[Idiopathic cold agglutinin disease. Clinical aspects, therapy and course in 6 patients].
Clinical features, diagnostic procedure, therapy, course of the disease and prognosis in 6 patients with severe idiopathic chronic cold agglutinin disease are described. In 5 patients the main complaint was cold mediated acrocyanosis. The cold agglutinin in all patients was of anti-I type and belonged to IgM immunoglobulin. Keeping warm provided symptomatic relief and the hemolysis decreased to a milder form. Treatment with glucocorticoids alone failed in two patients but succeeded in combination with chlorambucil or cyclophosphamid. One patient developed a lymphoproliferative disorder 11 years after diagnosis of idiopathic chronic cold agglutinin disease.
Budmiger
H
H
Departement für Innere Medizin, Universitätsspital Zürich.
Rhyner
K
K
Siegenthaler-Zuber
G
G
Bollinger
A
A
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000276:immunology*; D002699:Chlorambucil / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D004903:Erythrocyte Aggregation; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006461:Hemolysis; D006801:Humans; D007075:Immunoglobulin M / Q000302:isolation & purification*; D008297:Male
D005938:Glucocorticoids; D007075:Immunoglobulin M; D002699:Chlorambucil; D003520:Cyclophosphamide
Die idiopathische Kälteagglutininkrankheit. Klinik, Therapie und Verlauf bei 6 Patienten.