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Renal failure in pediatric Castleman disease: Four French cases with thrombotic microangiopathy
Author(s) -
Cousin Elie,
Flodrops Hugues,
Boyer Olivia,
Hogan Julien,
Ruin Mahe,
Couderc Anne,
Goujon JeanMichel,
Taque Sophie
Publication year - 2018
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.27045
Subject(s) - medicine , thrombotic microangiopathy , disease , pathology
Pediatric Castleman disease (CD) is an uncommon and poorly understood disorder of the lymph nodes. Renal failure has not been described in pediatric multicentric CD (MCD). We report four cases, who presented with polyadenopathy, organomegaly, edema and fluid accumulations, high blood pressure, and acute renal failure. In all cases, renal biopsy confirmed diffuse thrombotic microangiopathy. Definitive diagnosis of MCD was made by a biopsy of an affected lymph node located by computer tomography before initiation of corticosteroid therapy. Treatment of CD with corticosteroid therapy and rituximab was rapidly effective without relapse to date.