非清髓性异基因干细胞移植：获得性血管性水肿的一种新治疗选择？

Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?

作者信息

Zegers I H A, Aaldering K N A, Nieuwhof C M G, Schouten H C

机构信息

Department of Hematology, Radboud University Hospital, Nijmegen, the Netherlands.

出版信息

Neth J Med. 2015 Oct;73(8):383-5.

PMID:26478549

Abstract

INTRODUCTION

Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor.

CASE REPORT

A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody. Additional analysis showed that in the IgM fraction autoantibody activity against C1 esterase inhibitor was present. This confirmed the diagnosis of acquired angioedema in the presence of lymphoplasmacytic lymphoma. Despite standard therapy, there was an increase in the episodes of laryngeal oedema. Therefore it was decided to perform a non-myeloablative allogeneic haematopoietic stem cell transplantation, with his HLA-identical brother as donor. The post-transplantation course was without complications. Five years following alloSCT he is in complete remission without symptoms and with increased C1 esterase inhibitor activity.

DISCUSSION

In this case all other known treatment options for severe acquired angioedema failed. This is the first case describing treatment of severe acquired angioedema, caused by lymphoplasmacytic lymphoma, with an alloSCT.

摘要

引言

获得性血管性水肿是一种罕见的疾病，由于C1酯酶抑制剂活性降低，导致反复发作的危及生命的血管性水肿。

病例报告

一名57岁男性因双手、嘴唇、舌头、阴囊和喉咙反复肿胀前来我院就诊。实验室检查显示存在IgM κ单克隆抗体。进一步分析表明，在IgM组分中存在针对C1酯酶抑制剂的自身抗体活性。这证实了在存在淋巴浆细胞淋巴瘤的情况下获得性血管性水肿的诊断。尽管进行了标准治疗，但喉水肿发作仍有增加。因此，决定进行非清髓性异基因造血干细胞移植，供者为其HLA配型相同的兄弟。移植后过程无并发症。异基因造血干细胞移植后五年，他完全缓解，无症状，C1酯酶抑制剂活性增加。