DDAVP: a useful alternative to blood components in moderate hemophilia A and von Willebrand disease.

The effect of the vasopressin analog 1-deamino-8-d-arginine vasopressin (DDAVP) was studied in three normal individuals, 31 subjects with von Willebrand disease, and seven subjects with mild or moderate hemophilia A. None of those with von Willebrand disease had qualitative abnormalities of factor VIII-related antigen (F VIII:RAg). Both intranasal (2 to 4 micrograms/kg) and intravenous (0.2 micrograms/kg) DDAVP were used. All normal subjects, 27 of 31 with von Willebrand disease, and six of seven with hemophilia had a more than 200% increase in F VIII coagulant activity, with lesser but definite increases in F VIII:RAg and ristocetin cofactor activity. Two subjects with severe von Willebrand disease had no increase in F VIII-related activities. In six subjects with von Willebrand disease who had prolonged bleeding times, there was transient correction after DDAVP therapy. None of eight subjects who received DDAVP prior to surgical procedures had any unusual bleeding during or after surgery. None received any blood products. No untoward side effects were noted in any of the 41 subjects. We conclude that DDAVP is a safe and effective alternative to the use of blood products in certain individuals with von Willebrand disease and hemophilia A.

Infections in leukemic children: a prospective analysis.

A 28-month prospective study of 54 leukemic children was carried out to determine the incidence and type of infection associated with febrile episodes. Fever was caused by infections in 84 of 199 episodes (71%). Two-thirds of the febrile episodes and 57% of the documented infections occurred when leukemic activity was demonstrable. However, only nine of 29 febrile episodes which occurred at the time of initial diagnosis of acute leukemia were due to infection. All serious bacterial infections occurred in children with absolute granulocyte counts less than 500/mm3. Septicemia was responsible for seven of the 17 deaths which occurred during the period of observation. The five children with Pseudomonas infections were colonized 10 to 30 days before they developed their infection. The majority of viral infections occurred in patients in remission, and were principally caused by cytomegalovirus, varicella-zoster virus, or Epstein-Barr virus. With the exception of one patient who died with a complex infection (CMV and Pneumocystis carinii), the children in this study responded well to viral infections.