Erlinda M. Gordon

Reduced Titers of Hageman Factor (Factor XII) in Orientals

Hageman factor (factor XII) is a procoagulant upon which the surface-mediated generation of fibrinolytic activity and kinin formation depends. We found that the clotpromoting activity and antigenic properties of Hageman factor were present in lower levels in the plasma of a small group of Oriental subjects than in that of American whites, whereas the levels of plasma prekallikrein (Fletcher factor), high-molecular-weight kininogen (Fitzgerald factor), and plasma thromboplastin antecedent (factor XI) in plasma samples from Oriental subjects did not significantly differ from those from normal white subjects. In one family, there was an excess of plasma Hageman factor antigens with respect to functional activity. These findings stress the importance of defining the racial characteristics of subjects used to establish standards for clotting assays.

Burkitt lymphoma in a patient with classic hemophilia receiving factor VIII concentrates

PATIENTS WITH CLASSIC HEMOPHILIA (factor VIII deficiency) frequently develop expanding hematomas? The index of suspicion for a malignant tumor is therefore low when a hemophilic patient has a mass lesion. We describe a patient whose problems exemplified the difficulties that a clinician may encounter in diagnosing and treating a malignant tumor in a patient with classic hemophilia. The findings also raise the question of whether Burkitt lymphoma developing in a hemophilic patient may have resulted from the transmission of a blood-borne oncogenic agent through infusion of lyophilized preparations of factor VIII,

Reduction of contact factors in sickle cell disease

Surface-mediated reactions of clotting were compared in 21 black children with homozygous sickle cell disease, 12 age-matched controls, and 15 adults. Both the coagulant and antigen titers of Hageman factor (factor XII) were decreased in asymptomatic patients compared with those in the control groups. These findings were associated with slight but significant reductions in the plasma titers of prekallikrein and high molecular weight kininogen. A further decrease from the initially low titers of these contact factors was observed during vaso-occlusive crises. Additionally, we observed a disparate relationship between Hageman factor coagulant activity and its antigen titers. These data provide evidence for reduction of the contact factors in patients with homozygous sickle cell disease.

Psychogenic Purpura in Adolescent Patients

The occurrence of psychogenic purpura has been documented in adults. The authors present their experience with nine adolescent and young adult patients observed since 1981. The age of eight of these patients ranged from 13 to 18 years. One patient with cystic fibrosis was 23-years- old. All patients presented with painful inflammatory lesions followed by bruising. There was no evidence of an infectious process or a hematologic, immunologic, or renal abnormality. Integral to the diagnosis was the existence of an underlying emotional disorder. In all patients, bruising decreased after diagnosis was explained to the patient and family. Four patients required psycho therapy. Results of 2-year follow-up suggest that early intervention may produce a favorable outcome in this age group.

910 EVIDENCE FOR REDUCTION OF CONTACT FACTORS IN SICKLE CELL DISEASE

Evidence supporting the existence of intravascular coagulation in homozygous sickle cell (HbSS) disease has been reported. In this study, surface-mediated reactions of clotting were compared in 21 black children with HbSS disease and 12 age-matched controls. Both the coagulant and antigen titers of Hageman factor were decreased (mean coagulant titer 0.49 ± S.D. 0.29 u/ml; mean antigen titer 0.77 ± 0.27 u/ml) in asymptomatic HbSS patients compared to the control group (mean coagulant titer. 1.01 ± 0.39 u/ml; mean antigen titer 0.94 ± 0.25 u/ml). A disparate relationship between the Hageman factor coagulant and antigen titers were observed in HbSS patients. These findings were associated with a slight decrease in the plasma titers of prekallikrein and HMW kininogen (mean 0.78 ± 0.14 and 0.75 ± 0.15 u/ml respectively in HbSS patients compared to 1.05 ± 0.22 and 0.86 ± 0.09 u/ml respectively in the control group). A further reduction from the initially low titers of these contact factors was observed during vaso-occlusive crises (mean Hageman factor titer 0.32 ± 0.15 u/ml; mean prekallikrein titer 0.38 ± 0.18 u/ml; mean HMW kininogen titer 0.56 ± 0.17 u/ml). These data indicate that the initial participants of the intrinsic pathway of coagulation are consumed in patients with HbSS disease. Further, these processes appear to accelerate during vaso-occlusive crises.