Myeloproliferative Syndrome

Werner's Syndrome with Myelofibrosis Following Diphenylhydantoin Therapy after Meningiomectomy

“…As far as can be judged from histological similarity, myelofibrosis might have been caused by the administration of DPH. However, opinions differ as to whether myelofibrosis is mesenchymal 20 or lymphoreticular in origin, 21 and occurrence of myelofibrosis in Werner's syndrome can not be completely ruled out 18 . Moreover, the occurrence of myelofibrosis during DPH medication has not been previously reported.…”

The Optimal Management of Polycythaemia Vera

“…Furthermore, even a haematocrit of 45% would still be too high for some women (Drenou et al , 1992; Lamy et al , 1997). In essence, the effectiveness of phlebotomy in this, the only randomized, prospective, therapeutic trial for polycythaemia vera, and the treatment option most unpopular with the organizing committee (Berlin, 1995), was rendered unevaluable. Thus, the contentions that both phlebotomy per se and an increased phlebotomy requirement were associated with an increased risk of thrombosis (Berk et al , 1986) are unproved; the former because of study design and the latter because the phlebotomy requirement in polycythaemia vera is directly related to the available iron supply and not to disease tempo (Finch et al , 1950; Messinezy et al , 1985).…”

“…First, polycythaemia vera is a clonal disorder with the primary defect residing in a multipotent haematopoietic progenitor cell (Adamson et al , 1976). Second, there is currently no clinically applicable clonal marker to distinguish polycythaemia vera from non‐clonal disorders when it presents with erythrocytosis (Szur et al , 1959; Najean et al , 1981) or from its' companion myeloproliferative disorders when it does not (Lopas & Josephson, 1964; Parmentier et al , 1977; Taylor et al , 1989; Janssen et al , 1990). Third, without a clonal marker, it is not possible to determine whether a particular therapy is potentially curative as opposed to merely palliative.…”

Transition of Myelofibrosis to Polycythaemia Vera

“…PV often develops increasing marrow fibrosis and myeloid metaplasia, which have been c,onsidered to be terminal events in all patients, if they live long enough (Wasserman 1954). The reverse transformation from established M F to PV seems to be a rare event, well documented in only 7 cases (Robson 1953, Lopas & Josephson 1964, Hersko & Polliack 1971, Soderstrom et al 1975, Parmentier et al 1977, Pettit et al 1978. Valid conclusions on the nature and the spontaneous occurrence of this transition may be difficult.…”

“…Thus 3 patients received splenic irradiation (Parmentier et al 1977, Pettit et a1 1978, and in 1 patient, splenectomy was performed prior to the polycythaemic development (Soderstrom et a1 1975). Treatment with alkylating agents might also have influenced the transition from MF to PV (Lopas & Josephson 1964, Pettit et a1 1978. Accordingly, Ward & Block (1971) suggested that a transformation from MF to PV does not occur naturally, and that the cases thus reported might represent an unmasking of preexistent PV, confounded by gastrointestinal bleeding or might represent an effect of myelosuppressive therapy.…”