[New results on hyperglobulinemic purpura of Waldenström in childhood (author's transl)].
The case of a 13 year old girl with benign purpura hypergammaglobulinemica Waldenström. Besides the characteristic clinical symptoms and the respective laboratory results new findings we demonstrated, which might shed some light into the pathogenesis of this disease. The transformation rate of lymphocytes was decreased particularly after stimulation with ConA. The T-lymphocytes which are reduced in their function are unable to sufficiently control the activity of mesenchymal cells which results in an increased production of glycosaminoglycans. The demonstration of glycosaminoglycan-inclusion-bodies in the mononuclear bone marrow cells of the patient could be explained by the above mentioned mechanism. Upon therapy with D-penicillamine the clinical state improved along with a reduction of the sedimentation rate, the serum protein and the serum gamma-globulin.
D000293:Adolescent; D001853:Bone Marrow / Q000473:pathology; D003208:Concanavalin A; D005260:Female; D006025:Glycosaminoglycans / Q000032:analysis; D006801:Humans; D002479:Inclusion Bodies; D008213:Lymphocyte Activation; D010396:Penicillamine / Q000627:therapeutic use; D011694:Purpura, Hyperglobulinemic / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D013601:T-Lymphocytes
D006025:Glycosaminoglycans; D003208:Concanavalin A; D010396:Penicillamine
Neue Befunde bei der benignen Purpura hypergammaglobulinaemica Waldenström im Kindesalter.