Acquired angioedema with lymphoproliferative disorder: association of C1
inhibitor deficiency with cellular abnormality
AD Schreiber, B Zweiman, P Atkins, F Goldwein, G Pietra, B Atkinson and NI Abdou
A patient with a lymphoproliferative disorder, angioedema, and an acquired
deficiency of the inhibitor of the activated first component of complement
was studied. The patient's complement profile revealed depletion of the
first component of complement, which has not been seen in angioedema of the
hereditary type. There was no evidence for C1- depleting activity in the
patient's plasma. The majority of the patient's peripheral blood
mononuclear cells resembled B cells in their memebrane receptor properties
and in that they carried easily detectable immunoglobulin, predominantly
IgM. However, these cells were unusual in that they phagocytosed both latex
particles and C3-coated erythrocytes. Morphological study of the cells
infiltrating the patient's lung revealed immature, atypical, and
plasmacytoid lymphocytes and immunoblasts. Both the patient's peripheral
blood mononuclear cells and a suspension of cells from the pulmonary
infiltrate were capable of depleting the first component of complement and
its inhibitor from homologous plasma. Normal ABO-compatible cells did not
possess this property. The data suggested that the patient's abnormal
lymphoid cells may have interacted with the complement system to produce a
biochemical defect and a clinical syndrome closely resembling angioedema of
the hereditary type.
Volume 48,
Issue 4,
pp. 567-580,
10/01/1976
Copyright © 1976 by The American Society of Hematology
