Scripties UMCG - Rijksuniversiteit Groningen
 
English | Nederlands

Syndroom van POEMS : Verslag van een zeldzame plasmacel dyscrasie in Nederland

(2016) Langbroek, P.

Summary
POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, sclerotic bone lesions, Castleman disease, elevated VEGF levels, organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis and polycythemia. In this retrospective study an overview is presented of the characteristics, treatment and course of patients diagnosed with POEMS syndrome in the Netherlands. 27 patients were included. The mean age was 51 years, 67% was male. The median time between onset of disease and diagnosis was 15 months (range 2-36 months). 74% presented with polyneuropathy as one of the initial symptoms. The most frequent characteristics besides a plasma cell dyscrasia and polyneuropathy (mandatory for diagnosis) were elevated VEGF levels (100%), skin changes (78%), endocrinopathy (71%) and sclerotic bone lesions (70%). The median follow-up time was 32 months. Lenalidomide/Dexamethasone therapy was used in 56% of patiënts and was effective in 73%. High-dose melphalan followed by an autologous stem cell re-infusion was the most frequent (65%) and most effective (100%) treatment. Eventually 65% of patient had a hematologic response, 100% a VEGF-response and 71% a neurologic response, while 88% of treatments were successfully completed. 1 patient died as a consequence of the disease. Treatment with corticosteroids alone appeared to be the only treatment with a negative effect on the hematologic response (p=0,04). The time to diagnosis, disease-characteristics and the other treatments were not associated with the clinical, haematologic, neurologic or VEGF-response. In conclusion, when diagnosis is finally made, treatment can be very effective. The proposed responses for disease follow-up seem to be lacking an association with therapy effectiveness.





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