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首页> 外文期刊>European Journal of Haematology >Cutaneous macroglobulinosis treated with bortezomib and rituximab.
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Cutaneous macroglobulinosis treated with bortezomib and rituximab.

机译：硼替佐米和利妥昔单抗治疗皮肤巨球蛋白病。

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A 67 yr-old male was referred for a second relapse of Waldenstrom's macroglobulinemia (WM) revealed by the recurrence of pancytopenia and skin lesions. The patient displayed multiple erythematous, non-prurigin-ous, 1-2 mm papules located at the anterior face of the knees and calves (Fig. 1A). Histopathological examination of one hyperkeratotic papule revealed an eosino-philic infiltrate of the superficial and mid-dermis with no malignant cells (Fig. 1B). Periodic-acid-Schiff staining was positive (Fig. 1C) while Congo red staining remained negative. Direct fluorescence showed the presence of IgM deposits confirming the diagnosis of cutaneous macroglo-bulinosis (CM). The patient was treated with a combination of bortezomib and rituximab. This regimen induced a partial response with a >=50% reduction of M-spike and patient became transfusion independent. However, the skin lesions remained unchanged.
机译：一名67岁的男性因全血细胞减少症和皮肤病变的复发而发现Waldenstrom巨球蛋白血症（WM）第二次复发。该患者在膝盖和小腿的前侧显示出多个红斑，非瘙痒性的1-2 mm丘疹（图1A）。对一个过度角化的丘疹进行组织病理学检查，发现表皮和真皮中部有嗜酸性浸润，没有恶性细胞（图1B）。高碘酸-席夫氏染色为阳性（图1C），刚果红染色为阴性。直接荧光显示IgM沉积物的存在，证实了皮肤巨球蛋白病（CM）的诊断。该患者接受了硼替佐米和利妥昔单抗的联合治疗。该方案诱导了部分反应，M-峰降低了> = 50％，并且患者变得与输血无关。然而，皮肤病变保持不变。

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