Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 3948-3952 |
| Number of pages | 5 |
| Journal | World Journal of Gastroenterology |
| Volume | 17 |
| Issue number | 34 |
| DOIs | |
| Publication status | Published - Sep 14 2011 |
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Keywords
- Bone marrow
- Mastcells
- Systemicmastocytosis
- Tryptase
ASJC Scopus subject areas
- Gastroenterology
Cite this
Severe chronic diarrhea and maculopapular rash : A case report. / Elvevi, Alessandra; Branchi, Federica; Grifoni, Federica; Conte, Dario; Gianelli, Umberto.
In: World Journal of Gastroenterology, Vol. 17, No. 34, 14.09.2011, p. 3948-3952.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Severe chronic diarrhea and maculopapular rash
T2 - A case report
AU - Elvevi, Alessandra
AU - Branchi, Federica
AU - Grifoni, Federica
AU - Conte, Dario
AU - Gianelli, Umberto
PY - 2011/9/14
Y1 - 2011/9/14
N2 - 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.
AB - 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.
KW - Bone marrow
KW - Mastcells
KW - Systemicmastocytosis
KW - Tryptase
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UR - http://www.scopus.com/inward/citedby.url?scp=80054827517&partnerID=8YFLogxK
U2 - 10.3748/wjg.v17.i34.3948
DO - 10.3748/wjg.v17.i34.3948
M3 - Article
C2 - 22025884
AN - SCOPUS:80054827517
VL - 17
SP - 3948
EP - 3952
JO - World Journal of Gastroenterology
JF - World Journal of Gastroenterology
SN - 1007-9327
IS - 34
ER -