À propos d’un cas de leucémie plasmocytaire primitive à IgE

International audienceWe report here a case of primitive plasma cell leukemia with immunoglobulin (Ig) E. IgE myeloma is an exceptional variant of multiple myeloma, with a very poor prognosis. Its biological diagnosis requires specific analyzes in order to detect IgE gammopathy. Plasma cell leukemia (PCL) is also a very rare and very severe form of multiple myeloma. There are two variants: primitive PCL (pPCL) occurring de novo and secondary PCL (sPCL), evolution of a preexisting myeloma. Its diagnosis is essentially biological since it is defined by a blood plasmocytosis greater than 2 G/L or 20% of the leucocytes.Nous rapportons ici un cas de leucémie plasmocytaire primitive à immunoglobulines (Ig) E. Le myélome à IgE est une variante exceptionnelle du myélome multiple, associée à un pronostic très péjoratif. Son diagnostic biologique nécessite la réalisation d’analyses spécifiques pour la mise en évidence d’une gammapathie à IgE. La leucémie à plasmocytes (LP) est également une forme très rare et grave du myélome multiple. Il en existe deux variantes : la LP primitive (LPp) survenant de novo et la LP secondaire (LPs), évolution d’un myélome préexistant. Le diagnostic de la LP est essentiellement biologique puisqu’elle est définie par l’existence d’une plasmocytose circulante supérieure à 2 G/L ou 20 % des leucocytes

A primitive plasma cell leukemia with immunoglobulin (Ig) E

International audienc

Harmonisation of full blood count reports, recommendations of the French-speaking cellular haematology group (GFHC)

To propose recommendations related to the presentation, content and formulation of full blood count analysis reports

In vivo and in vitro sensitivity of blastic plasmacytoid dendritic cell neoplasm to SL-401, an interleukin-3 receptor targeted biologic agent.

International audienceBlastic plasmacytoid dendritic cell neoplasm is an aggressive malignancy derived from plasmacytoid dendritic cells. There is currently no accepted standard of care for treating this neoplasm, and therapeutic strategies have never been prospectively evaluated. Since blastic plasmacytoid dendritic cell neoplasm cells express high levels of interleukin-3 receptor α chain (IL3-Rα or CD123), antitumor effects of the interleukin-3 receptor-targeted drug SL-401 against blastic plasmacytoid dendritic cell neoplasm were evaluated in vitro and in vivo. The cytotoxicity of SL-401 was assessed in patient-derived blastic plasmacytoid dendritic cell neoplasm cell lines (CAL-1 and GEN2.2) and in primary blastic plasmacytoid dendritic cell neoplasm cells isolated from 12 patients using flow cytometry and an in vitro cytotoxicity assay. The cytotoxic effects of SL-401 were compared to those of several relevant cytotoxic agents. SL-401 exhibited a robust cytotoxicity against blastic plasmacytoid dendritic cell neoplasm cells in a dose-dependent manner. Additionally, the cytotoxic effects of SL-401 were observed at substantially lower concentrations than those achieved in clinical trials to date. Survival of mice inoculated with a blastic plasmacytoid dendritic cell neoplasm cell line and treated with a single cycle of SL-401 was significantly longer than that of untreated controls (median survival, 58 versus 17 days, P<0.001). These findings indicate that blastic plasmacytoid dendritic cell neoplasm cells are highly sensitive to SL-401, and support further evaluation of SL-401 in patients suffering from blastic plasmacytoid dendritic cell neoplasm

Isolated isochromosomes i(X)(p10) and idic(X)(q13) are associated with myeloid malignancies and dysplastic features

International audienc

East Asian capitalism: Towards a normative framework

This essay attempts to identify the features of East Asian capitalism that seem, prima facie, to serve desirable social and political purposes while also being compatible with, if not beneficial for, the requirements of economic productivity in an age of intense international competition. It argues that East Asian countries should build on areas of competitive advantage instead of heeding calls to shed all the old ways of doing things in favor of American-style shareholder capitalism.