Document Detail


Polycythemia rubra vera versus secondary polycythemias. A clinicopathological evaluation of distinctive features in 199 patients.
MedLine Citation:
PMID:  11261821     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
To determine parameters of distinctive value in polycythemia rubra vera (PV) versus secondary polycythemias (SP), a clinicopathological study was performed on 199 patients. These presented with a borderline to marked elevation of the hemoglobin level (> 18 g/dl in men and > 16 g/dl in women). Evaluations of clinical features and bone marrow histopathology were carried out independently. According to the results derived from laboratory data and representative pretreatment trephine biopsies, three groups of patients emerged: group I presenting with the concordant clinical and morphological findings of early to manifest PV (136 patients), group II consisting of 55 patients with the congruent signs and symptoms of SP mostly caused by various chronic bronchopulmonal disorders, and finally eight patients (group III) with divergent findings. Between group I and II patients (PV versus SP), a number of clinical parameters proved to be significantly different. With the exception, of the red cell mass, platelet count, leukocyte alkaline phosphatase, LDH, spleen size, and the erythropoietin level had a significantly discriminating impact. Morphological features of distinctive value consisted of a set of specific lesions. Contrasting SP with an only borderline to slight increase in cellularity associated with a moderate enlargement of the erythroblastic islets, PV was always characterized by a significant increase in hematopoiesis, revealing a trilinear proliferation (panmyelosis). Megakaryopoiesis was strikingly different in PV as compared to SP by displaying clustering and a pleomorphous appearance. i.e., very small and giant megakaryocytes with staghorn-like nuclei were neighboring each other. Moreover, conspicuous alterations of the interstitial compartment were recognizable in SP. These consisted of deposits of cell debris in histiocytic reticular cells, iron-laden macrophages, and a plasmacytosis, implying an inflammatory reaction. These changes were only very rarely observed in PV, as opposed to a minimal to slight increase in reticulin fibers in about 12% of patients. In conclusion, a more elaborate evaluation of bone marrow features resulted in a set of diagnostic criteria with discriminating capacity that should be considered in prospective clinical trials.
Authors:
J Thiele; H M Kvasnicka; K Muehlhausen; S Walter; R Zankovich; V Diehl
Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Pathology, research and practice     Volume:  197     ISSN:  0344-0338     ISO Abbreviation:  Pathol. Res. Pract.     Publication Date:  2001  
Date Detail:
Created Date:  2001-03-22     Completed Date:  2001-06-21     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7806109     Medline TA:  Pathol Res Pract     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  77-84     Citation Subset:  IM    
Affiliation:
Institute of Pathology, University of Cologne, Germany.
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MeSH Terms
Descriptor/Qualifier:
Bone Marrow / pathology*
Bone Marrow Examination
Bronchitis / complications,  pathology
Bronchopneumonia / complications,  pathology
Chronic Disease
Female
Hematopoiesis
Humans
Macrophages / pathology
Male
Megakaryocytes / pathology
Middle Aged
Polycythemia / etiology,  pathology*
Polycythemia Vera / etiology,  pathology*
Retrospective Studies
Single-Blind Method

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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