Systemicmastocytosis (SM) is a heterogeneous disease of the bone marrow characterized by abnormal growth,accumulation and activation ofclonal mastcells (MCs). We reporta case of SM with multi-organ involvement. A 30-year-old man presented with diarrhea, flushing, maculopapular rashwithitching and weightloss. The upper and lower gastrointestinal endoscopies showed macro scopic involvement of stomac hand duodenum; mucosal samples fromstomach, duodenum, colon and distal ileum showed mucosal infiltration by large, spindle-shaped MCs with abnormal surface molecule expression (CD2 and CD25), a picture fully consistent with SM, according to the World Health Organization diagnostic criteria. A computed to mography scan showed diffuse lymphadenopathy, hepatosplenomegaly and diffuse small bowel involvement. Bone marrow aspirate and biopsy were diagnosticfor SM; serumtryptase levels were increased (209 ng/mL, normal values < 20 ng/mL). The conclusive diagnosis was smouldering SM. There were no therapeuticindications except for treatment of symptoms. The patient was strictly followed up because of the risk of aggressive evolution.
Severe chronic diarrhea and maculopapular rash : a case report / A. Elvevi, F. Grifoni, F. Branchi, U. Gianelli, D. Conte. - In: WORLD JOURNAL OF GASTROENTEROLOGY. - ISSN 1007-9327. - 17:34(2011 Sep 14), pp. 3948-3952.
|Titolo:||Severe chronic diarrhea and maculopapular rash : a case report|
ELVEVI, ALESSANDRA (Primo)
GRIFONI, FEDERICA (Secondo)
GIANELLI, UMBERTO (Penultimo)
CONTE, DARIO (Ultimo)
|Parole Chiave:||chronic diarrhea ; maculopapular rash|
|Settore Scientifico Disciplinare:||Settore MED/12 - Gastroenterologia|
|Data di pubblicazione:||14-set-2011|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.3748/wjg.v17.i34.3948|
|Appare nelle tipologie:||01 - Articolo su periodico|