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, Clq, and C4 values. Regression of acquired Cl esterase inhibitor deficiency associated with spontaneous partial remission of lymphoma has not been reported previously.
Acquired deficiency of C1 esterase inhibitor causing angio-oedema has been reported as a rare phenomenon associated with non-Hodgkin's lymphoma. (1-3) We report a case of acquired Cl esterase inhibitor deficiency in a patient with splenic marginal zone lymphoma who subsequently showed spontaneous regression.
A 52 year old woman was referred to the haematology department for the investigation of lymphocytosis, discovered on routine testing. She was asymptomatic and denied fever, night sweats, or weight loss. Physical examination revealed no abnormality and in particular no palpable lymphadenopathy or hepatosplenomegaly.
Subsequent investigations revealed: haemoglobin, 130 g/litre; white cell count, 27 x [10.sup.9]/litre (differential: neutrophils, 5.6 x [10.sup.9]/litre; lymphocytes, 15.4 x [10.sup.9]/litre) and platelet count, 278 x [10.sup.9]/litre. The peripheral blood film showed atypical lymphoid cells with irregular cytoplasmic outline, suggesting splenic marginal zone lymphoma. The serum urea, …