Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema.
نویسندگان
چکیده
A 52 year old woman with marginal zone lymphoma developed recurrent episodes of angio-oedema and was found to have C1 esterase inhibitor deficiency. She declined chemotherapy for the lymphoma. Fourteen months after her initial presentation she was found to be in partial remission, and this was confirmed by peripheral blood film and bone marrow examinations. This was associated with normalisation of C1 esterase inhibitor, C1q, and C4 values. Regression of acquired C1 esterase inhibitor deficiency associated with spontaneous partial remission of lymphoma has not been reported previously.
منابع مشابه
A nationwide study of acquired C1-inhibitor deficiency in France
Acquired angioedema (AAE) due to C1-inhibitor (C1INH) deficiency is rare. Treatment options for acute attacks are variable and used off-label. Successful treatment of the associated lymphoma with rituximab seems to prevent acute attacks in subjects with AAE. The aim of this study was to describe AAE manifestations, its associated diseases, and patients' responses to treatments in a representati...
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متن کاملRecurrent angio-oedema--three cases of C1 inhibitor deficiency.
C1 Inhibitor deficiency is a rare disorder, characterised by recurrent angio-oedema of skin, upper respiratory and gastrointestinal tracts. It can be a mimicker of acute abdomen or anaphylaxis to drug or food and lead on to unnecessary overtreatment. Three case reports of such patients with history of recurrent abdominal pain and angio-oedema due to C1 Inhibitor deficiency is reported here.
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ورودعنوان ژورنال:
- Journal of clinical pathology
دوره 55 10 شماره
صفحات -
تاریخ انتشار 2002