Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Variants of chronic myeloproliferative disorders (CMPD) were compared according to their clinical features and classified by bone marrow biopsy appearances. Subsequently, this classification was further evaluated using survival data and histological variables from iliac crest biopsy specimens of an additional 1391 patients, making a total of 2241 patients available for analysis of outcome. The patients were grouped again into three main classes: "typical"; "variant"; and "transformed". "Typical" comprised the "classic" groups. "Variant" included the less uniform myeloproliferative syndromes, distinguished also by more variable clinical features, a different prognosis, and a greater tendency to fibrotic and blastic transformation. "Transformed" defined the end stages of both "typical" and "variant" types. Ten subgroups were distinguished by different histology and prognosis. Particular prognostic importance was assigned to atypia and immaturity of haemopoiesis, predominance of individual haemopoietic cell line, number and anomalies of megakaryocytes and progressive fibrosis. It is suggested that the proposed subclassification would be helpful for studies of epidemiology and therapeutic trials by allowing more homogeneous groups to be recognised.
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PMID:Chronic myeloproliferative disorders: prognostic importance of new working classification. 237 Mar 4

Spleen is a common site of extramedullary hematopoiesis. Extramedullary hematopoiesis seen in non-neoplastic conditions can occasionally be extensive and raise concerns for a myeloid neoplasm. We compared the morphologic and immunohistochemical features of splenic hematopoietic proliferations seen in neoplastic myeloid disorders (eg chronic myeloproliferative disorders, myelodysplastic/myeloproliferative disorders and acute myeloid leukemias) to extramedullary hematopoiesis seen in a variety of reactive conditions. In all, 80 spleen specimens were reviewed. The presence of each marrow-derived lineage, dysplasia and immunohistochemical results were evaluated (CD34, CD117, myeloperoxidase, CD68, p53, TdT, CD42b and hemoglobin). Neoplastic hematopoietic proliferations in chronic myeloproliferative disorders are characterized by trilineage hematopoiesis with significant dysplasia in all cell lineages. Acute myeloid leukemia showed an increase in immature forms, which were highlighted by immunohistochemistry. Reactive extramedullary hematopoiesis showed variability in histologic features. Post-bone marrow transplant and thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome spleens showed extramedullary hematopoiesis with some morphologic features of immaturity, which could simulate chronic myeloproliferative disorder. However, they lacked characteristic immunohistochemical features of neoplastic myeloid disorders such as positivity for CD34 or CD117.
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PMID:Morphologic and immunohistochemical evaluation of splenic hematopoietic proliferations in neoplastic and benign disorders. 1611 26