Aplastic Anemia

Primary acquired bone marrow

failure syndrome
Y Zhang
 Case
• ＊ Huang ， male ， 19yrs
• Epistaxis for 3 days,accompany with
petechia and oral mucosal hematoma
• Fever for 1 day
• Anemic signs
 Case
• CBC

Hgb(g/L WBC(×109/
PLT (×109/L)
) L)

*Huan
71 1.05 7
g
Norm pancytopenia
130-175 3.5-9.5 100-300
al
 Bone marrow
hematopoiesis
Aplastic Anemia
Primary acquired bone marrow
failure syndrome
Definition
• primary acquired bone marrow failure syndro
me
• Severe Aplastic Anemia,SAA
• Non-Severe Aplastic Anemia,NSAA
Epidemiology
• Incidence:7.4/million
• Old people have higher morbidity.
Clinical manifestation
• epistaxis,petechia,oral mucosal hematoma bleeding

• fever infection
Pancytopenia
• anemic signs anemia
 Laboratory tests
• CBC ： reticulocyte decrease 、 normal MCV
pancytopenia 、

Hgb WBC PLT MCV Reti

(g/L) (×109/L) (×109/L) (fl) (×1012/L)
*Huang 71 1.05 7 94.1 0.002
Normal 130-175 3.5-9.5 100-300 82-100 0.024-0.084
Laboratory tests

normal NSAA SAA/*Huang

Blood pancytopenia,normal
smear: morphology
 Laboratory tests

normal NSAA SAA/*Huang

Bone marrow swear ：
hematopoietic cell decrease,
Megakaryocyte decrease or disappear
 Laboratory tests

normal NSAA SAA/*Huang

Bone marrow hematopoietic tissue decrease,fat tissue increase
biopsy ：
 Clinical manifestation of *Huang
Diagnose
or not ？
bleeding,pneumonia,anemia
pancytopenia 、 Reti decrease 、 normal MCV
hematopoietic cell decrease ， megakaryocytes disappear
hematopoietic tissue decrease, fat tissue increase

blood smear bone marrow smear bone marrow biopsy

 Diagnostic criteria of AA
1. pancytopenia,reticulocytes decrease
2. no hepatomegaly and splenomegaly
3. hematopoietic cell decrease,megkaryocytes decrease or disappear
4. hematopoietic tissue decrease,fat tissue increase

5. eliminate other diseases that cause pancytopenia

Differential diagnosis
• The following diseases also can cause pancytopenia
1.Other type bone marrow failure
second Virus infection, chemicals, radiation
：enetic ：＜ 40yrs,gene tests
g
2.Other diseases
hematopoietic diseases ： PNH 、 MDS 、 IRP 、 HLH
non-hematopoietic diseases ： AD 、 metastatic
tumors
*Huang was diagonised AA
by excluding other diseases
that cause pancytopenia.
Subtypes of AA
• SAA:(match 2/3)
N<0.5×109/L
Plt ＜ 20×109 ／ L
Reti ＜ 15×109 ／ L
• NSAA ：
Question:*Huang SAA or NSAA
Hgb WBC PLT MCV Reti
(g/L) (×109/L) (×109/L) (fl) (×1012/L)
*Huang 71 1.05 7 94.1 0.002
Normal 130-175 3.5-9.5 100-300 82-100 0.024-0.084
Causes
• Unknown
Mechanism
Mechanism
• Celluar immune injury
CD8+T Cell HSPCs

INF-γ

TNF-α
apoptosis↑ proliferation↓
ATG,CSA

Bone marrow failure

Mechanism
Treatment
SAA NSAA

Allo-HSCT CSA based

Antithymocyte globulin(ATG):3-5g/kg/d,5d
CyclosporinA(CSA):5mg/kg/d,1yr at least

Support care is the foundation

Treatment
SAA

<40yr
allo-HSCT s

suitable donor

no contraindication
Prognosis
• response rate of ATG+CSA is 70-80% (>3m)
• response rate of allo-HSCT is 80%(1m)
• response rate of CSA based treatment is 50%(>
6m)
Three months latter

blood smear bone marrow smear bone marrow biopsy

A year latter
Summary

• CD8+T↑ • Timely diagnosis

• Bone marrow failure • Timely treatment
• pancytopenia • Maximum restoration
• Anemia,infection,bleedin
g
• Exclusion diagnosis
Case1
A female patient,45yrs,hypermenorrhea for two weeks,the skin is
scattered over the petechia.CBC:RBC:2.68×1012/L,Hgb:76g/L,MCV:
82fl,WBC:1.02×109/L,N:0.46×109/L,PLT:15×109/L,Reti:0.03×1012/L.

PML
Case2
A male patient,65yrs,cough and fever for three days.pale face can be
found,CBC:RBC:2.35×1012/L,Hgb:68g/L,MCV:90fl,WBC:3.02×109/L,
N:2.46×109/L,PLT:48×109/L,Reti:0.018×1012/L.

MM
Case3
A female patient,41yrs,epistaxis for five days.38.6℃,moist crackles
can be heared in lower right lung,CBC:RBC:3.35×1012/L,Hgb:96g/L,
MCV:98fl,WBC:0.96×109/L,N:0.17×109/L,PLT:5×109/L,Reti:0.02×1
012/L.

ALL
Question
• How to diagnose aplastic anemia
• Which lab tests should be done
• Why
Thanks!