Aplastic Anemia
(AA)
Zhongshan Hospital,Fudan University
Zou Shanhua
。
Outline
? Bone marrow hematopoiesis
failure led by chemicals,
radiation,biological factors and
unknown etiology,
? The common clinical
presentation is mild to severe
pancytopenia,
Epidemiology
? The incidence rate of AA,0.74
per 100,000,
AAA, 0.14 per 100,000,
CAA, 0.60 per 100,000,
? Distribution,most AA patients
are young and middle-aged
people,male more than female
Etiology
? Primary AA,unknown causes，
about 60~70%
? Secondary AA
Etiology of Secondary AA
? Chemicals
Drug,antibiotics,antineoplastic
agent,NSAIDs
Other chemicals,
benzene and benzene derivatives,
arsenic,heavy metals,insecticides
Etiology of Secondary AA
? Radiation
? Viral infection,Hepatitis virus,EBV
Mechanism
? Hematopoietic stem cell defect
Stem cells count decrease
Stem cells proliferation
capacity decrease
Mechanism
? Hematopoietic microenviroment
defect
stromal cells
hematopoietic growth factor
microcirculation
Mechanism
? Immunologic derangement
CD4/CD8（ ↓,inversion)
γ-IFN,TNFα,IL-2↑
immunosuppressive therapy
Clinical Features
? Clinical features,
anemia,infection,hemorrhage
? Differences,
AAA(SAA-Ⅰ ),CAA,SAA-Ⅱ
Laboratory study—
complete blood count
? Common feature,
anemia,reticulocyte count ↓
granulocytopenia
thrombocytopenia
percent of lymphocyte increase
relatively
Laboratory study—
complete blood count
? SAA,
Ret < 1%
Granulocyte count <0.5× 109/L
BPC<20× 109/L
? CAA,
Ret > 1%
Granulocyte count >0.5× 109/ L
BPC>20× 109/L
Laboratory study－ bone
marrow
? Common feature,
Naked eye,bone marrow is
plenty of oil drops
Microscope,
hypoplasia or polar hypoplasia;
hematopoietic cells decrease,
nonhematopoietic cells increase
Laboratory study－ bone
marrow
? SAA:polar hypoplasia,progenitor
cell can hardly be seen,no
megakaryocyte
? CAA,hypoplasia,megakaryocytes
decrease or disappear
Laboratory study－ bone
marrow biopsy
? More valuable than marrow aspiration
? Ratio of hematopoietic tissue/fatty tissue
? Megakaryocyte proliferation can be
evaluated accurately;
Laboratory study
? CFU-GM↓
? NAP ↑
? EPO ↑
Criteria of diagnosis
? Pancytopenia,reticulocyte absolute
count↓
? Without splenomegaly
? Hypoplasia or polar hypoplasia of bone
marrow; if not,megakaryocyte should
disappear;
? Biopsy,hematopoietic tissue↓
fatty tissue↑
? Excluding other diseases with
pancytopenia
? For anemia,ordinary treatment is
ineffective
Criteria of diagnosis
AAA(SAA-Ⅰ )
CAA
SAA-Ⅱ
Differential diagnosis
? PNH,
there is CD55 and CD59-depleted cell clone
? MDS,
essence,clonal disease,
dyshaematopoiesis,
chromosal aberration
? Aleaukemic leukemia
Differential diagnosis
? malignant histocytosis
? myelofibrosis
treatment
Support treatment,
? Enviroment protection and self-
protection
? Transfusion
? Infection and hemorrhage of
prenvention or treatment
treatment
? Androgen,
Testosterone Propionate,
Stanozolol,
Testosterone Undecanoate
? Immunosuppression,
ALG,ATG,cyclosporine
High-dose immunoglobulin or methyprednisolone
? Marrow transplantation
? Hematopoietic growth factors,
G-CSF,GM-CSF,EPO
prognosis
?SAA,
?CAA,