Aplast Anemia （再生障碍性贫血）

陈进伟

Aplastic Anemia( 再生障碍性贫血）

Aplastic Anemia is a pancytopenia （全血

细胞减少） syndrome appearing as anemia,

infection and bleeding by multiple causes

that result in decrease or failure of hem

opoietic ( 造血） function of BM.

Etiology and pathogenesis

Cholormycine division quantity and/or

1.Drugs Sedative (-) synthesis of protein (-) quality abnormality of

anti-cancer mRNA stem cells

(Beed theory; defective

stem cell)

2.Chemical toxic Beyene

Substance inorganic arsenic (-)

3.Virus infection (+) antibodies resisting stem cells

(EB;hepatitis) self-immunition (+) IFN↑ (-)

(insect theory; disorder immunine function)

4.Physical factors copy of DNA (-) regeneration of hematopoietic

(X-ray,r-ray) (-) cells

damage microenviroment (-)hypoproliferation or failure of BM

(soil theory; defect of hemopoietic microenviroment)

Clinic appearance and classificationAAA(SAA-I;SAA-II) CAA (light AA)

onset Acute sharp Gradualoutstanding

manifestation

Bleeding(viscera 内脏 )Infection (serious; septicemia 败血症 )

Slight Bleeding(skin; mucosa 粘膜 ) Anemia

Blood pictureReticulocyte BM

prognosis

N<0.5×109/LPt<20×109/L<15×109/LHypoproliferation in many parts;non-hemopoietic cell↑

BadDead in 6-12 month by hemorrhage in brain and/or infection

N>0.5×109/L Pt>20×109/L >15×109/L Hypoproliferation orhyperproliferation non-hemopoietic cell↑

chronic procedure

　 Standuard of diagnosis:１． Pancytopenia ; decreased of re

ticularcytic absolute value （网织ＲＢＣ绝对计数）

２． Usually unenlargement of liver and spleen

3. Bone marrow hypoproliferation（增生低下 ) of one site at least or hyperproliferation( 增生活跃） with megakaryocyte （巨核细胞） decreased

:

４． Increased of non-hematohistiololast （非造血组织细胞）

５． Being excepted other pancytopenia disease

６． Uneffect of usual anti-Aneamia treatment

Differential diagnosis1.Myelodysplastic syndrome(MDS;

骨髓增生异常综合症）　　 normoblast （幼稚细胞） in blood p

icture

Morbid hemapoietsis ( 病态造血） 2.paroxysmal nocturnal hemoglobinur

ia (PNH; 阵发性睡眠性血红蛋白尿） Ham’s test , Rouse test (+)

3.Malignant histiocytosis( 恶性组织细胞病） 　 jaundice （黄疸） ,and enlargement of lymphonodus （淋巴结） , liver and spleen.

　　 Abnormal histiocyte （异常组织细胞） in BM

4. Aleukemic leukemia Blast cells ( 原始细胞） can be found in peripheral blood and/or BM

Treatment Principle of treatment

1. got rid of possible pathogeny

2. Classified and united treatment. AAA: immunsuppressive + BMT +cell factor CAA: male hormone mainly. immunosuppressive agent .sos 3. Early diagnosis,early treatment.

4.supportive therapy1) Individual and environment should be

kept clean

2). Control infection （控制感染 ）

3). Stopping bleeding: prednison( 强的松）

4). Blood transfusion: Hb<60g/L. Compo

sition transfusion （成分输血）

druge 一 . Male hormone: (first selected for CAA)

(1) Effect 　　 a. directly stimulating renal EPO b. directly stimulating regeneration（增殖） and division （分裂） of stem cells. (2) Side effect: 　 a. damage liver:

　 b. manly:

(3) Dosage （剂量） :

　 testosterone propionate （丙酸睾丸酮）

　　　 50-100mg im Qd or QOD stanozol （司坦唑；康力龙） 2-4mg

　　　　　　　　　　　　　　　　　 po 　 tid danozol （哒那唑） 　 2.5-5mg 　　　　　　　　　　　　　 po 　 tid effective rate>75　

Standard of BM restored

① restored to normality of Pt.

② Product rate of clony forming unit of bone marrow cell is Normality

二 .Immunosuppressive agent1.ALG/ATGa. effect: correcting immune disor

der and stimulating hematopoietic （造血） function of BM (effective rate 79%)

b. ALG/ATG 10-15mg/kg/ 日 + DXM 10mg + N.S 500ml ／ iv gtt ．　 5days/coures.

(First take skin test)

c. By-effect:

hypersensitivity （超敏反应） ;

　　 serum-disease （血清病）

bleeding （出血）

2. McAb-T (Monoclonal Antibody-T ；T- 淋巴细胞单克隆抗体 )

OKT-3 5mg+NS 150ml / iv gtt .

QdΧ 7days.

(inhibiting IL-2,IFN secreted by TLC. indirectly stimulating hematopioetic function of BM) ．

effective rate 75.8%

3. CsA (Cyclosporin A ；环胞菌素Ａ ) CsA 0.4~ ５ mg/kg/day, Po , 3 weeks- 3months/course.

( inhibiting product IL-2 and secretion interferon of TLC).

effective 55±%

By-effect: damage function of liver and renal

4.Methylprednisolone( 甲基强的松龙） 20~30mg/kg by iv, QdΧ 3-14da

y.5.γ-Globulin 0.5-0.9g/kg,by iv QdΧ5

days

三 .cell factors:1. GM-CSF 8~60ug/kg/d . H. 14d/course .>3month 2. EPO 6000u /d. H. Q3W→Q2w→QW > 3 months effective rate 83.3% - 100%

3. IL-3:

250~500ug/M2/d . H. 15d/course

prompt development of pluripotent stem cell( 多能干细胞）

四 .Bone Marrow Transplantation (BMT)

There are there kinds of BMT Syn-BMT （同基因 BMT ） All-BMT （同种异基因 BMT ） ABMT （自体 BMT ） transfusion mounts of BM cell ar

e need o.9~ 14.2Χ 10.8/kg

The indication for BMT:

1.Younger patient (<40) with AAA/SAA

2.not or small blood transfusion, without any infection

3.There is donor’s HLA matching

to the patient

There are some diffcults:

1. Actually it is diffcult to get homogenic （同基因） BM although syn-BMT has high success

2. GVHD (Graft versus Host disease;移植抗宿主病） and GR (graft reject)

often occurs with Bleeding or infection.

３ .Money is too expansive(>200000yuan)

transplant of funicular blood stem cells

Transfusion of fetus liver cells

Prognosis( 预后 )

1/3~1/2 of AAA will died in intracranial hemorrhage （颅内出血） and/or septicemia （败血症） several months to 1 year after onset.

1/3~1/4 of CAA will delay for many years.

[Prevention] not abuse harmful drugs to BM

protection environment,

prevention from toxic substances