Tuberculosis associated thrombocytopenic purpura: effectiveness of antituberculous therapy

Association of immune thrombocytopenic purpura and tuberculosis is a rare condition. In 5 patients presenting with this association, anti-tuberculous therapy was effective on both tuberculosis and thrombocytopenia suggesting a causal relationship between tuberculosis and immune thrombocytopenic purpur

Intravascular lymphoma presenting as a specific pulmonary embolism and acute respiratory failure: a case report

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B cell depletion in immune thrombocytopenia reveals splenic long-lived plasma cells.

International audiencePrimary immune thrombocytopenia (ITP) is a disorder caused by autoantibody-mediated platelet destruction and decreased platelet production. Rituximab, a B cell-depleting agent, has become the first-line treatment for ITP; however, patients with refractory disease usually require splenectomy. We identified antibody-secreting cells as the major splenic B cell population that is resistant to rituximab. The phenotype, antibody specificity, and gene expression profile of these cells were characterized and compared to those of antibody-secreting cells from untreated ITP spleens and from healthy tissues. Antiplatelet-specific plasma cells (PC) were detected in the spleens of patients with ITP up to 6 months after rituximab treatment, and the PC population displayed a long-lived program similar to the one of bone marrow PC, thus explaining for most of these patients the absence of response to rituximab and the response to splenectomy. When analyzed by multiplex PCR at the single-cell level, normal splenic PC showed a markedly different gene expression profile, with an intermediate signature, including genes characteristic of both long-lived PC and proliferating plasmablasts. Surprisingly, long-lived PC were not detected in untreated ITP spleens. These results suggest that the milieu generated by B cell depletion promotes the differentiation and settlement of long-lived PC in the spleen

Br J Haematol

International audienc

Maladie de Castleman multicentrique non associée à l'infection VIH (une ou plusieurs maladies?)

LE KREMLIN-B.- PARIS 11-BU Méd (940432101) / SudocSudocFranceF

Déficit sélectif en IgM (manifestations cliniques et caractéristiques immunologiques chez 10 patients)

Le déficit sélectif en IgM n est pas individualisé dans la classification internationale des déficits immunitaires primitifs. Les données de la littérature suggèrent une association avec diverses manifestations infectieuses, allergiques et auto-immunes. Nous avons étudié les manifestations cliniques et les caractéristiques immunologiques des patients inclus dans les cohortes DEFI et CEREDIH ayant un déficit sélectif en IgM. 29 patients étaient inclus dans ces cohortes avec un diagnostic d hypo IgM ; 19 ont été exclus car il avaient un déficit en sous-classes d IgG ou en IgA associé, un dosage des sous-classes non renseigné ou un déficit immunitaire complexe associé. 10 patients avaient un déficit sélectif en IgM. 50% des patients ont présenté au moins une infection sévère, comprenant des infections à pneumocoque mais également à d autres bactéries à cocci gram positif (streptocoques et staphylocoque doré). Ces infections sévères ne semblaient pas corrélées au taux d IgM sérique. L anomalie la plus fréquente sur le phénotypage lymphocytaire était une diminution des lymphocytes B de la zone marginale (8 patients). Le déficit sélectif en IgM est rare. Dans notre série, les infections sévères à pneumocoque sont fréquentes mais ne semblent pas récidivantes chez un même patient. Ces infections sévères justifient une prophylaxie anti-infectieuse par les vaccinations, avec une évaluation de la réponse vaccinale. L éducation du patient sur la conduite à tenir en cas de fièvre est également un élément important de la prise en charge.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF

A Web-Based Delphi Study for Eliciting Helpful Criteria in the Positive Diagnosis of Hemophagocytic Syndrome in Adult Patients

International audienceThe diagnosis of the reactive form of hemophagocytic syndrome in adults remains particularly difficult since none of the clinical or laboratory manifestations are specific. We undertook a study in order to elicit which features constitute helpful criteria for a positive diagnosis. In this Delphi study, the features investigated in the questionnaire and the experts invited to participate in the survey were issued from a bibliographic search. The questionnaire was iteratively proposed to experts via a web-based application with a feedback of the results observed at the preceding Delphi round. Experts were asked to label each investigated criterion in one of the following categories: absolutely required, important, of minor interest, or not assessable in the routine practice environment. A positive consensus was a priori defined as at least 75% answers observed in the categories absolutely required and important. The questionnaire investigated 26 criteria and 24 experts originating from 13 countries participated in the second and final Delphi round. A positive consensus was reached for the nine following criteria: unilineage cytopenia, bicytopenia, pancytopenia, presence of hemophagocytosis pictures on a bone marrow aspirate or on a tissue biopsy, high ferritin level, fever, organomegaly, presence of a predisposing underlying disease, and high level of lactate dehydrogenase. A negative consensus was reached for 13 criteria, and an absence of consensus was observed for 4 criteria. The study constitutes the first initiative to date for defining international guidelines devoted to the positive diagnosis of the reactive form of hemophagocytic syndrome