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[Myeloid pseudo-splenomegaly in a patient with a myeloma].

Presse Med

November 2002

Service d'hématologie clinique, Hôpital d'instruction des armées Percy, Clamart, France.

Introduction: Lymphoproliferative syndromes are rarely complicated by medullar fibrosis simulating myeloid splenomegaly.

Observation: We report an unusual case of an IgD myeloma revealed in a context of myeloid splenomegaly in a 37 year-old man, initially admitted for severe anaemia associated with a voluminous splenomegaly.

Commentaries: The occurrence of myeloid splenomegaly during myeloma is extremely rare and only 14 cases have been reported.

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Risks and benefits of splenectomy in myelofibrosis: an analysis of 39 cases.

Nouv Rev Fr Hematol (1978)

October 1994

Department of Visceral Surgery, Hôpital Saint-Louis, Paris, France.

From 1980 to 1993, 39 splenectomies were performed in the Department of Visceral Surgery of Saint-Louis Hospital, in patients referred for myelofibrosis associated with myeloid splenomegaly. The short term morbidity was considerable: 33 serious haemorrhagic, infectious or thrombotic complications including 5 fatal accidents were observed in 18 patients. Severe thrombotic or infectious complications leading to 6 further deaths occurred in 8 patients over the two years following splenectomy, while six cases of acute leukaemia appeared between 6 months and 3 years after splenectomy.

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Clinical and laboratory assessment and therapeutic problems in longstanding polycythaemia vera.

Nouv Rev Fr Hematol (1978)

April 1994

Département de Médecine Nucléaire, Hôpital Saint-Louis, Paris, France.

With the very long life expectancy of Polycythaemia Vera late complications are often observed: progressive resistance to treatment, bad tolerance to maintenance by phlebotomy, progression towards myelofibrosis. Resistance to phosphorus 32 is reflected by a progressive reduction in the duration of remission and by a gradually decreasing remission rate. A complete resistance appears after a mean duration of disease of 7 years.

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The very-long-term follow-up of patients initially included in the PVSG protocols provides useful information. The excess risk of cancer after chlorambucil appears to persist for 5 years after stopping this treatment. The risk of leukaemia induced by marrow suppression (32P or chemotherapy) was marked before the 10th year, but low thereafter.

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Indications, procedure and results for the treatment of polycythaemia vera by bleeding, pipobroman and hydroxyurea.

Nouv Rev Fr Hematol (1978)

February 1994

Service d'Hématologie Clinique, Hôpital Beaujon, Clichy, France.

The present report is based on an analysis of the evolution of 720 cases of Polycythaemia vera treated with pipobroman and 624 cases treated with hydroxyurea. General modes of treatment are identical for the two drugs, consisting of initial therapy at relatively high dose aimed at obtaining complete remission and maintenance therapy essential to conserve the improved clinical status. Both types of treatment must be adapted to suit the patient.

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