Aplastic

Anemia
Child Health Department
School of Medicine
University of Sumatera Utara

Definition

Aplastic Anemia is disorder of hematopoiesis

characterized by severe and generalized reduction
or depletion of erythroid , myeloid and
megakaryocytic elements in the marrow with
resultant peripheral pancytopenia

Classification
I.Constitutional
1. With congenital anomalies (Fanconi anemia)
2. Without congenital anomalies

II.Acquired
1.Idiopathic : without evidence of any etiology agent
2.Associated with radiation, chemical , drugs ,infection ,
systemic diseases, immunologic disorders , pregnancy,
leukemia

Fanconis Anemia

Essentials of diagnosis
Thrombocytopenia or neutropenia progressing to
pancytopenia
Macrocytosis
Multiple congenital anomalies
Increased chromosome breakage in peripheral blood
lymphocytes

Clinical features
Direct result of depressed hematopoietic function
petechiae ,ecchymoses,purpura,epistaxis,
pallor,fatigue,dyspnea,fever
Absent hepatomegaly or splenomegaly
Congenital anomali :
abnormal pigmentation of the skin ,short stature with
delicate features , skeletal malformation , renal
anomalies , microcephaly , microphthalmia, strabismus ,
ear anomalies

Acquired Aplastic Anemia

Acquired Aplastic Anemia (AA) or Acquired bone
marrow failure : pancytopenia with a hypocellular bone
marrow absence of an inherited syndrome
Childhood (< 15 y) :
Incidence :1-3/1000.000/year
>70% severe disease at the time of presentation
Male > female
Asia 2 or 3 fold higher than West due environment or
genetic difference

The International Agranulocytosis and Aplastic Anemia

Study
Aplastic Anemia :
Hemoglobin 100 g/dL
Platelet count 50 x 10/L
Granulocytes 1.5 x 10/L
Bone marrow biopsy : decrease in celularity and absence
of significant fibrosis or neoplastic infiltration

The International Agranulocytosis and Aplastic

Anemia Study (IAAS) :
Severe aplastic anemia (SAA)
cellularity of bone marraw less than 25% with two of peripheral
blood findings:
- granulocytes < 0.5 x 10 /L
- platelets < 20 x 10 / L
- absolute reticulocytes 40 x 10 /L (< 1% when corrected for
Ht)
Very severe aplastic anemia
Criteria for SAA and granulocyte count is < 0.2 x 10/ L
Moderate Aplastic Anemia
ANC 500-1.500 /mm, platelet count 20.000-100.000/mm,
reticulocyte <1%

Etiology

Idiopathic :70 80 %
Secondary :- drugs and toxins
- viral infections
- Hepatitis-associated AA
- Immune disorders
- Paroxysmal nocturnal hemoglobinuria

Pathophysiology
autoimmune attack on hematopoietic progenitor cells,
inherent stem cell defects , and defects of the bone
marrow stroma or microenvironment

Clinical presentation
Symptoms related to their cytopenias
weakness and pallor
petechiae , purpura, bleeding
frequent or severe infections
pancytopenia with empty bone marrow

Diagnosis and Laboratory evaluation

Family and developmental histories
History previous hematologic derangements,drug
exposure
Careful physical examination
Lab : - complete blood count
- peripheral blood smear
- Level Hb F
- Red cell indices : MCV
- Bone Marrow examination

Treatment

Supportive care
Androgen and glucocorticosteroid therapy
Hematopoietic stem cell transplantation (HSCT)
Immunosuppressive therapy
Removal of the causative agent