No abstract

After a review of the literature, a series of 21 patients with ITP treated with non-steroidal immunodepressive agents is presented. The agents were azathioprine (AZP), methotrexate (MTX), cyclophosphamide (CPH), vinblastine (VLB) and L-asparaginase (L-ase). No permanent, unmaintained remission in chronic, self-perpetuating cases of ITP could be obtained, even after splenectomy. However, AZP was useful in the treatment of chronic cases, either alone or in combination with steroids, where it displayed a useful steroid sparing effect. CPH and MTX were generally ineffective. VLB induced striking temporary thrombocytoses, and L-ase was followed by an early thrombocytosis, but proved too toxic to be employed consistently. Single case reports and remission percentages are reported.

No abstract

A 7-year-old male child with congenital hypoplastic anaemia (Diamond-Blackfan), having originally responded to corticosteroids, but having subsequently become refractory and erythroblastopenic since 4 years, was treated with a combination of cyclophosphamide (CY; 2.1 g) and antilymphocytic globulin (ALG; 8 g), both by the intravenous route. Erythroblastic repopulation of the bone marrow, albeit dyserythropoietic in character, reticulocytosis and erythrocytic increment took place, but only for a short period. The significance of these findings is discussed in the light of recent progress in the understanding of the disease and of its treatment.

Treatment of severe aplastic anemia (SAA) in Europe between 1970 and 1986 is reviewed. 487 patients received an HLA-identical BMT: results are encouraging and currently suggest a 65% survival. However, many patients cannot be offered this procedure because of the absence of an appropriate donor. Forty-five patients were given a non-HLA identical BMT: results are dependent on the degree of mismatch. Immunosuppression (IS) was given to 509 patients: 50% of these survive. Some mechanisms regulating in vitro hematopoiesis are discussed, together with their relevance in the treatment of SAA.