The Italian Registry of Myelofibrosis with Myeloid Metaplasia (RIMM) was born in 1998 as to establish a national research network devoted to diagnosis and care for myelofibrosis. By the end of 1999, the network covered nearly exhaustively the Italian hematology and pathology units and included one third of the internal medicine wards. A prospective register was thus started on June 1999 to collect newly diagnosed patients. By June 2000 190 patients were recorded: according to the Italian Consensus Conference on diagnostic criteria, 90 patients had sufficient criteria, while 50 still missed some; further 10 patients were excluded (diagnosis before June 1999 or alternative diagnoses proved) and 40 were referred by pathologists only. Alternative diagnoses were: myelodysplastic syndrome with bone marrow fibrosis (3 patients), paroxysmal nocturnal hemoglobinuria ( 1 patient), atypical myeloproliferative disorder (1 patient) and spent polycytemia vera ( 1 patient). Mean and median age of the enrolled patients were 69 and 72 years respectively, with a male to female ratio of 2.1. Sixty-two percent of the patients were referred by internists. The mean age of those referred by the hematologists was significantly lower than by the internists: 69 versus 73 years. Mean spleen length at diagnosis was 18 cm on US: in 11 patients (8.4%) it was shorter than 12 cm and in 25 ( 19%) it was longer than 20 cm. Mean value of hemoglobin at diagnosis was 10 g/dL (range: 3.9-20). Of the diagnostic criteria, clusters of atypical megakaryocytes in bone marrow were present in 72% of the patients. Reliable data on peripheral blood smears were available for 52% patients: they reported immature circulating cells (94%) and tear drop cells (76%). By analyzing the RIMM forms, we could track 5 diagnostic pathways: I) 17% of the patients evolved from a previous myeloproliferative disease; 2) 15% received the diagnosis during hospital stay for an acute event unrelated to the disease; 3) 20% for an acute event related to the disease; 4) 25% were diagnosed incidentally at check up for another disease and 5) in 23% the diagnosis was made by a hematologist consulted for anemia-driven fatigue. We could also track the lead time to diagnosis: > 2 years in 84% of the patients. Cluster analysis and CART discriminated subpopulations of patients according to plasma values of lactic dehydrogenase, platelets and neutrophil count. The three main subpopulations significantly differed for the overall risk (according to Dupriez). The first year actuarial splenomegaly progression was 2 cm/ year and mortality 19%/ year. We can conclude that the RIMM is a potentially valuable and precious tool to study an unselected population of patients with newly diagnosed myelofibrosis. We are now verifying the completeness of referrals and starting clinical trials for enrolled patients.
|Issue number||11 PART II|
|Publication status||Published - 2000|
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