Clinicopathology and histochemistry on bone marrow biopsies in chronic myeloproliferative disorders--a clue to diagnosis and classification.
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.
Thiele
J
J
Institute of Pathology, University of Cologne, Joseph-Stelzmannstr. 9, D-50924 Cologne, Germany. j.thiele@uni-koeln.de
Kvasnicka
H M
HM
D000368:Aged; D001853:Bone Marrow / Q000737:chemistry / Q000473:pathology*; D001856:Bone Marrow Examination / Q000379:methods; D003937:Diagnosis, Differential; D005260:Female; D006400:Hematocrit; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008533:Megakaryocytes / Q000473:pathology; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D010976:Platelet Count; D011086:Polycythemia / Q000175:diagnosis; D011087:Polycythemia Vera / Q000175:diagnosis; D012189:Retrospective Studies; D012907:Smoking / Q000097:blood; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000453:epidemiology; D013922:Thrombocytosis / Q000175:diagnosis / Q000453:epidemiology
D006454:Hemoglobins