8/9/2019 K-12.2Anemia Aplastik 2

1/16

plastic

nemia

Child Health Department

School of MedicineUniversity of Sumatera Utara

8/9/2019 K-12.2Anemia Aplastik 2

2/16

Aplastic Anemia is disorder of hematopoiesis

characterized by severe and generalized reduction

or depletion of erythroid , myeloid and

megakaryocytic elements in the marro ith

resultant peripheral pancytopenia

Definition

8/9/2019 K-12.2Anemia Aplastik 2

3/16

8/9/2019 K-12.2Anemia Aplastik 2

4/16

Classification

I.Constitutional 1. With congenital anomalies (Fanconi anemia)

2. Without congenital anomalies

II.Acquired

1.Idiopathic : without evidence o an! etiolog! agent 2.Associated with radiation" chemical " drugs "inection "

s!stemic diseases" immunologic disorders " pregnanc!"leu#emia

8/9/2019 K-12.2Anemia Aplastik 2

5/16

8/9/2019 K-12.2Anemia Aplastik 2

6/16

Clinical features

$ irect result o depressed hematopoietic unction

petechiae "ecch!moses"purpura"epistais"

pallor"atigue"d!spnea"ever

$ A&sent hepatomegal! or splenomegal!

$ Congenital anomali :

a&normal pigmentation o the s#in "short stature with

delicate eatures " s#eletal malormation " renal

anomalies " microcephal! " microphthalmia" stra&ismus "

ear anomalies

8/9/2019 K-12.2Anemia Aplastik 2

7/16

8/9/2019 K-12.2Anemia Aplastik 2

8/16

Ac$uired Aplastic Anemia

Ac$uired Aplastic Anemia %AA& or Ac$uired bone

marro failure : panc!topenia with a h!pocellular &one

marrow a&sence o an inherited s!ndrome

Childhood (* 1+ !) :

$ Incidence :1,-1///.///!ear

$ 0/ severe disease at the time o presentation

$ 'ale 0 emale$ Asia 2 or - old higher than West due environment or

genetic dierence

8/9/2019 K-12.2Anemia Aplastik 2

9/16

'he (nternational Agranulocytosis and Aplastic Anemia

Study

Aplastic Anemia :

3emoglo&in 4 1// gd5

6latelet count 4 +/ 1/ 5

7ranuloc!tes 4 1.+ 1/ 5

8one marrow &iops! : decrease in celularit! and a&sence

o signiicant i&rosis or neoplastic iniltration

8/9/2019 K-12.2Anemia Aplastik 2

10/16

The International Agranulocytosis and Aplastic

Anemia Study (IAAS) :

Severe aplastic anemia (SAA)

cellularity of bone marra less than )*+ ith to of peripheral

blood findings

- granulocytes . /0* 1 2/ 34

- platelets . )/ 1 2/ 3 4

- absolute reticulocytes 5 6/ 1 2/ 34 %. 2+ hen corrected forHt&

Very severe aplastic anemia

Criteria for SAA and granulocyte count is . /0) 1 2/ 3 4

Moderate Aplastic Anemia

A7C *//-20*// 3mm8, platelet count )/0///-2//0///3mm8,reticulocyte .2+

8/9/2019 K-12.2Anemia Aplastik 2

11/16

#tiology

$ Idiopathic :/ 9 /

$ ;econdar! :, drugs and toins

, viral inections , 3epatitis,associated AA

, Immune disorders

, 6aro!smal nocturnal hemoglo&inuria

8/9/2019 K-12.2Anemia Aplastik 2

12/16

9athophysiology

autoimmune attac! on hematopoietic progenitor cells,

inherent stem cell defects , and defects of the bonemarrow stroma or microenvironment

8/9/2019 K-12.2Anemia Aplastik 2

13/16

Clinical presentation;!mptoms related to their c!topenias

$ wea#ness and pallor

$ petechiae " purpura" &leeding

$ requent or severe inections

$ panc!topenia with empt! &one marrow

8/9/2019 K-12.2Anemia Aplastik 2

14/16

8/9/2019 K-12.2Anemia Aplastik 2

15/16

'reatment

;upportive care

Androgen and glucocorticosteroid therap!

3ematopoietic stem cell transplantation (3;C%)Immunosuppressive therap!

8/9/2019 K-12.2Anemia Aplastik 2

16/16