Analysis Of Prognostic Risk Factors In 172 Patients With Primary Treated Rosai-Dorfman Disease In Respect To Relapse/Progression

Background:Rosai-Dorfman disease,also named as sinus histiocytosis with massive lymphadenopathy(SHML),is a rare histiocytosis characterized by massive bilateral painless cervical lymphadenopathy.Pathological and immunohistochemical examination remains as the gold standard for the diagnosis of this rare disorder.To date,Rosai-Dorfman disease are sporadically reported in the literature.Its pathogenesis is still unknown.Consensus has not been reached on its optimal management around treatment and follow-up.Objective:To describe the clinical characteristics and risk factors relevant with relapse or progression in primary treated Rosai-Dorfman disease.Methods:We reviewed EMBASE-,MEDLINE-,Cochrane-,Ovid-and Pub Med-based to find out all cases of RDD described between 1973 and 2003.Language was restricted to English.Issues of age,gender,clinical presentation,labarotory exzamination,treatment and follow-up are required for each study.Chi-square test and Logistic regression model are used to screen relapse/progression risk factors for primary treated RosaiDorfman disease.Results:1.After primary treatment,patients with anemia at the onset of disease usually suffer a higher relapse/progression risk than those with normal hemoglobin(c2=5.749,P=0.019).As is with those with hyperimmunoglobulinemia(c2 =9.856,P= 0.003).2.Logistic regression analysis reveal that anemia(P=0.028,OR=2.332,95%CI : 1.096-4.961)and hyperimmunoglobulinemia(P=0.002,OR=3.279,95%CI:1.525-7.050)are two independent risk factors of relapse/progression for primary treated patients.3.According to a novel prognostic scoring system established for primary treated Rosai-Dorfman disease to indicate the relapse/progression risk,patients could be divided into two groups,namely: the low risk group and the high risk group(c2=10.500,P=0.003).4.As for extranodal cutaneous Rosai-Dorfman disease,patients with more than one extranodal organ involvment have a higher relapse/progression risk in comparison with the purely cutaneous RDD patients(c2=8.669,P=0.012).In terms of extranodal patients with central nervous system complication,the involvement of other extranodal organs does not exert influence on relapse/progression(c2=3.068,P=0.154).Relapse/progression events do not differ significantly between the purely cutaneous RDD patients and those with exclusive central nervous system involvement(c2=0.048,P=1.000).Conlcusions:1.Anemia and hyperimmunoglobulinemia are the independent risk factors for predicting relapse/progression in primary treated RosaiDorfman disease.2.Primary treated RDD patients with coexistence of anemia and hyperimmunoglobulinemia usually suffer a higher risk of relapse/progression.3.Extranodal cutaneous RDD patients with multiple organ involvement usually have a remarkable propensity for relapse/progression compared with purely cutaneous patients.However,this correlation is not applicable to patients with central nervous system involvement.There is no remarkable difference between purely cutaneous patients and those with isolated focus in central nervous system in respect to relapse/progression risk after primary treatment.