BACKGROUND Rosai–Dorfman disease(RDD)is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents.The common manifestation of RDD is massive and painless bilateral cervical lymph...BACKGROUND Rosai–Dorfman disease(RDD)is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents.The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease.While extranodal involvement in RDD is common,the spleen is an infrequent site of disease.CASE SUMMARY We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy.She had fever,and blood tests showed leukocytosis,anemia,and elevated erythrocyte sedimentation rate and Creactive protein.Ultrasound,computed tomography,and magnetic resonance images demonstrated multiple splenic masses.Despite antibiotic therapy,her symptoms were not relived.She underwent diagnostic splenectomy and was discharged with recovery.CONCLUSION In pediatric patients with refractory infectious symptoms or hematological abnormalities,clinicians should suspect RDD,even in patients without significant lymphadenopathy.展开更多
窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实...窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实验室指标支持该病，且属于儿科少见病，在儿科临床极易误诊和漏诊，本文对其诊断及治疗方法等进行归纳总结，以提高对该疾病的认识。展开更多
文摘BACKGROUND Rosai–Dorfman disease(RDD)is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents.The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease.While extranodal involvement in RDD is common,the spleen is an infrequent site of disease.CASE SUMMARY We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy.She had fever,and blood tests showed leukocytosis,anemia,and elevated erythrocyte sedimentation rate and Creactive protein.Ultrasound,computed tomography,and magnetic resonance images demonstrated multiple splenic masses.Despite antibiotic therapy,her symptoms were not relived.She underwent diagnostic splenectomy and was discharged with recovery.CONCLUSION In pediatric patients with refractory infectious symptoms or hematological abnormalities,clinicians should suspect RDD,even in patients without significant lymphadenopathy.
文摘窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实验室指标支持该病，且属于儿科少见病，在儿科临床极易误诊和漏诊，本文对其诊断及治疗方法等进行归纳总结，以提高对该疾病的认识。
