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J Clin Pathol 2002;55:789-790 doi:10.1136/jcp.55.10.789
  • Case report

Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema

  1. M K Phanish1,
  2. A Owen2,
  3. D H Parry3
  1. 1Renal Department, Gwynedd Hospital, Bangor LL57 2PW, North Wales, UK
  2. 2Department of Clinical Chemistry, Gwynedd Hospital
  3. 3Department of Haematology, Gwynedd Hospital
  1. Correspondence to:
 Dr D H Parry, Department of Haematology, Gwynedd Hospital, Bangor LL57 2PW, North Wales, UK;
 Huw.Parry{at}nww-tr.wales.nhs.uk
  • Accepted 11 April 2002

Abstract

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.

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