Maintenance treatment of adults with chronic refractory immune thrombocytopenic purpura using repeated intravenous infusions of gammaglobulin [see comments]

Abstract

Intravenous infusion of gammaglobulin (IVGG) has been extensively used in the treatment of immune thrombocytopenic purpura (ITP) in adults to acutely raise the platelet count but not as a maintenance therapy. This report describes the maintenance treatment of adults with chronic ITP using repeated infusions of 800 to 1,000 mg/kg of IVGG. Sixteen of 40 patients were able to discontinue all therapy after receiving between one and 15 infusions. Five patients achieved remission and 11 other patients became stable without therapy (SWT) maintaining a platelet count greater than 20,000/microL without bleeding. The average quantity of gammaglobulin received for all patients was 606 g per patient. Of the 30 patients who underwent but did not respond to splenectomy, 11 (37%) were able to discontinue all therapy by either achieving remission (5) or becoming SWT (6). None of the five patients who achieved remission did so after only the initial therapy; all first received between one and 12 maintenance infusions. The ten splenectomized patients who were unresponsive to IVGG also failed to subsequently respond to conventional therapy including immunosuppressive agents and androgens. No toxicity of IVGG was seen except for postinfusion headaches. IVGG is an effective although expensive maintenance therapy for adults with ITP and is useful in patients who have not responded to splenectomy.

High Levels of Peripheral Blood NK Cells in Women Suffering from Recurrent Spontaneous Abortion are Reverted from High‐Dose Intravenous Immunoglobulins

“…Down‐regulation of CD56 + /16 + NK cells by IVIG infusion results in a favourable pregnancy outcome in women with elevated NK cells. The obvious deduction is the possibility of a chronic and long‐lasting beneficial effect of IVIG therapy, justified by its immunoregulatory properties, which include feedback inhibition of antibody synthesis, 29 blockade of the immunoglobulin Fc γ R, 30,31 blockade of transplacental transfer of IgG 32 regulation of the immune system through interactions with the idiotype network including the presence of anti‐idiotype molecules in IVIG that could modify B and T cell functions, 33,34 elimination of autoantibodies by increasing the catabolism of immunoglobulins, a mechanism acting in patients with anti‐cardiolipin (CL) antibodies where IVIG work as anti‐anti‐CL, 15–21 reduction of NK cell activity, 35 and modulatory effects on cell‐mediated responses, 36 for example, there is a significant increase in free GM‐CSF blood concentration after IVIG infusion 21 …”

The use of vinca alkaloids in preparation for splenectomy of corticosteroid refractory chronic immune thrombocytopenic purpura patients

“…The percentage of patients with significant platelet count increase after intravenous immunoglobulin infusions was reported after Salama, Mueller‐Eckhardt and Kiefel (1983); Bussel et al. (1988); Blanchette, Freedman and Garvey (1998) and included our own experience (Misiak et al.…”

“…, 2003). The platelet count ≥50 × 10 9 /l is considered hemostatically safe for splenectomy (Bussel et al. , 1988).…”

Refractory immune thrombocytopenic purpura: current strategies for investigation and management

“…IV anti‐D is less effective in patients who have undergone splenectomy (Scaradavou et al , 1997). As patients become increasingly refractory to therapy with these agents, alternative treatment options are required (Bussel et al , 1988).…”