A family is reported with multiple cases of neonatal neutropenia. The first child died of severe infection, the second was asymptomatic, the third and the fourth children had a benign course of agranulocytosis which lasted about 10 weeks and was followed by spontaneous recovery. A potent leukoagglutinin was found in the maternal serum which agglutinated leukocytes obtained from the father and all three available children but failed to agglutinate the mother’s own cells. Identical leukoagglutinin was found in the last baby’s serum; it disappeared after the baby recovered. The disorder is tentatively named "isoimmune neonatal neutropenia" and is considered to represent a condition analogous to erythroblastosis fetalis.
Ninety-five of 322 patients were found to have diminished plasma fibrin stabilizing factor (FSF) activity as measured by a modification of a threshold assay method.
Repeat assays were normal in 85 of these 95 specimens after the addition of cysteine or other sulfhydryl-containing compounds to the plasma, suggesting that FSF was inactive in these instances.
Abnormal specimens obtained from six patients with acute granulocytic leukemia, and three obstetrical patients with hypofibrinogenemia had decreased concentrations of FSF since the repeated assays were normal after 6 hours of incubation but not after the addition of cysteine. One abnormal specimen obtained from a severe cirrhotic in hepatic coma failed to show normal FSF activity after the addition of cysteine, after incubation and after mixing with equal volumes of normal plasma. This suggested the presence of an inhibitor of FSF. No specific correlation could be made between diminished FSF activity and hemorrhagic symptoms.
It is proposed that normal hepatic function is necessary for the activation of FSF or for the removal of an inhibitor.
Platelet-associated IgG (PAIgG) was measured by a simple rapid nephelometric technique using washed solubilized platelets and commercially available, prestandardized reagents. Normal subjects with normal platelet counts had PAIgG levels of 2.1-6.7 fg/platelet. Subjects with idiopathic immune thrombocytopenic purpura (ITP) had levels of 7.2-43.3 fg/platelet. Ninety percent of ITP patients had values exceeding 2 SD units of the mean of normal subjects. Elevated values were also found in 17% of patients with recovered ITP, patients with SLE with and without thrombocytopenia, patients with thrombocytopenia occurring during septicemia, and patients with IGg myeloma. Results can be obtained within several hours of receipt of blood specimen, and are similar to the reports that used more complex techniques.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.