Document Detail

Primary (essential) thrombocythemia versus initial (hyperplastic) stages of agnogenic myeloid metaplasia with thrombocytosis--a critical evaluation of clinical and histomorphological data.
MedLine Citation:
PMID:  2474228     Owner:  NLM     Status:  MEDLINE    
In 40 patients (17 male, 23 female, median age 57 years) with the presumptive diagnosis of primary (essential) thrombocythemia (PTH) according to the diagnostic requirements of the Polycythemia-Vera-Study-Group (PVSG) a follow-up study and a histological evaluation of initial trephine biopsies of the bone marrow were performed. Thorough review of the hematological data during the lengthy course of disease (observation time ranging from 1.5-10.5 years) and the histomorphology of the bone marrow implied a discrimination into two groups of patients. Group I patients (n = 26; 10 male, 16 female) were compatible with PTH according to our follow-up studies. Group II patients consisted of 14 cases (7 male, 7 female) which suggested retrospectively early hyperplastic stages of agnogenic myeloid metaplasia (AMM) with concomitant thrombocytosis. In PTH (group I patients) there was a sustained elevation of the platelet count lasting for several years accompanied by stable other blood values. Early AMM (group II patients) was characterized by an insidious decline of the initially elevated thrombocyte count, starting in a few patients already 4-6 months after admission. In AMM there was further an increase in hepatosplenomegaly observable together with the level of LDH and the score of the leukocyte alkaline phosphatase, and finally an evolution of a leukoerythroblastic blood picture could be noticed. Initial histopathology of the bone marrow revealed a profound proliferation of a not severely dysplastic megakaryopoiesis in group I patients (PTH) and a normal content of reticulin fibers. In early thrombocythemic AMM (group II patients) conspicuous abnormalities of megakaryocytes were accompanied by a slight to moderate increase in argyrophilic fibers and a left-shifted neutrophilic granulocyto- as well as erythropoiesis. These differences of certain histomorphological features could be substantiated by morphometric analysis. Our findings suggest that even the rigid requirements for the diagnosis of PTH as proposed by the PVSG may not be sufficiently restrictive to exclude patients with early hyperplastic stages of thrombocythemic AMM.
J Thiele; R Zankovich; T Steinberg; B Kremer; R Fischer; V Diehl
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta haematologica     Volume:  81     ISSN:  0001-5792     ISO Abbreviation:  Acta Haematol.     Publication Date:  1989  
Date Detail:
Created Date:  1989-08-30     Completed Date:  1989-08-30     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0141053     Medline TA:  Acta Haematol     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  192-202     Citation Subset:  IM    
Institute of Pathology, University of Cologne, FRG.
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MeSH Terms
Biopsy, Needle
Bone Marrow / pathology
Diagnosis, Differential
Follow-Up Studies
Middle Aged
Primary Myelofibrosis / blood,  diagnosis*,  pathology
Retrospective Studies
Staining and Labeling / methods
Thrombocythemia, Essential / blood,  diagnosis*,  pathology
Thrombocytosis / blood,  diagnosis*,  pathology

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