Diffuse adenomatoid tumor of the uterus with a serosal papillary cystic component.

A 39-year-old woman undergoing immunosuppressive therapy following kidney transplantation for systemic lupus erythematosus presented with a uterine adenomatoid tumor that diffusely infiltrated the entire myometrium and contained a serosal papillary cystic component that resembled a cystic mesothelioma. This is the first reported case of an adenomatoid tumor showing both of these features. Although adenomatoid tumors are considered benign, the patient may be at risk for recurrence of the papillary cystic component (which is known to recur in 50% of cases) if this tumor reflects an inability to limit neoplastic processes.

Zollinger-Ellison syndrome in a patient with normal screening gastrin level.

A 59-year-old female presented with multifocal peptic ulcer disease and diarrhea. A fasting serum gastrin level obtained while the patient was receiving no antacid therapy was normal. A secretin stimulation test was positive. A small gastrinoma was found in the anterior duodenal wall at exploratory laparotomy. Normal fasting gastrin levels do occur in patients with overt Zollinger-Ellison syndrome and should not deter further investigation if clinical suspicion of this syndrome is high.

Phosphoglycerate kinase San Francisco: a new variant associated with hemolytic anemia but not with neuromuscular manifestations.

Phosphoglycerate kinase deficiency is a rare, x-linked glycolytic defect that, when severe, can be associated with hemolytic anemia, rhabdomyolysis, or neurological disorders. We report here a new phosphoglycerate kinase variant discovered in a boy with severe hemolytic anemia but no evidence of neuromuscular disease or developmental delay. The biochemical properties of the variant enzyme (greatly increased kmATP and km3-phosphoglycerate; normal pH optimum, electrophoretic mobility, and substrate specificity; resistance to heat inactivation) establish its uniqueness. Separation of light and dense red cells by centrifugation showed no greater loss of phosphoglycerate kinase activity in dense ("old") variant cells than in normal cells. We postulate that the striking stability of the variant enzyme allows cells capable of protein synthesis to accumulate sufficient enzyme to limit neuromuscular sequelae.

Prolongation of sickle cell survival by dimethyl adipimidate is compromised by immune sensitization.

The effect of dimethyl adipimidate (DMA), an amino-reactive crosslinking reagent with demonstrated antisickling properties in vitro, on the survival of 51Cr-labeled autologous sickle cells was evaluated in five adult males with sickle cell anemia. The survival of cells pretreated with 5 mmol/L DMA (pH 7.4), normal (t1/2 28-33 days) in four subjects and near-normal (t1/2 20 days) in the fifth, was considerably longer than that usually observed in sickle cell disease. In fact, the effect of DMA on the survival of sickle cells in vivo equals or exceeds that of any other agent tested to date. In three subjects, the survival of a second infusion of DMA-treated red cells was much shorter (t1/2 1.8, 3, 4.5 days) than in the initial study. An antibody was detected in the serum of these subjects that was directed to DMA-treated red cells. Modification of the immunogenicity of treated cells will be required before further consideration of DMA for use in the therapy of sickle cell anemia.