Adam Zdeněk, Pour Luděk, Řehák Zdeněk, Dvořáková Klára, Koukalová Renata, Feit Josef, Kameník Petr, Krejčí Marta, Štork Martin, Krejčí Martin, Sandecká Viera, Boichuk Ivanna, Král Zdeněk
Vnitr Lek. 2021 Winter;67(6):352-356.
Necrobiotic xanthogranuloma (NXG) is a rare chronic condition, belonging to the group non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly monoclonal gammopathy, MGUS and multiple myeloma. The case reported here NXG was diagnosed after 1 years of evolution in patient with asymptomatic multiple myeloma. After treatment with bortezomib, lenalidomid and dexamethasone, there was evident abrupt decrease of monoclonal immunoglobulin to not measurable level (complete remission of multiple myeloma) and in the same time was evident disappearance of cutaneous and hepatic lesions of NXG on FDG-PET/CT. The etiopathogenetic association of monoclonal immunoglobulin with NXG is documented in this case report with disappearance of NXG in the time of disappearance of monoclonal immunoglobulin.
坏死性黄色肉芽肿（NXG）是一种罕见的慢性疾病，属于非朗格汉斯细胞组织细胞增生症，因其可能累及皮肤外并与全身性疾病相关，特别是单克隆丙种球蛋白病、意义未明的单克隆丙种球蛋白血症（MGUS）和多发性骨髓瘤，所以具有相关性。本文报道的这例NXG患者在无症状多发性骨髓瘤病程1年后被确诊。在用硼替佐米、来那度胺和地塞米松治疗后，单克隆免疫球蛋白明显急剧下降至不可测水平（多发性骨髓瘤完全缓解），同时，FDG-PET/CT显示NXG的皮肤和肝脏病变明显消失。本病例报告记录了单克隆免疫球蛋白与NXG的病因学关联，即随着单克隆免疫球蛋白的消失，NXG也消失。
