NK ACTIVITY IN HEMOPHILIA AND VON WILLEBRAND DISEASE

Abstract

Impaired natural killer (NK) activity has been reported in patients with autoimmune diseases, tumors & also certain hematologic disorders. Patients with hemophilia (Hem) are at risk for development of AIDS & may also exhibit immune abnormalities. We investigated 41 patients focusing on NK activity compared with other immunologic parameters & clinical status. Patients were divided into subgroups: Hem A treated with lyophilized commercial Factor VIII concentrates (LYOPH-C) or from voluntary donors (LYOPH-V); Hem A with Factor VIII inhibitors; Hem B & untreated patients with Hem A or Von Willebrand's. Overall, patients had significantly ↓ NK cytotoxicity (42.4 ± 15.3% vs 61.7 ± 15.0%, p<0.01). Although the LYOPH-V group had a slightly higher NK activity (49.9 ± 13.9%), there was no significant difference among patient groups. Untreated patients exhibited ↓ NK activity (39.1 ± 13.6%, p<0.01). There was no relationship between reduced NK activity & other immunologic parameters including blastogenesis, MLC, T cell markers, QIG's, auto Ab & circulating immune complexes (CIC). In patients with + CIC, there was a higher rate of ↓ NK activity compared with those - for CIC (8/27 vs 2/14) but these were not statistically different. There was also no relationship between NK activity & patients' clinical status including severity of disease, CMV Ab & amount of plasma product infusion. This study suggests that patients with various bleeding disorders may have underlying immune abnormalities which are unrelated to specific plasma product treatment.

Log in or create a free account to read this content

Gain free access to this article, as well as selected content from this journal and more on nature.com

Access through your institution

or

Sign in or create an account

Continue with Google

Continue with ORCiD