72例儿童急性再生障碍性贫血临床分析

发布时间：2018-06-24 09:12

本文选题：儿童 + 急性再生障碍性贫血　；参考：《郑州大学》2012年硕士论文

【摘要】：目的探讨儿童急性再生障碍性贫血病因、临床特点、实验室检查及不同治疗方法的临床疗效。方法回顾性分析1996年1月-2010年11月在郑州大学第一附属医院儿内科住院、行骨髓穿刺术和(或)骨髓活检术确诊的72例急性再障患儿的资料,收集所有患儿的一般情况(年龄、性别等)、临床资料(起病原因、临床表现等)、实验室检查(外周血象、骨髓细胞学检查、骨髓活检、免疫学指标)等,根据不同的治疗方案,分组为1996年1月-2004年12月：Ⅰ组[大剂量甲基强的松龙(HDMP)方案治疗组]10例、2005年1月-2010年11月：Ⅱ组[环孢菌素A(CSA)+雄激素方案治疗组]54例和Ⅲ组(CSA+ATG方案治疗组)8例。按中华医学会儿科分会血液学组.小儿再生障碍性贫血的诊疗建议的疗效评定标准,比较3组间总有效率的差异。结果 1.一般情况：72例急性再障患儿,男：女比例为1.18：1,平均年龄(10.75±3.26)岁,其中5～10岁占44.4%,68.1%的患儿居住在农村。 2.起病原因：72例急性再障患儿,有化学、物理因素接触史者占6.9%,其中与农药有接触史者占12.5%,与苯及其衍生物等有接触史占者8.3%,有放射线接触史者占1.39%；其中农村患儿占75%(12例),城市患儿占25%(4例)有药物接触史者占2.8%,其中氯霉素占2.8%驱虫药物占4.2%噻嗪类利尿剂占1.4%；其中城市患儿占66.7%(10例),农村患儿占33.3%(5例)。有病毒感染者占36.1%,其中43.1%的患儿感染CBVIgM;城市患儿占60.6%(40例),农村患儿占39.4%(6例)。 3.临床症状：贫血者占70.8%,其中以面色苍白表现者占76.2%；出血者占68.1%,其中皮肤出血者占75.5%,其余部位出血者(如鼻出血者、牙龈出血等)占小部分；感染者占59.4%,其中呼吸道感染者占87.8%,败血症占4.2%,肠道感染占2.78%,牙龈炎占1.39%,皮肤蜂窝组织炎占1.39%。出血伴贫血者占54.2%；感染伴有贫血者占6.94%(5例)；感染伴出血者占8.33%；出血、贫血及感染者占13.9%。 4.体格检查：不同程度贫血貌的患儿占70.8%,有不同程度面色苍白及口唇、甲床苍白,其中重度贫血貌者占90.2%；淋巴结肿大者占45.8%；肝脏肿大者占40.3%；心脏可闻及杂音的患儿占2.8%。 5.辅助检查： (1)血常规检查结果示：外周血一系减低者占12.5%,两系减低者占30.6%,三系减低者占66.7%,其中L计数平均(3.72±2.18)×109L-1,L百分比平均(72±18)%；Ret计数平均(19±8)×109L-1,Ret百分比平均(0.30±0.20)%；MCV在正常范围者占75%,RDW在正常范围者占93.1%,MCV正常、RDW增高的患儿占75%,MCH在正常范围者占77.8%,MCHC在正常范围者占80.6%。 (2)骨髓细胞学检查结果示：骨髓增生低下者占72.2%,粒红系比值减低或倒置者占88.9%,淋巴细胞比值可高达80%～90%,87.5%的患儿可见骨髓小粒,其中非造血组织50%,未见巨核细胞者占58.3%。 (3)骨髓活检结果示：骨髓增生减低者占83.3%,脂肪组织增生活跃者占91.7%,粒系增生减低者占83.3%,红系增生减低者占91.7%,未见巨核系增生活跃； (4)免疫学指标示：治疗前检测33例患儿T淋巴细胞亚群示CD3+T、NKT、 CD4+T细胞比例降低,CD8+T细胞比例升高,CD4+/CD8+比值下降或倒置；免疫抑制治疗随访6个月复查结果示CD4+T细胞比例、CD4+/CD8+比值升高；CD8+T细胞比例降低；CD3+T、NKT细胞比例与治疗前无明显变化；其中15例患儿的CD4+T细胞比例、CD4+/CD8+比值、CD8+T细胞比例未恢复到正常范围内,继续给予免疫抑制治疗6个月后仅有4例未恢复到正常范围。 6.治疗：Ⅰ组总有效率高于Ⅱ组(X2=5.806,P0.05)；Ⅲ组总有效率高于Ⅱ组(X2=6.394,P0.05)；Ⅲ组总有效率高于Ⅰ组(X2=6.102,P0.05) 结论 1.急性再障以5-10岁儿童多见,男孩多于女孩。城市患儿因某些药物服用及病毒感染多见,农村患儿因与农药等化学因素接触多见。 2.急性再障外周血红细胞以MCV正常、MCH正常、MCHC正常、RDW升高多见即正细胞正色素非均一性贫血为主。 3.急性再障患儿存在T淋巴细胞亚群功能异常,免疫抑制治疗可使其恢复。 4. CSA+ATG联合方案治疗儿童急性再障的疗效最佳。
[Abstract]:objective
Objective to investigate the etiology, clinical characteristics, laboratory examination and clinical efficacy of different treatment methods in children with acute aplastic anemia.
Method
A retrospective analysis was made of 72 cases of acute aplastic anemia in the First Affiliated Hospital of Zhengzhou University, January 1996 -2010, which were hospitalized in the First Affiliated Hospital of Zhengzhou University. The data of children with acute aplastic anemia diagnosed by bone marrow aspiration and / or bone marrow biopsy were collected, and the general conditions (age, sex, etc.), clinical data (cause of disease, clinical manifestation, etc.), and laboratory examination (peripheral blood, bone, bone, bone, and bone) were collected. Myeloid cytology, bone marrow biopsy, immunological index, etc., according to different treatments, were divided into group I (HDMP) group]10 in group I [large dose methylprednisolone (HDMP), November: group]54 in group II (cyclosporin A (CSA) + androgen in group II and group III (CSA+ATG regimen group) 8 cases). According to the hematology section of the pediatrics branch of the Chinese Medical Association, the curative effect evaluation criteria for the diagnosis and treatment of aplastic anemia in children were compared. The difference of the total effective rate between the 3 groups was compared.
Result
1. general situation: 72 cases of acute aplastic anemia, male: the female ratio is 1.18:1, the average age (10.75 + 3.26) years old, 5~10 year old 44.4%, 68.1% children live in the countryside.
2. reasons for the onset of disease: 72 cases of acute aplastic anemia, chemical and physical contact history accounted for 6.9%, of which 12.5% were exposed to pesticides, 8.3% with benzene and its derivatives, 1.39% with radiation exposure history, 75% (12 cases) in rural children, 25% (4 cases) in city children (4 cases), and chlorine in 2.8%. Mycophentin accounted for 1.4% of 4.2% thiazine diuretics, accounting for 66.7% (10 cases), 33.3% (5) in rural children and 36.1% in rural children, of which 43.1% of the children were infected with CBVIgM, 60.6% (40) in urban children and 39.4% in rural areas (6 cases).
3. clinical symptoms: anemia accounted for 70.8%, of which 76.2% were pallid, 68.1% of the bleeding, 75.5% of the skin bleeding, and the rest of the bleeding (such as nosebleed, gingival bleeding) accounted for a small portion; the infected persons accounted for 59.4%, the respiratory tract infection accounted for 87.8%, septicemia accounted for 4.2%, intestinal infection 2.78%, gingivitis in 1.39% Cutaneous cellulitis accounted for 54.2% of 1.39%. bleeding accompanied by anemia, 6.94% (5 cases) with anemia and 8.33% with bleeding, hemorrhage, anemia and infection in 13.9%..
4. physical examination: children with different degrees of anemia accounted for 70.8%, with varying degrees of paleness and lip, a bed paleness, with severe anemia in 90.2%, lymph node enlargement in 45.8%, liver enlargement in 40.3%, and 2.8%. in children with heart smelling and murmur.
5. auxiliary examination:
(1) the results of blood routine examination showed that one of the peripheral blood was 12.5%, the two lines were 30.6%, and the three system was 66.7%, of which the L count was (3.72 + 2.18) * 109L-1, the percentage of L was (72 + 18)%, the average of the Ret count (19 +) 109L-1, the average of Ret (0.30 + 0.20)%, the MCV in the normal range, and the RDW in the normal range. 1%, MCV was normal, RDW increased in 75%, MCH in the normal range accounted for 77.8%, MCHC in normal range accounted for 80.6%.
(2) the results of bone marrow cytology showed that the number of myelodysplastic patients was 72.2%, the ratio of the erythroid system decreased or the inversion was 88.9%, the ratio of lymphocyte was as high as 80% to 90%, and 87.5% of the children had bone marrow particles, among which the non hematopoietic tissue was 50%, and no megakaryocyte was found in 58.3%..
(3) the results of bone marrow biopsy showed that 83.3% of those with hypoplastic myelodysplasia, 91.7% in adipose tissue, 83.3% in granulocytic hypoplasia and 91.7% in erythroid hyperplasia, no megakaryocyplasia was not active.
(4) immunology indicated that before treatment, 33 cases of T lymphocyte subsets showed that the proportion of CD3+T, NKT, CD4+T cells decreased, the proportion of CD8+T cells increased, the ratio of CD4+/CD8+ decreased or inverted; the ratio of CD4+T cells, CD4+/CD8+ ratio increased, CD8+T cell ratio decreased, CD3+T, NKT cell ratio was decreased in the follow-up of immunosuppressive therapy for 6 months. The proportion of CD4+T cells, the ratio of CD4+/CD8+, and the proportion of CD8+T cells in 15 cases were not recovered to the normal range, and only 4 cases were not recovered to the normal range after 6 months of immunosuppressive therapy.
6. treatment: the total effective rate of group I was higher than that of group II (X2=5.806, P0.05), and the total effective rate in group III was higher than group II (X2=6.394, P0.05), and the total effective rate in group III was higher than group I (X2=6.102, P0.05).
conclusion
1. children with acute aplastic anemia are more common in 5-10 years of age, more boys than girls. Children in urban areas are often seen because of some drugs and virus infection, and children in rural areas are exposed to chemical factors such as pesticides.
2. the peripheral blood red blood cells of acute aplastic anemia were normal in MCV, normal in MCH, normal in MCHC and increased in RDW.
3. there are abnormal T lymphocyte subsets in children with acute aplastic anemia, and immunosuppressive therapy can restore them.
4. CSA+ATG combined regimen is the best in the treatment of acute aplastic anemia in children.
【学位授予单位】：郑州大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R725.5

【参考文献】