The development of factor VIII inhibitors has been a major problem in the management of hemophiliacs. The use of prothrombin complex concentrates (PCC) has been advocated as a method of overcoming ...this problem. We utilized PCC in the treatment of nine hemophiliac patients who had significant levels of inhibitor. Acute bleeding episodes were successfully treated in all nine patients. Single infusions of PCC were usually adequate for control of joint and soft tissue bleeding episodes, while multiple infusions were usually required for more serious hemorrhages. In addition, programs of repeated infusions were used to heal chronically inflamed joints in three patients. The only side effect of treatment was one episode of asymptomatic elevation of liver function values that resolved spontaneously. There were no thrombotic events associated with the PCC infusions. Therapy with PCC is recommended for the management of hemophiliacs with factor VIII inhibitors.
Platelet-associated IgG was studied in children with acute and chronic ITP and in patients with thrombocytopenic SLE, using the microtiter solid-phase radioimmunoassay. Of the children with acute ...ITP, 85% had elevated PAIgG levels. The degree of elevation of PAIgG at onset of disease did not correlate with the development of chronicity. Of the children with acute ITP, clinically and hematologically indistinguishable from the rest, 15% had normal PAIgG values. All of 22 children with chronic ITP had elevated PAIgG values. Although there was good correlation between the platelet count and the PAIgG value in children with chronic ITP, the association was not as striking in those with acute ITP; thus, factors in addition to the level of PAIgG may contribute to the thrombocytopenia in the latter group. Patients with SLE and thrombocytopenia had higher values of PAIgG than would be predicted from the platelet count; the PAIgG value is probably not the only factor determining the degree of immune thrombocytopenia.
While home care treatment for hemophilia is not a panacea and does not supplant good comprehensive care, it does seem to offer improved medical care for those hemophiliacs enrolled in such a program. ...The results have been very encouraging in the programs of this type throughout the United States. Since most programs have been in existence for 5 years or less, long-term data have not accumulated. However, the evidence seems to suggest that crippling is less in children and that orthopedic deformities do not progress at the same rate in adults. Children have improved school attendance; young men may attend college and graduate schools without difficulty. Adults may hold a job without fear of absenteeism. There are fewer dollar costs of the disease for the patient and the psychiatric cost of this chronic illness is markedly less. The quality of life is improved and the patient may develop a new self-confidence which allows him to function as a productive member of society.
A blinded randomized multicenter trial of two non-activated prothrombin complex concentrates was carried out to determine the clinical effectivity in the treatment of acute hemarthrosis in ...hemophiliac patients with inhibitors. The one product was prepared via DEAE Sephadex chromatography, while the second was fractionated via various precipitation procedures including polyethylene glycol. Equivalence of the two products was established with less than 15% difference in efficacy rates.
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