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Biomedical subjects

B M Taleghani

Publications and source records attributed to B M Taleghani.

7 recordsLinked to original sources

Therapy of coagulation factor VIII autoantibodies with long-term extracorporeal protein A adsorption and immunosuppression.

UNLABELLED: Acquired factor VIII (FVIII) inhibitors in non-haemophiliacs may pose serious treatment problems. MATERIALS AND METHODS: For IgG-adsorptions we utilized an automated plasma separation device and a plasma flow monitor. CASE REPORTS: A 63-year old woman showed life-threatening bleeding because of an inhibitor. After stabilization by porcine FVIII three cycles of a modified Malmoe treatment protocol were applied, followed by long-term cyclophosphamide p.o. and weekly IgG-adsorptions. Within twelve months the patient exhibited a complete remission. A 54-year old man presented a comparable history. Because of a good response after the first cycle he was subsequently switched to the long-term therapy. Up to now (> 7 months) FVIII activities and inhibitor titers remained stable (10-20% rsp. < 3 BU/ml). In both cases no FVIII substitution therapy was necessary. CONCLUSIONS: A modified Malmoe protocol combined with long-term cyclophosphamide p.o. and weekly IgG-adsorptions seems to be an efficient, safe and cost-effective regimen for non-haemophiliacs with FVIII inhibitors.

Autoantibodies↗

Treatment of severe myasthenia gravis with protein A immunoadsorption and cyclophosphamide.

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction caused by antibodies against the nicotinic acetylcholine receptor (AChR-Ab). We report a 16-year-old girl with severe MG who showed a poor response to plasma exchange and tryptophan-linked polyvinylalcohol gel immunoadsorption. Further improvement of muscle strength and decline of AChR-Ab could be achieved after initiation of protein-A immunoadsorption (PA-IA). Maintenance therapy with PA-IA and intravenous pulses of cyclophosphamide resulted in a stabilisation of the disease, with a complete remission during the follow-up period of six months. We suggest that PA-IA may be valuable and safe in the management of patients with severe MG, and maintenance therapy with PA-IA and cyclophosphamide may prevent serious and potentially life-threatening relapses of the disease.

Adolescent↗