Platelet autoantibodies and lupus-associated thrombocytopenia.

To characterize the antigenic targets of anti-platelet antibodies (APA) found in systemic lupus erythematosus (SLE)-associated thrombocytopenia, 48 patients with immune thrombocytopenia and SLE were compared with 20 patients with SLE who had never been thrombocytopenic. Both cases and controls were tested for circulating APA by an indirect platelet suspension immunofluorescence assay (PSIIFT) and by indirect monoclonal antibody specific immobilization of platelet antigens (MAIPA). A direct platelet suspension immunofluorescence assay (PSIFT) was also used for antibodies bound to platelets in vivo in thrombocytopenic patients; 13 of them with high titres of platelets-bound APA were investigated by direct and indirect MAIPA and platelet eluate analysis.

Autoimmune thrombocytopenic Purpura and Helicobacter pylori infection.

Background: The mechanisms triggering the production of platelet autoantibodies in autoimmune thrombocytopenic purpura (AITP) are poorly understood. Recently, marked improvements in platelet counts have been reported in patients with AITP and concurrent Helicobacter pylori infection after eradication of H pylori by a standard antibiotic regimen. We looked for an association between H pylori infection and AITP in adults.

Patterns of mortality in sickle cell disease in adults in France and England.

Introduction: An understanding of the causes of death among patients with sickle cell disease may be informative for both epidemiology and pathogenesis. This information should aid anticipation of dangerous clinical conditions, counselling patients and design of preventive therapies.

Patients And Methods: All deaths known to four European sickle cell disease centres over a 10-year period were retrospectively analysed.

Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases.

Objective: To evaluate the response to treatment in a large cohort of patients with systemic lupus erythematosus (SLE) associated with autoimmune thrombocytopenia.

Methods: Response to treatment was assessed retrospectively in 59 patients with SLE, either definite (n = 44) or incomplete (n = 15), associated with frank autoimmune thrombocytopenia (defined as platelet count < 50 x 10(9)/l). Response to treatment was classified as complete (CR: platelet count > 150 x 10(9)/l), partial (PR: platelet count > 50 x 10/l), or failure (FR) in the other cases.

Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial.

Background: Treatment of adults with autoimmune thrombocytopenic purpura (AITP) is based more on individual experience than on results of controlled studies. We compared intravenous immunoglobulin with high-dose methylprednisolone in untreated adults with severe AITP and assessed efficacy of subsequent oral steroids compared with placebo. Primary outcome was number of days with platelet count greater than 50 x 10(9)/L within the first 21 days.

The nonexpression of CD36 on reticulocytes and mature red blood cells does not modify the clinical course of patients with sickle cell anemia.

It is thought that an increase in the adhesion of circulating reticulocytes to the vascular endothelium may initiate the vascular occlusion underlying the painful crises and organ failures typical of sickle cell disease (SCD). At least 2 receptors, usually present on reticulocytes, seem to be involved in this adhesion process: glycoprotein CD36 (glycoprotein IV) and integrin alpha(4)beta(1) (very late activation antigen--4). Recently, a high frequency of the platelet CD36--deficient phenotype was reported in black Africans.

[Treatment of major sickle cell syndromes. The point of view of the internist].

Major sickle cell syndromes constitute at present the most important risk of genetic disease in the wider Paris area. Sickle cell patients are exposed to vaso-occlusive attacks and to chronic visceral complications of ischemic origin capable of affecting all organs. Acute vaso-occlusive complications represent the first cause of hospitalisation and the main cause of death for the adult patient.

[Sickle cell disease in adults: which emergency care by the internists?].

Introduction: Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the most prevalent genetic disease at birth in the Ile-de-France area. Internists are involved in the management of acute complications, particularly acute vaso-occlusive crisis.

[Hemolytic anemia disclosing Wilson's disease. Report of 2 cases].

Introduction: The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease.

Exegesis: We report two cases of Wilson's disease revealed by acute intravascular hemolytic anemia associated with liver failure.

Acute chest syndrome in adults with sickle cell disease.

Study Objectives: Acute chest syndrome (ACS) is a frequent and potentially severe pulmonary illness in sickle cell disease (SCD). The aim of the study was to report the clinical features and outcome of consecutive ACS episodes in adult patients in a French SCD center. All patients were treated according to an uniform therapeutic protocol applying transfusion only in the more severe clinical form of ACS.

Screening for autoimmune markers is unnecessary during follow-up of adults with autoimmune thrombocytopenic purpura and no autoimmune markers at onset.

In an attempt to evaluate the frequency of autoimmune markers in autoimmune thrombocytopenic purpura (AITP) and to determine if autoimmune markers in patients with isolated AITP were associated with particular disease manifestations, we analyzed records of 122 consecutive adults with AITP. Twenty-nine patients (24%) had significant titers of one or several autoimmune markers at AITP onset. Among them, 16 (13%) had antinuclear antibodies.

Intravenous immunoglobulin for adults with autoimmune thrombocytopenic purpura: results of a randomized trial comparing 0.5 and 1 g/kg b.w.

Since the first reports demonstrating the ability of a total dose of 2 g/kg body weight (b.w.) of intravenous immunoglobulin (IVIg) to increase the platelet count in patients with autoimmune thrombocytopenic purpura (AITP), the optimal dose has remained controversial.

Lack of relevance of the acetylator status on dapsone response in chronic autoimmune thrombocytopenic purpura.

Dapsone provides an alternative treatment for patients with chronic autoimmune thrombocytopenic purpura (AITP) who had inadequate response to conventional therapy. However, the efficacy of this treatment is achieved in only 50% of patients. Dapsone is partly metabolized by the polymorphic N-acetyltransferase 2% and 50% of Caucasian patients show a genetically determined slow acetylator phenotype.

The response to high-dose intravenous immunoglobulin or steroids is not predictive of outcome after splenectomy in adults with autoimmune thrombocytopenic purpura.

The response to high-dose intravenous immunoglobulin (IVIg) was recently reported to be predictive of outcome after splenectomy in patients with autoimmune thrombocytopenic purpura (AITP). We analysed the records of 75 adults with chronic AITP who received IVIg and subsequently underwent splenectomy. There was no significant difference in the response rate to splenectomy according to whether or not patients had responded to IVIg (81% v 67%, P = 0.

[Intravenous immunoglobulins and autoimmune thrombopenic purpura].

Objective: To determine the usefulness of intravenous immunoglobulin in the treatment of autoimmune thrombocytopenic purpura (AITP).

Results: IVIg infusion is an important agent in managing AITP because most patients experienced a rapid increased platelet count. Its precise place in the management of these patients is however controversial, particularly on account of its high cost and transient effect.

CD36 deficiency is frequent and can cause platelet immunization in Africans.

Background: CD36 is expressed on several cell lineages. About 5 to 10 percent of Asians lack platelet membrane CD36 (pCD36), but the frequency of pCD36 deficiency in other ethnic groups is not known. Persons who are pCD36-negative are apparently healthy but can develop CD36 isoimmunization.

Acute swelling of the limbs: magnetic resonance pictorial review of fascial and muscle signal changes.

Objective: This pictorial review analyzes the magnetic resonance (MR) fascial/muscular changes in 69 patients referred as emergencies with acute swelling of the limbs (ASL) from various causes.

Methods And Material: A prospective MR imaging (MRI) study of 69 patients referred as emergencies for ASL was performed. Our population consisted of 45 patients with skin and soft-tissue infections (cellulitis and necrotizing fasciitis, and pyomyositis), six patients with soft-tissue inflammatory diseases (dermatomyositis, graft-versus-host disease), 11 patients with acute deep venous thrombosis, three patients with rhabdomyolysis, one patient with acute denervation and three other patients with rare diseases.

[Hemolysis and schizocytosis, malabsorption and the "folate trap": unusual semiological peculiarities associated with vitamin B12 deficiency].

Introduction: Hemolysis and red cell fragmentation accompanying vitamin B12 deficiency may misdirect the diagnosis. Signs of malabsorption and abnormalities related to folic acid metabolism characterized by discrepancies between folic acid normal serum levels and erythrocytic folic acid levels may also exist.

Exegesis: We report the occurrence of hemolysis and red cell fragmentation mimicking microangiopathic hemolytic anemia, malabsorption and folic acid deficiency in the course of vitamin B12 deficiency.

Pulmonary embolism; lung scanning interpretation: about words.

Objective: To assess clinicians' agreement on how they interpret lung scan reports with regard to the diagnosis of pulmonary embolism.

Design: In this prospective study, nuclear medicine physicians provided two types of reports for each lung scan: a routine descriptive report and a short form with a standardized conclusion on the likelihood of pulmonary embolism: "high probability," "no conclusion," and "diagnosis excluded." Three independent blinded senior clinicians reviewed all routine reports and chose one of the following conclusions: "high probability," "no conclusion," or "diagnosis excluded.

Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare variant of plasma cell dyscrasia with multiple systemic manifestations. We followed the progress of 20 patients with POEMS syndrome in our institution over a 10-yr period. Pulmonary hypertension (PH) was observed in five patients.