新生儿MN溶血病一例并21例文献复习

目的 探讨新生儿MN溶血病的临床特点及诊治经验,更好地预防治疗本病.方法 报道新生儿MN溶血病1例,并对国内近20年来已报告的21例进行文献复习.结果 新生儿MN溶血病的报道有增多趋势.有4/22例发生于第1胎,男:女=10:8;患儿血型19/21例为MN、2/21例为M,其母血型10/21例为N、11/21例为NN;直接抗人球蛋白试验7/18例为阳性、4/18例为弱阳性、7/18例为阴性,抗体释放试验13/16例为阳性、3/16例为阴性,游离抗体试验17/17例为阳性;母抗体类型:21/21例IgG抗-M均(+),10/21例IgM抗M亦(+);患儿抗体类型:22/22例均为IgG抗M(+);患儿均有不同程度的贫血、黄疸;11/15例在出生24 h内出现黄疸;4/13例直接胆红素也明显增高;出现黄疸后,多数给予了光疗;4/15例给予了大剂量静脉注射免疫球蛋白;8/22例实施了换血治疗;死亡3/22例、治愈19/22例.结论 抗-M抗体引起的新生儿溶血病其病情轻重相差较大,重者甚至死胎或者需要换血.对于反复死胎、流产、胎儿水肿、严重贫血或多年不育的患者,在排除了其他原因及新生儿ABO、Rh溶血病后,应查MN血型及其抗体进一步明确本病.有效的光疗、效果显著的大剂量静脉注射免疫球蛋白、金属卟啉类药物等治疗,已经明显减少了需要换血的患儿数量.换血多采用全自动周围血管双管同步换血法.

Hemolytic disease of neonates due to anti-M: report of one case and review of reports of 21 cases

Objective To analyze and summarize clinical manifestation of hemolytic disease of the newborn (HDN) due to anti-M. Methods Data of one case of HDN due to anti-M and the reports of 21 cases seen in the past 20 years at the home country were reviewed and analyzed. Results There was an increasing number of reports of cases with HDN due to anti-M. Among the 22 cases, four were the first fetus. Of 18 infants, ten were male, and eight were female. The blood group was MN in 19/21 infants, and was M in 2/21 infants. The blood group was N in 10/21 mothers, and was NN in 11/21 mothers. Among the 18 infants, the direct antiglobulin test of 7 infants were positive, of 4 infants were dubiously positive, and of 7 infants was negative. Among the 16 infants, the antibody release test of 13 infants was positive, and of 3 infants were negative. Among 17 infants, the free antibody test of all was positive. Among the 21 mothers, the anti-M of IgG were positive in all mothers, and along with IgM in 11 mothers. The anti-M of IgG was positive in all infants. Mild or severe anemia and icterus were found in all cases. Among the 15 cases, jaundice was evident on the 1st day of life in 11 cases. Among 13 cases, marked elevation of both indirect-and direct-reacting bilirubin levels was reported in 4 cases. Phototherapy was applied when jaundice became evident. High-dose intravenous immunoglobulin was given to 4/15 cases. Exchange transfusion were performed in 8 of 22 cases. Three cases died, and 19 cases were cured. Conclusion HDN of varying degrees of severity has been reported in association with anti-M and can even lead to intrauterine deaths or requiting treatment with exchange transfusion. If the mother has a history of prior intrauterine deaths, abortion, hydrops fetalis, severe fetal anemia or infertile, MN blood group and anti-M antibodies should be tested after excluding the possibility of other causes and HDN due to ABO or Rh blood group incompatibility.As the efficacy of phototherapy increases, the role of exchange transfusion in acute management is rapidly decreasing. High-dose intravenous immunoglobulin and/or intramuscular metalloporphyrins may further reduce the need for exchange transfusion. The exchange transfusion may be performed through peripheral arterial (drawn out) and venous (infused in) lines.