Inhibitors of factor VIII in black patients with hemophilia.

Background: Black patients with hemophilia A (factor VIII deficiency) are twice as likely as white patients to produce inhibitors against factor VIII proteins given as replacement therapy. There are six wild-type factor VIII proteins, designated H1 through H6, but only two (H1 and H2) match the recombinant factor VIII products used clinically. H1 and H2 are found in all racial groups and are the only factor VIII proteins found in the white population to date.

Reinstituting warfarin in patients who develop warfarin skin necrosis.

Skin necrosis is a rare but serious complication of oral anticoagulation with coumarin derivatives. Frequently, the necrosis can be extensive and may result in major morbidity and mortality. The majority of these patients require prolonged anticoagulation for life-threatening conditions such as deep various thrombosis and pulmonary embolism.

Fatal thrombocytopenic coagulopathy after cardiopulmonary bypass: clinicopathologic correlations implicating heparin.

Heparin-associated thrombotic thrombocytopenia after cardiopulmonary bypass is frequently lethal. The propensity for this syndrome generally goes unrecognized because thrombocytopenia is common in the early postoperative period and because testing for heparin-induced platelet antibody may not distinguish between patients with thrombocytopenia alone and those in whom associated thrombi (the white clot syndrome) may develop. Moreover, differentiation between heparin-associated thrombotic thrombocytopenia and a consumptive coagulopathy may be difficult, and intervention may be inappropriate because of diametrically opposite treatments.

When a patient with a bleeding disorder needs dental work. How you can work with the dentist to prevent a crisis.

Patients with bleeding disorders need close cooperation between their physician and their dental practitioner to receive safe, comprehensive dental care. When indicated, physicians must advise a compromised treatment plan to avoid deep injections or surgical procedures that may initiate a bleeding crisis in patients at risk. The conditions most commonly seen that require special consideration are long-term use of antithrombotic agents, platelet dysfunction caused by chronic renal failure, and congenital clotting factor deficiencies.

Streptokinase therapy for deep vein thrombosis: a comprehensive review of the English literature.

A comprehensive review of the English literature evaluating streptokinase (SK) in the treatment of deep venous thrombosis (DVT) of the lower extremity reveals complete lysis of thrombi in as many as 70% of patients studied. The degree of lysis is affected by duration of symptoms before treatment, degree of occlusion, thrombus location, and development of a plasma proteolytic state. SK-treated patients have not been found to have a greater incidence of major hemorrhagic complications when compared with heparin-treated patients.

Hb Evans or alpha 262(E11)Val----Met beta 2; an unstable hemoglobin causing a mild hemolytic anemia.

Structural analysis of the alpha chain of the hemoglobin from a Caucasian female with a mild hemolytic anemia showed the presence of a variant with a Val----Met substitution at position alpha 62. The valine at this position forms one of the contacts with heme and its replacement by methionine will likely decrease heme binding and cause a distortion of the heme crevice and a decreased stability of the abnormal protein. Dot-blot analysis of amplified DNA with 32P-labeled synthetic oligonucleotide probes confirmed the suspected G----A mutation in the first position of codon 62, and also located the mutation in the alpha 2-globin gene.

Desensitization to factor VIII in a patient with classic hemophilia and C2 deficiency.

Factor VIII therapy has been reported to cause anaphylactic reactions in patients with hemophilia. Desensitization attempts have been complicated by severe allergic reactions that have prevented the achievement of protective factor VIII levels. We report successful administration of factor VIII by a graded dose desensitization protocol in a 36-year-old man with hemophilia A who had previously experienced anaphylactic reactions to factor VIII infusions.

Lack of toxicity of oral and intrapulmonary group B streptococcal lipoteichoic acid.

Lipoteichoic acid (LTA) was prepared from type III group B streptococci and administered by topical oral application or intravenous or intratracheal injection in weanling and adult white New Zealand rabbits. Tritiated [3H]LTA in tissues and body fluids was measured by scintillation spectrometry. Five minutes to 120 h after intravenous injection of 10 mg (17 x 10(6) dpm) of [3H]LTA, none was present in blood.

Partial characterization of an erythropoiesis inhibitory factor.

An inhibitory factor of erythropoiesis, obtained from normal human urine, is indicated to be a complex of a fragment of alpha 1-acid glycoprotein and prostaglandin F2 alpha. Immunoelectrophoresis reveals two protein components in the EIF complex which separate during acrylamide gel electrophoresis. A gamma-globulin (MW 185,000) is a carrier of the complex.

Unregulated production of virus and/or sperm specific anti-idiotypic antibodies as a cause of AIDS.

A network of idiotypic and anti-idiotypic antibodies is often suggested as the basis for cellular interactions that maintain a steady-state immunological equilibrium. This hypothesis proposes that repeated exposure to certain external antigens--ie, both viral and sperm--stimulates an unregulated production of a uniquely potent immunomodulating idiotypic antibody(ies). In a genetically predisposed individual, this particular antibody(ies), which is also an autoantibody(ies), results in a cellular immune deficiency.

Chronic erythroid hyperplasia and accelerated bone turnover.

Bone atrophy is generally thought to be the etiology of the decreased skeletal mass and fractures found in patients with ineffective hematopoiesis and associated erythroid hyperplasia. A bone biopsy from a patient with chronic erythroid hyperplasia and diffuse cortical osteopenia revealed a normal trabecular bone volume, excess osteoid, numerous osteoblasts, and increased osteoclastic resorptive surface. The increased fractional labeled surfaces and widely spaced double tetracycline labels indicated accelerated bone turnover, despite demonstrable iron deposits at the calcification front and cement lines and a low serum level of 25-hydroxyvitamin D.

Inhibition of Friend virus (FVP)-induced murine erythropoiesis with prostaglandin (PGF2 alpha): potentiation and inhibition of erythropoietin and prevention of both with PGD2.

An erythropoietin-independent virus-induced murine erythroleukemia (FVP) is used to compare the effects of an erythropoiesis inhibitory factor (EIF) isolated from human urine with the effects of prostaglandin F2 alpha. The consequent inhibition of FVP-induced erythropoiesis suggests that EIF and PGF2 alpha have similar effects on the FVP-induced erythropoiesis in mice, and the effect of PGF2 alpha is indirect. The similarity of the actions of EIF and PGF2 alpha may indicate a potential role for prostaglandins in the physiological control of some types of erythrocytosis.

Effect of intraarterial injection of heparin on the complications of percutaneous arterial catheterization in infants and children.

Thrombotic complications of percutaneous arterial catheterization still remain a significant and serious problem in infants and children. Systemic heparinization has been recommended for prevention of these complications. The purpose of this study was to evaluate the effect of intraarterial injection of heparin in reducing thrombotic complications following percutaneous femoral artery catheterization.

Inhibition of erythropoiesis by plasma component(s) from sheep, goats, and rabbits.

Plasma from hypertransfused and normal sheep and experimentally induced anemic and normal goats was fractionated by ultrafiltration. Fractions obtained were assayed for erythropoiesis stimulatory factor (ESF) or erythropoiesis inhibitory factor (EIF) activity (or both) in the posthypoxic polycythemic mouse assay. The most potent sheep plasma-inhibitor fraction was found in the retentate on a membrane with a cutoff at mol wt 50,000.

Osteosclerotic myeloma.

A 49-year-old man with an osteosclerotic lesion of a thoracic vertebra had peripheral neuropathy, lymphadenopathy, and elevated serum alkaline phosphatase and monoclonal immunoglobulin levels in the cerebrospinal fluid. These findings, not usually seen in ""classical'' myeloma, have been noted in different combinations in the osteosclerotic variety.

Marked cyclic leukocytosis-leukopenia in chronic myelogenous leukemia.

A spontaneous oscillation of the white blood cell count was observed in a 58 year old man with chronic myelogenous leukemia (CML). Similar cyclic variations were noted in the platelet and reticulocyte counts with no apparent alterations in marrow cellularity to account for such changes. Since direct correlation was noted between white blood cells, platelets, and reticulocyte counts versus spleen size, it suggests that splenic hemopoiesis may be responsible for these cyclic changes.