再生障礙性貧血

Aplastic Anemia

再生障礙性貧血

Definition

說(shuō)明

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

再生障礙性貧血是骨髓造血功能衰竭所引發(fā)的一種綜合癥，其特征是周邊各類(lèi)血細(xì)胞減少、骨髓再生不良。

Pathophysiology

病理生理學(xué)

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.

再障分獲得性和遺傳性（先天)兩種。獲得性再障成因包括藥物（氯霉素)、化學(xué)物質(zhì)（苯)、輻射、病毒感染（肝炎、EB病毒)，偶爾也與陣發(fā)性夜間血紅蛋白尿有關(guān)。范可尼貧血是最常見(jiàn)的遺傳性貧血，預(yù)后極差，50%的病人在確診后6個(gè)月內(nèi)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.

任何年齡都可發(fā)生再障。50%屬于原發(fā)性，兒童骨髓移植后長(zhǎng)期存活率可達(dá)70%－90%。

Clinical Manifestations

臨床表現(xiàn)

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.

癥狀表現(xiàn)取決于血小板減少（出血癥狀)、中性白細(xì)胞減少（細(xì)菌感染、發(fā)燒)和貧血（蒼白、疲勞、充血性心力衰竭、心悸亢進(jìn))等的程度。重度再障的特征是粒細(xì)胞計(jì)數(shù)低于500/ mm3，血小板計(jì)數(shù)低于20000/ mm3，網(wǎng)織紅細(xì)胞計(jì)數(shù)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).

臨床表現(xiàn)包括：瘀點(diǎn)、瘀斑、鼻衄（先發(fā)生)、口腔潰瘍、細(xì)菌性感染、發(fā)燒（后期發(fā)生)、貧血、蒼白、疲勞、心悸亢進(jìn)（晚期癥狀)及咖啡牛乳色斑、黑色素樣色素沉著、拇指缺如（范可尼貧血)。

Diagnostic Studies

診斷檢查

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

全血細(xì)胞計(jì)數(shù)鑒別－大紅細(xì)胞性貧血，粒細(xì)胞、單核細(xì)胞和淋巴細(xì)胞等減少

Platelet count--decreased

血小板計(jì)數(shù)－減少

Reticulocyte count--decreased

網(wǎng)織紅細(xì)胞－減少

Bone marrow aspiration and biopsy--hypocellular

骨髓穿刺與活檢－細(xì)胞減少

Hemoglobin electrophoresis---elevated fetal hemoglobin level

血紅素電泳－胎兒血紅蛋白增多

Red cell i antigen titer--elevated

紅細(xì)胞I血型抗原滴定－升高

Sugar water test--positive

糖水測(cè)試－陽(yáng)性

Ham's test--positive

哈姆氏試驗(yàn)－陽(yáng)性

Serum folate and B12 levels--normal or elevated

血清葉酸鹽和B12測(cè)試－范可尼貧血呈陽(yáng)性

Chromosome breakage test--positive for Fanconi's anemia

染色體斷裂試驗(yàn)－范可尼貧血呈陽(yáng)性

Medical Management

治療

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.

再障的首選治療是人淋巴細(xì)胞抗原相配的同胞姊妹骨髓移植。但有70%的 病例缺少這種相配。不過(guò)，父母一方與再障小孩相配的機(jī)會(huì)較多。如果進(jìn)行骨髓移植，就要馬上做人淋巴細(xì)胞抗原分型，血液制品也是用得越少越好，以免致敏。同 時(shí)，為了避免致敏，也不要采用小孩家人所捐的血液。在給骨髓移植患兒輸血前，始終要對(duì)血液制品進(jìn)行輻射濾過(guò)，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.

采用抗胸腺細(xì)胞球蛋白（ATG)或抗淋巴細(xì)胞球蛋白（ALG)免疫療法是不適宜于骨髓移植患兒的主要治療方法�；純嚎稍�3個(gè)月內(nèi)產(chǎn)生效果，也可能無(wú)效。環(huán)胞素A也是一種有效的免疫抑制劑，可用于治療再障。雄激素帥很少用，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:

支持療法包括使用抗生素和血液制品�？股�素用于治療發(fā)燒和中性粒細(xì)胞減少癥；無(wú)癥狀兒童無(wú)需使用預(yù)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－使血小板計(jì)數(shù)維持在20000/ mm3以上。可采用單人血小板提取法減少人淋巴細(xì)胞抗原數(shù)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.

濃集紅細(xì)胞－使血紅素維持在g/dl上（慢性貧血耐受好)。長(zhǎng)期治療時(shí)可用去鐵胺作螯合劑，防止鐵超負(fù)荷。

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

粒性白細(xì)胞－革蘭氏陰性膿毒癥病人使用。

Nursing Management

護(hù)理

Goals

目標(biāo)

Child will have gradual increase in red blood cells, white blood cells, and eventually platelets.醫(yī)學(xué).全在線(xiàn)www.med126.com

患兒紅血球、白血球及血小板逐漸增多。

Child will have fewer infections.

患兒感染減少

Child will have minimal bleeding episodes.

患兒出血減少到最低限度

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

患兒及家人了解家族護(hù)理及隨訪(fǎng)

Nursing Diagnoses

護(hù)理診斷

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

有口腔粘膜受損危險(xiǎn)　與組織缺氧和易損有關(guān)

Risk for infection related to increased susceptibility secondary to leukopenia

有感染危險(xiǎn)　與繼發(fā)于白血球減少癥的敏感度增加有關(guān)

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

活動(dòng)無(wú)耐力　與繼發(fā)于紅細(xì)胞減少計(jì)數(shù)下降的氧氣不足有關(guān)

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

有治療無(wú)效危險(xiǎn)　與并發(fā)癥病因、預(yù)防及癥狀知識(shí)不足有關(guān)

Nursing Interventions

護(hù)理措施

Identify and report signs and symptoms of hemorrhage.

識(shí)別并報(bào)告出血癥狀

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

生命體征（心尖搏動(dòng)加快、絲狀脈、血壓下降)

Bleeding sites

出血部位

Skin color (pallor) and signs of diaphoresis

膚色（蒼白)和出汗

Weakness

無(wú)力

Decreased level of consciousness

意識(shí)水平下降

Decreased platelet count

血小板計(jì)數(shù)減少

Protect from trauma.

防止外傷

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

　　禁用阿斯匹林或非甾體類(lèi)抗炎藥

Avoid use of intramuscular (IM) injection and suppositories.

　　避免使用肌內(nèi)注射及栓劑

Administer contraceptive to decrease excessive menstruation.

　　服用避孕藥以減少過(guò)度行經(jīng)

Provide good oral hygiene with soft toothbrush.

　　用軟牙刷做好口腔衛(wèi)生

Protect from infection.

防止感染

Limit contact with potential source of infection.

　　限制與潛在感染源的接觸

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

實(shí)施嚴(yán)格隔離（參照醫(yī)院相關(guān)規(guī)定及程序)

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

輸血，監(jiān)護(hù)患兒的輸血反應(yīng)

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

　　觀(guān)察不利反應(yīng)所引起的副作用（輸血反應(yīng))

Observe for signs of fluid overload.

　　觀(guān)察液體過(guò)剩癥狀

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

　　輸血前應(yīng)監(jiān)護(hù)生命征，輸血第一小時(shí)每15分觀(guān)察一次，隨后每一小時(shí)觀(guān)察一次

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

經(jīng)常休息。組織好護(hù)理活動(dòng)，增加其活動(dòng)耐力，防止疲勞

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

監(jiān)視患兒對(duì)藥物的治療反應(yīng)和不利反應(yīng)，觀(guān)察藥物的作用及副作用。

Prepare child and family for bone marrow transplant.

為患兒及家人作好骨髓移植準(zhǔn)備

Monitor for signs of bone marrow transplant complications.

監(jiān)視骨髓移植并發(fā)癥癥狀

Provide age-appropriate diversional and recreational activities.

根據(jù)患者年齡，分散其注意力，提供適當(dāng)?shù)膴蕵?lè)活動(dòng)

Provide age-appropriate explanation before procedures.

在手術(shù)前，對(duì)患者作適當(dāng)解釋

Patient Teaching

病人宣教

Instruct parents about measures to protect child from infection.

教育父母，使其了解小孩感染預(yù)防措施

Limit contact with infectious agents.

　　限制接觸感染源

Identify signs and symptoms of infection.

　　識(shí)別感染癥狀

Instruct parents to monitor for signs of complications.

指導(dǎo)父母監(jiān)視并發(fā)癥癥狀

Instruct parents about the administration of medication.

指導(dǎo)父母如何給藥

Monitor child's therapeutic response.

　　監(jiān)視患兒治療反應(yīng)

Monitor for untoward responses.

　　監(jiān)視不利反應(yīng)

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

身患兒及家人提供資訊，使其了解社區(qū)支持系統(tǒng)，適應(yīng)長(zhǎng)期護(hù)理需要

School reintegration

　　重新做好上學(xué)安排

Parent groups

　　安排家長(zhǎng)小組活動(dòng)

Child and sibling groups

　　安排患兒及姐妹活動(dòng)

Financial advice

　　提出經(jīng)濟(jì)建議