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Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases
Author(s) -
Boutboul David,
Fadlallah Jehane,
Chawki Sylvain,
Fieschi Claire,
Malphettes Marion,
Dossier Antoine,
Gérard Laurence,
Mordant Pierre,
Meignin Véronique,
Oksenhendler Eric,
Galicier Lionel
Publication year - 2019
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.15921
Subject(s) - medicine , asymptomatic , surgery , disease , retrospective cohort study , castleman disease , radiation therapy , regimen
Summary We retrospectively analysed 71 cases of Unicentric Castleman disease, a rare, usually asymptomatic, benign lymphoproliferative disorder presenting as a unique nodal mass. Although surgery is considered as the gold standard therapy, only 38 patients (54%) underwent initial surgical resection and 95% were cured. An additional 9 patients had surgery after an attempt at medical reduction. Reduction therapy was used in 21 patients with a 55% response rate, but without evidence for an optimal regimen. Radiotherapy was limited to 8 patients because of associated toxicity. Watch and wait was considered in 13 asymptomatic patients and 11 of these remained stable for up to 17 years.