Clinical study on the autoimmune hemolytic anemia and childhood-inset systemic lupus erythematosus

Abstract

Objective To summarize the characteristics of clinical features, laboratory tests and incidence of autoimmune hemolytic anemia(AIHA) in childhood-onset systemic lupus erythematosus (c-SLE). Methods A retrospective study including 89 children with c-SLE, among them, 9 cases of AIHA. AIHA was diagnosed by combination of the clinical manifestations and laboratory tests. Results AIHA was observed 10% (9/89) in c-SLE children. The differences of female gender, delay time, whether or not the first child, multiorgan involvement, autoantibodies profile, nephritis and its classification, low complement, EB-IgG, CMV-IgG and SLEDAI had no significant between c-SLE children with or without AIHA (P>0.05). c-SLE children with AIHA had a lower frequency of age at diagnosis(12 years old vs.14 years old, P=0.019), malar rash(11% vs. 59%, P=0.018), red blood cell[1.66(1.27-4.7)×1012/L vs. 3.82(1.71-5.38)×1012/L, P=0.007], hemoglobin[78(51-100)g/L vs. 107(57-146)g/L, P=0.001], lymphocyte count[2.0(1.2-2.6)×109/L vs. 1.3(0.2-5.5)×109/L, P=0.019] than c-SLE children without AIHA. Conclusions Our study identified AIHA as a rare and severe initial manifestation of c-SLE. Diagnosis is challenging due to the lack of typical symptoms(malar rash) of lupus and the requirement to exclude infection, malignancy and macrophage activation syndrome and so on. Therefore, it’s necessary to screening AIHA in all first-visit c-SLE children presenting a hypocytosis. Key words: Children; Onset systemic lupus erythematosus; Autoimmune hemolytic anemia