Johnson Susan T, McFarland Janice G, Kelly Kevin J, Casper James T, Gottschall Jerome L
Immunohematology Reference Laboratory, The Blood Center of Southeastern Wisconsin, Children's Hospital of Wisconsin, and the Medical College of Wisconsin, Milwaukee, Wisconsin 53201-2178, USA.
Transfusion. 2002 May;42(5):567-71. doi: 10.1046/j.1537-2995.2002.00093.x.
Autoimmune hemolytic anemia (AIHA) in children, although unusual, is often associated with recent infection. Several reports have identified the diphtheria-pertussis-tetanus (DPT) vaccination as a possible trigger for AIHA.
Life-threatening AIHA was diagnosed in a 6-week-old infant 5 days after receiving a DPT vaccination. The patient required daily transfusion and/or exchange transfusion for 3 weeks. RBCs from an Mk homozygote were found compatible with the patient's autoantibody. Transfusion of RBCs from an Mk homozygote and later RBCs from an individual (K.T.) with a variant glycophorin, Mi.VII, were required to sustain the patient's Hb level until autoantibody production ceased, as evidenced by a fall in antibody titer and the patient's Hct returning to normal.
The DAT was positive (3+) with only anti-C3 on presentation. An IgM cold reactive autoantibody with probable anti-Pr specificity and high thermal amplitude (37 degrees C) was identified in the serum. The DAT was no longer positive after transfusion with compatible blood.
This case represents life-threatening AIHA in an infant, temporally related to a DPT injection and responsive to a combination of immunosuppression and transfusion of rare compatible blood.
儿童自身免疫性溶血性贫血(AIHA)虽不常见,但常与近期感染有关。多篇报道指出白喉-百日咳-破伤风(DPT)疫苗接种可能是AIHA的诱发因素。
一名6周大婴儿在接种DPT疫苗5天后被诊断为危及生命的AIHA。该患者连续3周每天需要输血和/或换血治疗。发现来自Mk纯合子的红细胞与患者自身抗体相容。需要输注来自Mk纯合子的红细胞,随后输注来自具有变异血型糖蛋白Mi.VII的个体(K.T.)的红细胞,以维持患者的血红蛋白水平,直至抗体滴度下降且患者血细胞比容恢复正常,表明自身抗体产生停止。
入院时直接抗人球蛋白试验(DAT)呈阳性(3+),仅抗C3阳性。血清中鉴定出一种具有可能的抗-Pr特异性和高热幅度(37摄氏度)的IgM冷反应性自身抗体。输注相容血液后,DAT不再呈阳性。
该病例代表了一名婴儿危及生命的AIHA,在时间上与DPT注射有关,对免疫抑制和输注罕见的相容血液联合治疗有反应。