APLASTIC ANEMIA &

IRON DEFICIENCY ANEMIA

Dr dr H IRZA WAHID SpPD KHOM FINASIM

SUBDIVISION OF HEMATOLOGY & MEDICAL ONCOLOGY
DEPARTEMENT OF INTERNAL MEDICINE
FACULTY OF MEDICINE ANDALAS UNIVERSITY
Hematologi Dan Onkologi Medik
Hematologi : Ilmu + Darah

Hemopoesis :
Sum-sum tulang --------------------------------> Darah tepi

* Eritropoesis eritrosit Anemia / Polisitemia

* Megakariopoesis trombosit Trombositosis / Trombositopenia
* Granulopoesis leukosit Leukopenia / Leukositosis
* Monositopoesis monosit Monositopenia / Monositosis
* Limfopoesis limfosit Limfositopenia / Limfositosis

Extramedular --------------------------------------- > Hati / Limpa

Onkologi Medik : Ilmu + Tumor + Non Surgery

 ANEMIA
PARAMETER : Kadar hemoglobin Metode Sahli
Pria dewasa : Wanita dewasa : Hamil : Hb < 13 : < 12 : < 11 gr %

GEJALA & TANDA

Hb hipoksia kompensasi kardiovaskular
* Pucat * angina pektoris * kardiomegali
Mukosa * claudicatio intermiten * palpitasi
Kulit * tinitus * dispneu
* berkunang * bising sistolik
* cepat lelah * gagal jantung
DIAGNOSIS
Gradasi anemia ringan : sedang : berat : > 8 : 6 8 : < 6 gr %
Morfologi mikro / normo / makrositer -- hipo/normo/hiperkrom
Patofisiologi defisiensi aplastik hemolitik perdarahan
Etiologi Cacing, low intake, ggn imun, trauma, chronic disease
Anemic Ranges of Hemoglobin and Hematocrit Values
WHO. Worldwide Prevalence of Anaemia 1993-2005.5
*These are only guidelines and some physicians feel the thresholds should be higher for adults.

Age/Sex (yrs) Hemoglobin (g/dL) Hematocrit (%)

Children (0.5-4) < 11.0 < 33

Children (5-12) < 11.5 < 35

Children (12-15) < 12.0 < 36

Adult Men < 13.0 < 39

Non-pregnant Women < 12.0 < 36

Pregnant Women < 11.0 < 33

 APLASTIC ANEMIA

Definition
Peripheral Pancytopenia
Anemia
Leucopenia
Thrombocytopenia

Reticulocyto-penia

Aplastic bone marrow

Hypocellular with all elements
down; mostly fat and stroma
Residual hematopoietic cells
are normal
No malignancy or fibrosis
No megaloblastic
hematopoiesis
 Severity of Disease
Severe Aplastic Anemia (SAA)
Marrow of less than 25% normal cellularity
2 of 3 abnormal peripheral blood values
Absolute reticulocyte count < 40,000
ANC<500
Platelets <20,000

Very Severe Aplastic Anemia (VSAA)

Above with ANC <200
 History of Aplastic anaemia

Paul Ehrlich (1854-1915) described the

first case of aplastic anaemia in a
pregnant woman who died of marrow
failure in1888.

The term aplastic anaemia first used

by Anatole Chauffard in 1904.
 Aplastic Anemia epidemiology

annual incidence in Europe and US - 2 cases per

million population, but 4 cases in Bangkok 6 in
Thailand and 14 in Japan.
no racial predisposition exists in the United
States; however, prevalence is increased in the
Far East.
The male-to-female ratio is approximately 1:1.
Aplastic anemia occurs in all age groups.
a small peak in incidence in childhood.
a peak incidence in people aged 20-25 years, and a peak
in people older than 60 years.
 Aplastic Anemia - Etiology
Congenital/inherited (20%)
Patients usually have dysmorphic features or physical
stigmata. Occasionally, marrow failure may be the initial
presenting feature.
Fanconi anemia
Dyskeratosis congenita
Shwachman-Diamond syndrome
Familial aplastic anemia

Acquired
 Aplastic Anemia: (Cont.)
Acquired:
* Drugs
- Cytotoxic drugs - Antibiotics
- Chloramphenicol - Anti-inflammatory
- Anti-convulsant - Sulphonamides
- 2-3 months usually between exposure and the development of aplastic anemia.
* Radiations
* Chemicals e.g., Benzene and pesticides
* Viruses:
Hepatitis B, C
Herpes simplex
E-B virus
HIV
* Immune: SLE, RA (rheumatoid arthritis)
* Pregnancy
* PNH
* Idiopathic ( Primer ): 75%
Pathogenesis

Immune mechanism responsible for

most of the cases of Idiopathic acquired
aplastic anemia
Activated Cytotoxic T cells in Blood &
Bone marrow Bone marrow failure
Clinical Features

Anemia

Bleeding: Ecchymoses ,Bleeding gums,

Epistaxis

Infections: Fever,Mouth ulcers

Diagnosis

Blood peripheral smear :

Pancytopenia and reticulocytopenia

Bone marrow aspiration & biopsy :

Hypocellular / aplastic bone marrow with
increased fat spaces

Tests for underlying cause ( viral titers)

BM Aspiration BM Biopsy
 BM biopsy
hypocellular ,increased fat spaces
 Other causes of Pancytopenia:
Sign of congenital disease
Drugs,
Viral
Megaloblastic anemia
Bone Marrow infiltration or Replacement:
SLE, RA
Disseminated TB
PNH
Sepsis
If (-)
idiophatic or primary acquired aplastic anemia
 Aplastic Anemia - Treatment

Withdrawal of etiological agents.

Management of underlying disesase
Supportive.
Restoration of marrow activity:
Bone marrow transplant
Immunosuppressive treatment
- Prednisolone - Antilymphocyte globulin.
- Cyclosporin
- Splenectomy
Androgens
Growth factors
 APLASTIC ANEMIA treatment

Supportive care
Transfusion
Treatment of anemia
Treatment of bleeding
Prevention and treatment of
infection
 Hematopoietic stem cell
transplatation in severe aplastic
anemia
1. Advantages
- correction of hematopoietic defect
- long-term survival: 80% - 90% (HLA-matched sibling donor)
- majority of the patients appear to be cured
2. Restrictions
- age below 40
- suitable donor available in less than 30% (sibling)
- 25-40% risk of GVHD
- 5-15% risk of graft failure in multitransfused patients
- high mortality after MUD-HSCT
- solid tumors (12%)
 Immunosuppression
Immunosuppression is NOT curative
Goal is sustained remission
20-36% have recurrent aplastic anemia
20-36% develop clonal disorder, PNH, MDS or acute leukemia
Combination therapy is best
Antithymocyte globulin (ATG) first choice
Toxic side effect is serum sickness, tx with steroid
Can lower platelet counts, transfuse prn
Cyclosporine first choice
High dose corticosteroids tappering off
Alemtuzumab
Azathioprine
Mycophenolate mofetil
 Guidelines for the diagnosis and management of adult aplastic anaemia

British Journal of Haematology

Volume 172, Issue 2, pages 187-207, 16 NOV 2015 DOI: 10.1111/bjh.13853
http://onlinelibrary.wiley.com/doi/10.1111/bjh.13853/full#
 ANEMIA DEFISIENSI BESI

Prevalensi Anemia Defisiensi Besi di dunia

Afrika Amerika Indonesia

Latin

Laki dewasa 6% 3% 16-50%

Wanita tak hamil 20% 17-21% 25-48%
Wanita hamil 60% 39-46% 46-92%
 ANEMIA DEFISIENSI BESI
Metabolisme Besi
Intake ( Fe 3+ = Ferri )
( daging organ, kacang polong, air tebu, daun sup, kerang )

Fe 3+ Fe 2+ = Ferro
Mukosa duodenum / jejunum prox ( Absorbsi )

Fe 2 + Fe 3+
Transferin ( Transport )

Mioglobin / enzim Hemoglobin

(Jaringan) (Eritrosit)

Feritin / hemosiderin ( Storage )

( Sistim RE )
Etiologi
Asupan Kehilangan Fe Kebutuhan meningkat
- malnutrisi - perdarahan kronik - hamil / laktasi
(cacing tambang)
- ggn absorbsi - GGK / HD - anak-anak
 IRON DEFICIENCY - STAGES
Prelatent
reduction in iron stores without reduced serum iron levels
Hb (N), MCV (N), iron absorption (), transferin saturation
(N), serum ferritin (), marrow iron ()
Latent
iron stores are exhausted, but the blood hemoglobin level
remains normal
Hb (N), MCV (N), TIBC (), serum ferritin (), transferrin
saturation (), marrow iron (absent)
Iron deficiency anemia
blood hemoglobin concentration falls below the lower limit of
normal
Hb (), MCV (), TIBC (), serum ferritin (), transferrin
saturation (), marrow iron (absent)
 GEJALA KLINIS
- gejala anemia
- gejala defisiensi atrofi papil lidah, stomatitis angularis,
disfagia, koilonychia
- gejala penyakit dasar

DIAGNOSIS
* Anemia, retikulosit menurun
* Mikrositer, hipokrom, anulosit
* SI menurun / TIBC meningkat
* Transferin < 16 mg %
* Feritin serum < 12 mg %
* SST : Hiperplasia eritropoetik, pengecatan besi ( - )
* Pemeriksaan untuk mencari penyebab
 PENATALAKSANAAN

* Preparat besi 3 X 200 mg Hb normal

maintenance 3 X 100 mg (3 bln)
* Pengobatan penyakit dasar