Acute coagulopathy following infusion of prothrombin complex concentrate.

An acute coagulopathy developed in a 49 year old woman with severe liver disease after she received an infusion of prothrombin complex concentrate. The concentrate used in the infusion was subsequently studied by observing the effect of the concentrate on the partial thromboplastin times of various plasmas. The evidence suggests that activated coagulation factors, including activated factor X, were present in the concentrate, and probably played a role in initiating the acute change in the patient's coagulation status. Mechanisms whereby liver disease predisposes toward the development of such a coagulopathy are discussed. It would appear that prothrombin complex concentrates should be used in patients with liver disease only with utmost caution.

The occurrence of an auto-immune hemolytic anemia with anti-U specificity in a patient with myelodysplastic syndrome.

A 73-year-old man with myelodysplastic syndrome developed during the course of his illness auto-immune hemolytic anemia. The association between auto-immune hemolysis and myeloproliferative disease is extremely rare. An even more unique finding is the nature of the antibody which demonstrated single specificity against the U antigen.

Aortic insufficiency following mediastinal radiation for Hodgkin's disease.

Aortic insufficiency developed 13 months after mediastinal radiation in a patient with Hodgkin's disease. The estimated cardiac dose was 4200 rads. Several years later, a cerebral embolus occurred. The evidence for valvular and endocardial damage due to radiation is reviewed.

Idiopathic thrombocytopenic purpura in the elderly.

Idiopathic thrombocytopenic purpura (ITP) was diagnosed in 22 patients above the age of 50 during a seven-year period. There was no female preponderance. Steroid therapy was effective, particularly in preventing hemorrhage and is considered to be the treatment of choice. Splenectomy may be less beneficial than in younger patients with ITP. Complications of the disease and its treatment are minimal.

Philadelphia chromosome-negative chronic myelogenous leukemia with trisomy D.

Previous reports emphasize that trisomy 8 is the most common, single chromosomal abnormality in both Philadelphia (Ph1) chromosome-positive and Ph1-negative chronic myelogenous leukemia (CML). Karyotype analysis on a 78-year-old man with Ph1-negative CML showed trisomy D, confirmed on banding studies to be an extra No. 14 chromosome. Although the abnormality is likely to be related to the leukemic clone, the importance of this chromosomal deviation is not known.

Cryptogenic cirrhosis associated with methyldopa.

A 55-year-old woman developed cirrhosis with portal hypertension while taking methyldopa. No other cause for the cirrhosis was evident. In view of the known hepatotoxic effects of methyldopa, a causal relationship is implied. Possible mechanisms of drug toxicity include both immunologic reactions and direct hepatocellular damage by the drug or its metabolites.

Autoimmune hemolytic anemia associated with ulcerative colitis.

Three patients are reported in whom autoimmune hemolytic anemia developed during the course of ulcerative colitis. A review of the literature yielded 11 additional cases, and the clinical, immunologic, and therapeutic characteristics are summarized. The results of steroid therapy and splenectomy are similar to those for idiopathic autoimmune hemolytic anemia. The cause and effect relationship between these two diseases is not clear, but colectomy appears to produce remission in hemolysis when the latter is refractory to both steroids and splenectomy.

Monosomy 7 in a patient with pancytopenia and abnormal erythropoiesis.

In 1976, a patient with pancytopenia was found to have a population marrow cells with monosomy of chromosome 7 (45,XY,-7). Over the next 3 years he had continued abnormal hematopoiesis consisting of erythroid hyperplasia, ring sideroblasts, megaloblastic changes, and an increased proportion of myeloblasts. Sequential chromosome studies consistently showed the same abnormality without further karyotypic change. From the present study and comparable cases in the literature, there appears to be a distinct subgroup of patients with myeloproliferative disorders showing dyserythropoiesis with monosomy of deletion of chromosome 7 in the marrow cells.