Necrobiotic xanthogranuloma successfully treated with autologous stem cell transplantation

Abstract

Paraproteinemia can be complicated by necrobiotic xanthogranuloma. Therapeutic options for this progressive disease are limited, and there is no agreement on a single best strategy. We report the case of a patient with a massive periorbital infiltration narrowing the palpebral fissure and blinding the patient. Conventional myeloma therapy had only limited benefit in our patient. However, he was successfully treated with high-dose chemotherapy followed by autologous stem cell transplantation, rendering the patient free of symptoms. This is the first report of autologous stem cell transplantation in a patient with necrobiotic xanthogranuloma.

The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

“…Necrobiotic xanthogranuloma, a rare chronic disorder prevailing in the sixth decade, occurs in association with IgG monoclonal gammopathy (usually k subtype) in about 80% of patients. Although bone marrow examination may show plasmocytosis, true multiple myeloma is not frequent (10%) 44–46 . Cryoglobulinemia occurs in 8% of case.…”

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

“…Of note, the xanthomatous lesions of the 4 patients who received an immunomodulatory drug, usually in combination with dexamethasone, were considered to be improved in all cases. Variations in the MIg level were rarely reported and inconsistent: some reported a decrease in levels accompanied by an improvement in cutaneous lesions, 34,75 whereas others reported that skin lesions persisted despite a reduction in MIg levels. 76 …”

“…46,[64][65][66][67] Extracutaneous lesions were reported in 23 cases (17.6%), including 6 patients with several localizations. They were pulmonary (n ϭ 7), 8 cardiac (n ϭ 6), 8,37 oral cavity (n ϭ 4), 38,65 orbital (n ϭ 3), 8 hepatic (n ϭ 2), 8,37 spleen (n ϭ 2), 8 medullar (n ϭ 1), 68 genital (penis; n ϭ 1), 34 mastoid (n ϭ 1), 32 facial nerve (n ϭ 1), 32 and renal (n ϭ 1). 69 In addition, splenomegaly was noted in 22 patients (17%) and hepatomegaly in 17 (13%), without any further information.…”

“…We reviewed 216 well-described cases of xanthomatosis associated with MIg in the literature, including 37 cases of HX, 3 63 of NX, 6,9-30 and 116 of NXG 4,8,[31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] (Tables 3 and 4). The main patient characteristics are listed in Tables 3 and 4.…”

Successful multimodality treatment of recalcitrant necrobiotic xanthogranuloma using electron beam radiation and intravenous immunoglobulin

“…Cases of complete response of NXG to treatment with PUVA, systemic chemotherapy agents, and IVIg have been reported in the literature . More severe refractory cases associated with underlying plasma cell dyscrasias have been treated with stem cell transplant . Laser ablation may be another procedural technique to treat individual NXG lesions, with pain in such cases managed by regional nerve blocks .…”