Factor V deficiency in Philadelphia-positive chronic myelogenous leukemia

Factor V deficiency has been identified in 8 of 8 patients 7--20 yr of age, with Philadelphia-positive (Ph1+) chronic myelogenous leukemia (CML). In these 8 patients, factor V deficiency was not due to hepatic dysfunction, factor V inhibitors, or disseminated intravascular coagulation. In 3 patients, factor V activity rose 10%--12% (0.10--0.12 U/ml) after the infusion of 28--31 ml/kg body weight of fresh frozen plasma (FFP). The rise persisted less than 14 hr. The mean measured postinfusion rise in factor V was 18% of the expected rise calculated from the volume of FFP infused in the patients' plasma volume. In 4 patients, a small transient rise in factor V activity occurred after splenectomy or plateletpheresis. Factor V deficiency was completely corrected after a marked reduction in bone marrow cellularity in 2 patients with Ph1+ CML treated with extensive chemotherapy, total body irradiation, and bone marrow transplantation. Factor V deficiency was retrospectively observed in 6 of 20 patients, ages 20--80 yr, with Ph1+ CML and 3 of 6 patients with other myeloproliferative disorders. The factor V deficiency appears to be associated with the large myeloid- megakaryocytic cell mass characteristic of CML and other myeloproliferative disorders.     

Positive end-expiratory pressure: Implications for tidal volume changes in anesthesia machine ventilation

In clinical practice, the addition of positive end-expiratory pressure (PEEP) into a standard anesthesia circle circuit decreases the delivered tidal volume (DTV) to a patient. We studied the magnitude of the DTV/PEEP relationship in two commonly used anesthesia systems. In addition, the magnitude of the DTV/PEEP relationship varies with both pulmonary compliance and volume of gas contained in the patient's breathing system between the ventilator and PEEP valve site, and this was also evaluated. Routine monitoring of expired tidal volume should be used whenever PEEP is added to an anesthesia circuit.