAM-1 and ICAM-1) and chemokines (MCP-1, IL-8 and MIP-1α) were observed in both patients with NXG and NX, revealing a specific xanthoma inflammatory signature which was inversely correlated with plasma levels of anti-inflammatory HDL. However, patients with NXG were distinguished by elevated levels of IL-15 and a marked increase in the rate of intermediate CD14++CD16+ monocytes. CONCLUSION: This study revealed that NXG is characterized by impaired macrophage lipid homeostasis associated with a systemic inflammatory profile that may result from the interaction of MIg and lipoproteins.
Asunto(s)
Xantogranuloma Necrobiótico/etiología , Paraproteinemias/etiología , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , HDL-Colesterol/metabolismo , Citocinas/metabolismo , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Inmunoglobulina G/metabolismo , Inflamación/metabolismo , Leucocitos Mononucleares/metabolismo , Metabolismo de los Lípidos/fisiología , Macrófagos/metabolismo , Masculino , Persona de Mediana Edad , Xantogranuloma Necrobiótico/metabolismo , Paraproteinemias/metabolismo , FenotipoRESUMEN
We report a unique case of periocular nonnecrobiotic necrobiotic xanthogranuloma in a 52-year-old white woman with Sjögren syndrome who was subsequently found to have an immunoglobulin G paraproteinemia and coexisting small lymphocytic lymphoma. Therapy with fludarabine, cytoxan, and rituximab (FCR) resulted in a dramatic resolution of her sicca symptoms and periocular xanthogranulomas. This case further illustrates the association of hematolymphoid disorders with cutaneous xanthogranulomatous disease and the importance of additional appropriate laboratory and radiologic investigation for the accurate diagnosis of an underlying malignancy.