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Pediatric‐onset mastocytosis: A long term clinical follow‐up and correlation with bone marrow histopathology
Author(s) -
Uzzaman Ashraf,
Maric Irina,
Noel Pierre,
Kettelhut Brett V.,
Metcalfe Dean D.,
Carter Melody C.
Publication year - 2009
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.22125
Subject(s) - medicine , systemic mastocytosis , disease , bone marrow , histopathology , systemic disease , pathology , dermatology
Background Pediatric onset mastocytosis usually presents as urticaria pigmentosa; and less often as diffuse cutaneous mastocytosis. While the literature indicates that disease often resolves, there has been a move to more aggressive therapy for mastocytosis early in life. We addressed the long term prognosis of pediatric‐onset disease by examining 17 children with mastocytosis which we had reported on in 1989 [1]. Procedure We successfully contacted 15 of these patients and data was collected regarding their clinical status. Original bone marrow specimens were re‐stained, re‐examined, and correlated with disease outcome using consensus criteria. Three of five patients with persistent disease underwent repeat bone marrow biopsies. Results There was complete regression of disease as defined by cutaneous findings and symptoms (clinical disease severity) in 10 of 15 patients (67%). Three patients had major (20%) and two had partial regression of disease (13%). Repeat marrow examinations on three patients with persistent disease documented systemic mastocytosis based on marrow findings in one patient who had partial regression of disease and was the only patient with initial morphologic evidence of systemic disease. Of the remaining two patients, one demonstrated partial regression and the other major regression of disease; and neither had evidence of systemic mastocytosis. Conclusion This study demonstrates that initial bone marrow biopsies were prognostic in that those without evidence of systemic disease experienced disease regression; and that the long term prognosis for children managed symptomatically with mastocytosis is highly encouraging. Pediatr Blood Cancer 2009;53:629–634. © 2009 Wiley‐Liss, Inc.