Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A clinicopathological study was carried out to address the currently still controversial issues of: 1) thrombocythaemias in chronic myeloproliferative disorders (MPDs); 2) Initial, prefibrotic idiopathic myelofibrosis (IMF); 3) discrimination of spurious polycythaemic states or polyglobuly (PG) from polycythaemia vera (PV); 4) unclassifiable MPDs. Based on a synoptical approach which implicates a comparative evaluation of laboratory data and histopathology of the bone marrow, the discriminating efficiency of both diagnostic tools has been emphasized. An elaborate evaluation of histotopography and cytological appearance of megakaryopoiesis is an invaluable aid to distinguish the different subtypes of MPDs which may eventually present with a significant elevation of the platelet count. Prefibrotic IMF is not only associated with a certain set of clinical symptoms (minimal to slight anaemia, splenomegaly, thrombocytosis), but should also be characterized by specific alterations of bone marrow morphology. Moreover, follow-up studies are in keeping with the finding that these patients evolve into typical IMF regarding laboratory parameters and ensuing myelofibrosis. Smokers polycythaemia--PG may be separated from early PV by the significant raise in the red cell mass and also by a few, easily determinable clinical parameters (i.e. EPO level, thrombocytosis, LAP). Both conditions can be distinguished by regarding bone marrow features (megakaryopoiesis, interstitial changes) which exert a distinctive impact. According to our experience the majority of patients categorized as unclassifiable MPDs include cases in which clinical or morphological data are inadequate to permit a more precise diagnosis. Only in a small proportion not a failing methodology, but initial stages of the disease process requires sequential examinations to reach a correct diagnosis.
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PMID:Clinicopathology and histochemistry on bone marrow biopsies in chronic myeloproliferative disorders--a clue to diagnosis and classification. 1131 59

Diagnosis of essential thrombocythemia (ET) has been usually established by regarding the criteria of the Polycythemia Vera Study Group. Accordingly, a retrospective clinicopathological study was performed on 120 patients with a follow-up ranging between 5 and 13 years and repeated bone marrow trephine examinations. Following the new WHO classification, at presentation patients revealed three distinctive patterns of bone marrow (BM) features: (true) ET in 43 patients, prefibrotic idiopathic myelofibrosis (IMF) in 50 patients, and early IMF in 27 patients. Heterogeneity of morphological features was associated with correspondingly expressed laboratory data. Contrasting initial and early IMF, patients with true ET displayed an about 80% probability to lack splenomegaly, anemia, and increase in the LDH and LAP values and also failed to show any myeloblasts or erythroblasts on the peripheral blood films. Follow-up examinations including sequential BM biopsies (mean interval 39 +/- 31 months) disclosed that of the 43 patients with true ET only one developed an increase in reticulin. On the other hand, 65 of 77 patients with prefibrotic and early IMF evolved into overt myelofibrosis-osteosclerosis. Moreover, survival analysis demonstrated significant differences in our patients. A neglectable proportion of life loss according to a sex- and age-matched general population was found in true ET (less than 11%) opposed to IMF without or mild fibrosis (range 21% to 32%).
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PMID:Follow-up examinations including sequential bone marrow biopsies in essential thrombocythemia (ET): a retrospective clinicopathological study of 120 patients. 1221 Aug 9