Homo sapiens
Ontology highlight
ABSTRACT:
PROVIDER: PRJNA97193 | ENA |
REPOSITORIES: ENA
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Project description:Comparison of gene expression data from thalidomide modulated- and unmodulated-MSCs from patients with idiopathic thrombocytopenic purpura (ITP). This experiment consists of 3 arrays.
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Project description:Whole blood transcriptional profiling of pediatric idiopathic thrombocytopenic purpura (ITP) patients comparing self-limited acute ITP patients with chronic ITP patients or progression-to-chronic ITP patients. Patient samples were collected during 3 different disease states: 8 samples from acute ITP pts (within 6 months of dx, during active disease), 4 samples from progression-to-chronic ITP pts (within 6 months of dx), and 14 samples from chronic ITP (after 6 months of dx).
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Project description:Splenic tissues from immune thrombocytopenia purpura (ITP) patients splenectomized after primary failure of treatment with the B-cell depleting agent rituximab were analyzed, and antibody-secreting cells were identified as the major B-cell population resisting the treatment. The phenotype, antibody specificity and gene expression profile of these cells were characterized and compared to antibody-secreting cells from untreated ITP spleens and from healthy tissues. Anti-platelet-specific plasma cells (PC) were detected in the spleen of ITP patients 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 singlecell 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 either in the inflammatory environment of the untreated ITP spleen. These results suggest that neither the normal nor the auto-immune splenic environment favors the differentiation and residence of long-lived PC, and that it is most probably the milieu generated by B-cell depletion that promotes their local settlement.
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