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Auto-immune Hemolytic Anemia Treated with d-Penicillamine
Report of a Case
- C. LOWELL EDWARDS, M.D.; and
- NAZARETH GENGOZIAN, PH.D.
- Requests for reprints should be addressed to Dr. Nazareth Gengozian, Medical Division, Oak Ridge Institute of Nuclear Studies, P. O. Box 117, Oak Ridge, Tenn. 37831.
Excerpt
Macroglobulins are a group of serum proteins, frequently referred to as paraproteins, having a molecular weight of at least one million and a sedimentation constant of more than 15 (usually 19 to 20) Svedberg units. Among the macroglobulins of clinical importance are the macroglobulin of Waldenström, the rheumatoid factor, some isohemagglutinins and Rh antibodies, some cryoglobulins, and cold agglutinins. In 1957, Deutsch and Morton (1) reported that macroglobulins could be depolymerized in vitro by incubation with certain mercaptans. It was later demonstrated that upon depolymerization the physiologic or immunologic properties of the proteins were lost even when there was some
Summario in Interlingua
Un patiente con auto-immun anemia hemolytic associate con multiple neoplasmas non respondeva a therapia corticosteroide o al tractamento con 6-mercaptopurina. In vitro, le reaction del anticorpore seral con le compositos sulfhydrylic, 2-mercaptoethanol e d-penicillamina, resultava in le inactivation del autoagglutinina, lo que pareva indicar que il se tractava de un macroglobulina. Le tractamento del patiente con un curso de octo dies de d-penicillamina esseva sequite de un declino del titro de autoagglutinina e su ultime disparition intra pauc dies post le suspension del therapia. Isto permitteva un successose transfusion, effectuante le restauration del previe valores erythrocytic, sin nove evidentia de un excessive destruction del transfusionate cellulas. Ben que le patiente remaneva Coombs-positive post le tractamento con d-penicillamina e durante le curso del subsequente menses, le autoagglutininas del sero non re-appareva. Isto es le secunde reporto del uso de d-penicillamina in le tractamento de auto-immun anemia hemolytic. Su efficacia pare esser associate con le natura macroglobulinic del auto-anticorpore.
Article and Author Information
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From the Medical Division, Oak Ridge Institute of Nuclear Studies, Oak Ridge, Tenn., under contract with the United States Atomic Energy Commission.
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- Received August 19, 1964.
- Accepted October 27, 1964.
