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Severe chronic diarrhea and maculopapular rash:A case report

Severe chronic diarrhea and maculopapular rash:A case report
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摘要 Systemic mastocytosis (SM) is a heterogeneous disease of the bone marrow characterized by abnormal growth, accumulation and activation of clonal mast cells (MCs). We report a case of SM with multiorgan involvement. A 30-year-old man presented with diarrhea, flushing, maculopapular rash with itching and weight loss. The upper and lower gastrointestinal endoscopies showed macroscopic involvement of stomach and duodenum; mucosal samples from stomach, duodenum, colon and distal ileum showed mucosal inf iltration by large, spindleshaped 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 tomography scan showed diffuse lymphadenopathy, hepatosplenomegaly and diffuse small bowel involvement. Bone marrow aspirate and biopsy were diagnostic for SM; serum tryptase levels were increased (209 ng/mL, normal values < 20 ng/mL). The conclusive diagnosis was smouldering SM. There wereno therapeutic indications except for treatment of symptoms. The patient was strictly followed up because of the risk of aggressive evolution. Systemic mastocytosis (SM) is a heterogeneous disease of the bone marrow characterized by abnormal growth, accumulation and activation of clonal mast cells (MCs). We report a case of SM with multiorgan involvement. A 30-year-old man presented with diarrhea, flushing, maculopapular rash with itching and weight loss. The upper and lower gastrointestinal endoscopies showed macroscopic involvement of stomach and duodenum; mucosal samples from stomach, duodenum, colon and distal ileum showed mucosal inf iltration by large, spindleshaped 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 tomography scan showed diffuse lymphadenopathy, hepatosplenomegaly and diffuse small bowel involvement. Bone marrow aspirate and biopsy were diagnostic for SM; serum tryptase levels were increased (209 ng/mL, normal values 〈 20 ng/mL). The conclusive diagnosis was smouldering SM. There wereno therapeutic indications except for treatment of symptoms. The patient was strictly followed up because of the risk of aggressive evolution.
出处 《World Journal of Gastroenterology》 SCIE CAS CSCD 2011年第34期3948-3952,共5页 世界胃肠病学杂志(英文版)
关键词 Mast cells Systemic mastocytosis Bone marrow Tryptase 慢性腹泻 丘疹 世界卫生组织 肥大细胞 异常生长 十二指肠 淋巴结肿大 计算机断层
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