再生障碍性贫血

Aplastic Anemia

再生障碍性贫血

Definition

说明

Aplastic anemia is a syndrome of bone-marrow failure characterized by peripheral pancytopenia and marrow hypoplasia.

再生障碍性贫血是骨髓造血功能衰竭所引发的一种综合症，其特征是周边各类血细胞减少、骨髓再生不良。

Pathophysiology

病理生理学

Aplastic anemia can be acquired or inherited. Acquired forms can be caused by drugs (chloramphenicol), chemicals (benzene), radiation, or viral infection (hepatitis, Epstein-Barr) and, in rare instances, are associated with paroxysmal nocturnal hemoglobinuria. Fanconi's anemia is the most common inherited type. Prognosis is grave. Fifty percent of patients die within 6 months of diagnosis.

再障分获得性和遗传性（先天)两种。获得性再障成因包括药物（氯霉素)、化学物质（苯)、辐射、病毒感染（肝炎、EB病毒)，偶尔也与阵发性夜间血红蛋白尿有关。范可尼贫血是最常见的遗传性贫血，预后极差，50%的病人在确诊后6个月内死亡。

Aplastic anemia may occur at any age. Fifty percent of cases are idiopathic. Long-term survival rate with bone marrow transplant (BMT) from histocompatible donors is as high as 70% to 90% in children.

任何年龄都可发生再障。50%属于原发性，儿童骨髓移植后长期存活率可达70%－90%。

Clinical Manifestations

临床表现

The manifestation of symptoms is dependent on the extent of the thrombocytopenia (hemorrhagic symptoms), neutropenia (bacterial infections, fever), and anemia (pallor, fatigue, congestive heart failure, tachycardia). Severe aplastic anemia is characterized by a granulocyte count of less than 500 per mm3, a platelet count of less than 20,000 per mm3, and a reticulocyte count of less than 1.

症状表现取决于血小板减少（出血症状)、中性白细胞减少（细菌感染、发烧)和贫血（苍白、疲劳、充血性心力衰竭、心悸亢进)等的程度。重度再障的特征是粒细胞计数低于500/ mm3，血小板计数低于20000/ mm3，网织红细胞计数低于1。

Clinical manifestations include petechiae, ecchymoses, epistaxis (occur first); oral ulcerations, bacterial infections, fever (occur later in course); anemia, pallor, fatigue, tachycardia (late signs); and cafe au lait spots, melanin-like hyperpigmentation, absent thumbs (Fanconi's anemia).

临床表现包括：瘀点、瘀斑、鼻衄（先发生)、口腔溃疡、细菌性感染、发烧（后期发生)、贫血、苍白、疲劳、心悸亢进（晚期症状)及咖啡牛乳色斑、黑色素样色素沉着、拇指缺如（范可尼贫血)。

Diagnostic Studies

诊断检查

Complete blood count with differential—macrocytic anemia; decreased granulocytes, monocytes, and lymphocytes

全血细胞计数鉴别－大红细胞性贫血，粒细胞、单核细胞和淋巴细胞等减少

Platelet count--decreased

血小板计数－减少

Reticulocyte count--decreased

网织红细胞－减少

Bone marrow aspiration and biopsy--hypocellular

骨髓穿刺与活检－细胞减少

Hemoglobin electrophoresis---elevated fetal hemoglobin level

血红素电泳－胎儿血红蛋白增多

Red cell i antigen titer--elevated

红细胞I血型抗原滴定－升高

Sugar water test--positive

糖水测试－阳性

Ham's test--positive

哈姆氏试验－阳性

Serum folate and B12 levels--normal or elevated

血清叶酸盐和B12测试－范可尼贫血呈阳性

Chromosome breakage test--positive for Fanconi's anemia

染色体断裂试验－范可尼贫血呈阳性

Medical Management

治疗

The first-choice treatment for aplastic anemia is bone marrow transplant with a sibling donor who is human lymphocyte antigens (HLA) matched. In more than 70% of cases there will be no sibling match. However, there is an increased chance that there will be a match between one parent and the child with aplastic anemia. If bone marrow transplant is to be done, HLA typing of the family is done immediately and blood products are used as little as possible to avoid sensitization. Also to avoid sensitization, blood should not be donated by the child's family. Blood products should always be irradiated and filtered to remove white blood cells before being given to a child who is a candidate for bone marrow transplant.

再障的首选治疗是人淋巴细胞抗原相配的同胞姊妹骨髓移植。但有70%的 病例缺少这种相配。不过，父母一方与再障小孩相配的机会较多。如果进行骨髓移植，就要马上做人淋巴细胞抗原分型，血液制品也是用得越少越好，以免致敏。同 时，为了避免致敏，也不要采用小孩家人所捐的血液。在给骨髓移植患儿输血前，始终要对血液制品进行辐射滤过，除去白血球。

Immunotherapy with either antithymocyte globulin (ATG) or antilymphocyte globulin (ALG) is the primary treatment for those children not candidates for bone marrow transplant. The child will respond within 3 months or not at all to this therapy. Cyclosporine A is also an effective immunosuppresant that can be used in the treatment of aplastic anemia. Androgens are rarely used unless no other treatment is available.

采用抗胸腺细胞球蛋白（ATG)或抗淋巴细胞球蛋白（ALG)免疫疗法是不适宜于骨髓移植患儿的主要治疗方法。患儿可在3个月内产生效果，也可能无效。环胞素A也是一种有效的免疫抑制剂，可用于治疗再障。雄激素帅很少用，除非迫不得已。

Supportive therapy includes use of antibiotics and administration of blood products. Antibiotics are used to treat fever and neutropenia; prophylactic antibiotics are not indicated for the asymptomatic child. Blood product administration may include the following:

支持疗法包括使用抗生素和血液制品。抗生素用于治疗发烧和中性粒细胞减少症；无症状儿童无需使用预防性抗生素。所用的血液制品有：

1. Platelets--to maintain platelet count greater than 20,000 per mm3. Use single-donor platelet pheresis to decrease number of human lymphocyte antigens to which the child is exposed.

血小板－使血小板计数维持在20000/ mm3以上。可采用单人血小板提取法减少人淋巴细胞抗原数量。

2. Packed red blood cells--to maintain hemoglobin (Hb) level higher than g/dl (chronic anemia is often well tolerated). For long-term therapy, use deferoxamine as chelating agent to prevent complications of iron overload.

浓集红细胞－使血红素维持在g/dl上（慢性贫血耐受好)。长期治疗时可用去铁胺作螯合剂，防止铁超负荷。

3. Granulocytes--to transfuse to patient who has gram negative sepsis.

粒性白细胞－革兰氏阴性脓毒症病人使用。

Nursing Management

护理

Goals

目标

Child will have gradual increase in red blood cells, white blood cells, and eventually platelets.医学.全在线www.med126.com

患儿红血球、白血球及血小板逐渐增多。

Child will have fewer infections.

患儿感染减少

Child will have minimal bleeding episodes.

患儿出血减少到最低限度

Child and family understand home care and follow-up needs.

患儿及家人了解家族护理及随访

Nursing Diagnoses

护理诊断

Risk for impaired oral mucous membrane related to tissue hypoxia and vulnerability

有口腔粘膜受损危险　与组织缺氧和易损有关

Risk for infection related to increased susceptibility secondary to leukopenia

有感染危险　与继发于白血球减少症的敏感度增加有关

Activity intolerance related to insufficient oxygen secondary to diminished red blood cell count

活动无耐力　与继发于红细胞减少计数下降的氧气不足有关

Risk for ineffective therapeutic regimen management related to insufficient knowledge of causes, prevention, and signs and symptoms of complications

有治疗无效危险　与并发症病因、预防及症状知识不足有关

Nursing Interventions

护理措施

Identify and report signs and symptoms of hemorrhage.

识别并报告出血症状

Vital signs (increased apical pulse, thready pulse, decreased blood pressure)

生命体征（心尖搏动加快、丝状脉、血压下降)

Bleeding sites

出血部位

Skin color (pallor) and signs of diaphoresis

肤色（苍白)和出汗

Weakness

无力

Decreased level of consciousness

意识水平下降

Decreased platelet count

血小板计数减少

Protect from trauma.

防止外伤

Do not administer aspirin or nonsteroidal antiinflammatory drugs (NSAIDs).

　　禁用阿斯匹林或非甾体类抗炎药

Avoid use of intramuscular (IM) injection and suppositories.

　　避免使用肌内注射及栓剂

Administer contraceptive to decrease excessive menstruation.

　　服用避孕药以减少过度行经

Provide good oral hygiene with soft toothbrush.

　　用软牙刷做好口腔卫生

Protect from infection.

防止感染

Limit contact with potential source of infection.

　　限制与潜在感染源的接触

Use strict isolation precautions (refer to institution's policies and procedures).

实施严格隔离（参照医院相关规定及程序)

Administer blood products and monitor child's response to their infusion.

输血，监护患儿的输血反应

Observe for side effects or untoward response (transfusion reaction).

　　观察不利反应所引起的副作用（输血反应)

Observe for signs of fluid overload.

　　观察液体过剩症状

Monitor vital signs before infusion; monitor every 15 minutes during first hour and then hourly during infusion.

　　输血前应监护生命征，输血第一小时每15分观察一次，随后每一小时观察一次

Provide frequent rest periods. Organize nursing care to increase activity tolerance and prevent fatigue.

经常休息。组织好护理活动，增加其活动耐力，防止疲劳

Monitor child's therapeutic and untoward response to medications; monitor the action and side effects of administered medications.

监视患儿对药物的治疗反应和不利反应，观察药物的作用及副作用。

Prepare child and family for bone marrow transplant.

为患儿及家人作好骨髓移植准备

Monitor for signs of bone marrow transplant complications.

监视骨髓移植并发症症状

Provide age-appropriate diversional and recreational activities.

根据患者年龄，分散其注意力，提供适当的娱乐活动

Provide age-appropriate explanation before procedures.

在手术前，对患者作适当解释

Patient Teaching

病人宣教

Instruct parents about measures to protect child from infection.

教育父母，使其了解小孩感染预防措施

Limit contact with infectious agents.

　　限制接触感染源

Identify signs and symptoms of infection.

　　识别感染症状

Instruct parents to monitor for signs of complications.

指导父母监视并发症症状

Instruct parents about the administration of medication.

指导父母如何给药

Monitor child's therapeutic response.

　　监视患儿治疗反应

Monitor for untoward responses.

　　监视不利反应

Provide child and family with information about community support systems for long-term adaptation.

身患儿及家人提供资讯，使其了解社区支持系统，适应长期护理需要

School reintegration

　　重新做好上学安排

Parent groups

　　安排家长小组活动

Child and sibling groups

　　安排患儿及姐妹活动

Financial advice

　　提出经济建议