Four patients with paroxysmal nocturnal haemoglobinuria (PNH) were treated with cyclosporine. The treatment with cyclosporine was based on the hypothesis that immune-mediated bone-marrow damage is the common pathogenetic mechanism of aplasia and PNH, with lack of GPI-linked ligands for an immune attack (i.e. LFA-3, CD58) rendering PNH cells a growth advantage over other bone marrow cells. In the first patient, presenting with a mixed AA/PNH syndrome, a gradual recovery from aplasia was seen after prolonged treatment with cyclosporine. In a second patient, with a mixed AA/PNH syndrome, no haematological improvement was noted during cyclosporine administration, but this patient became transfusion-independent with increasing neutrophil and platelet counts after a course of ATG in combination with androgen therapy. Both these patients showed an increment in the proportion of neutrophils with normal expression of GPI-linked proteins concurrently with the improvement of haematological characteristics. In the two other patients, presenting with typical PNH, cyclosporine treatment did not result in any change in haematological characteristics, nor in PNH parameters. No significant change in haemolytic parameters was seen in any of the patients. It is concluded that immunosuppressive therapy may be of benefit in patients with a mixed AA/PNH syndrome. This effect became apparent after prolonged treatment with cyclosporine in one patient, and after a subsequent course of ATG with concomitant androgen therapy in another.
This case report describes a case of chronic Epstein-Barr virus infection resulting in lymphadenopathy morphologically indistinguishable from Hodgkin's disease. Morphologic, immunologic, and virologic studies could be performed on three consecutive lymph nodes. For the first time in the literature, Epstein-Barr Nuclear Antigen (EBNA) could be demonstrated in the nuclei of Sternberg-Reed cells.
SummaryNew approaches and techniques for improving source material collection and Factor VIII production at Blood Bank level have been reported recently.Heparin has been shown to be of importance in increasing yields and stability of FVIII in the purification and concentration process. Work has been done to develop on a routine scale the heparin double cold precipitation technique for the production of a freeze-dried high yield purified FVIII concentrate.The product has been tested clinically in 4 severe hemophilia A patients for recovery, half-life and acute side-effects, using two dosages over 8 infusions. There was no significant difference between the two dosages. Mean recovery 99.1% and mean halflife 8 hr, ranging from 6.5 to 10.3 hr.No side-effects were observed.These results justify further exploration of the potential of heparin for high yield purified FVIII production.
Autologous platelets were harvested and cryopreserved in eight consecutive patients elected for ablative chemotherapy and autologous bone marrow transplantation (ABMT) for solid malignancy. There was a 19% loss in platelet count after the freeze thaw and wash procedure; with an in vitro functional loss of 40-60%. No correlation could be found for individual platelet transfusions between in vitro functional tests and in vivo recovery. Six consecutive patients received a total of 16 autologous platelet transfusions in the aplastic phase of ABMT. No bleeding was observed during the study period and there was no CMV infection in the recipients. While improvement in freezing and subsequent handling is desirable, autologous cryopreserved platelets can safely be used for the prophylaxis of bleeding during aplasia in patients treated with ABMT.
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