Diagnosis, pathogenesis and treatment of the myeloproliferative disorders essential thrombocythemia, polycythemia vera and essential megakaryocytic granulocytic metaplasia and myelofibrosis

Polycythemia Vera era 1939-1998 from Berkeley to Rotterdam and Change of PVSG into ECP Criteria of Myeloproliferative Disorders ET, PV and PMGM: Proceedings of the First Rotterdam MPD Workshop 1998 and beyond

“…I look forward to the ECLAP study to answer the question can aspirin lower the hemorrhagic and thrombotic complications [21]. As I read and re-read some of the recent literature [22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41], I must say that I do not come before you today with original ideas of what should be done. I can only add my choice to that of others and last for the sin of omission in failing to recognize many of the contributions of those here today I apologize and ask forgiveness and for not mentioning only the senior authors in this talk I apologize.…”

“…Reviews of the literature anno 1998 indicate that an overall hematological response has been achieved in more than 75% of cases within 6 to 12 months [38,58]. Doses of rIFN-alpha used to obtain hematological response with cloud-like nuclei which are not seen in Ph-ET and PV and Ph + ET and CML (Table 5 and Figures 7 and 8) [52][53][54][55][56][57]. Myelofibrosis in ET, PV and CMGM is graded in no reticulin fibrosis (MF 0), early reticulin fibrosis (MF 1), advanced reticulin fibrosis with minor collagen fibrosis (MF 2) and advanced collagen fibrosis with or without osteosclerosis (MF 3) [25,[61][62][63][64][65].…”

“…The early prefibrotic stages stages of AMM or prePMF (Table 5, Figures 7 and 8) and fibrotic stages of AMM or PMF with anemia and large spleens without any PV features according to the PVSG appears to be a distinct dual chronic or primary megakaryocytic granulocytic myeloproliferation (CMGM/PMGM) in the Hannover Bone Marrow Classification of the MPDs [56,57]. The histopathology of the bone marrow in PMGM is dominated by atypical enlarged (larger than in ET and PV) and immature megakaryocytes…”

“…The morphology and clustering of large mature and pleiomorphic megakaryocytes are indentical in ET, prodromal PV and early stage PV in both ET and PV who presented with ETT ( Figure 6). Michiels Ten Kate and van Vliet recognized ETT in ET and PV are very likely caused by hypersensitive thrombocythemia platelets, which spontaneously activate at high hear in the endarterial circulation and secrete their products thus forming aggregates that transiently plug the microcirculation or result in occlusive platelet and venous thrombosis, but fails to prevent the platelet-mediated microvascular circulation disturbances because thrombocythemia in PV in remission by phlebotomy alone persisted [18,50] Figure 5) [20,28,30,48,50,51,[52][53][54][55][56].…”

The 2006 → 2008 European Clinical, Molecular and Patholobiogical (2008 ECMP) Classification and the 2007 WHO Revised Criteria for Myeloproliferative Neoplasms

“…[16,48,49]. [22][23][24][25][26][27][28][29][30] and the 2008 ECMP classification respectively are based on three specific bone marrow histology criteria: 1) the presence of large dysmorphic megakaryocytes with immature cytoplasm and immature cloud-like nuclei not seen in ET and PV, 2) increased granulopoiesis but never disturbed in maturation and 3) no features of PV with relatively decreased erythropoiesis. 5 RCM in PV patients corresponded to hematocrit values between 0.48 and 0.76 in all, platelet count above 400 x10 9 /L in two-third and palpable spleen in two-third of about 400 PV patients in the PVSG-01 study [13].…”