Anemia: Prof. Dr. Rafita Ramayati, Spa (K) Prof. Dr..Rusdidjas, Spa (K) Dr. Oke Rina Ramayani, Spa BGN Ilmu Kes. Anak Fk-Usu

ANEMIA
Prof. dr. Rafita Ramayati,SpA(K)

Prof. dr..Rusdidjas, SpA(K)
dr. Oke Rina Ramayani, SpA
Bgn Ilmu Kes. Anak FK-USU
Definisi Anemia;
 Adalah berkurangnya kadar Hb (g%)
dalam darah dibandingkan dgn Standard
Referens.(Normal) Tergantung Usia,
Jenis Kelamin.
HEMATOLOGIC
HEMATOLOGIC VALUES
VALUES IN
IN NORMAL
NORMAL
INDIVIDUALS
INDIVIDUALS OF
OF VARIOUS
VARIOUS AGE
AGE GROUPS
GROUPS
Age (Usia) Hemoglobin Hematocrit MCV
Kelamin (gm/dl) (%) (cu microns)
mean(-2SD) mean(-2SD) mean(-2SD)
1-3 days 18.5(14.5) 56(45) 108(95)
0.5-2 yrs 12(10.5) 36(33) 78(70)
12-18 yrs
male 14.5(13) 43(37) 88(78)
female 14.0(12) 41(36) 90(78)
18-49
male 15.5(13.6-17.2) 47(39-49) 90(76-100)
female 14(12.0-15) 41(33-43) 90(76-100)
Klasifikasi Anemia
 1).Defisiensi Anemia (Kekurang bahan
utk produksi Hb) –Anemia def. besi
 2).Hemolytic Anemia ( Banyak
penghancuran RBC)
 3).Hemorrhagic Anemia (Hilang Cucur
darah)
 4).Aplstic Anemia (Frabrik pembuat
darah tidak bekerja)
Klasifikasi Morphologik
 Ukuran RBC :1). Microcytic
2). Normocytic
3). Macrocytic
 Warna RBC 1). Hypochromic
2). Normochromic
3). Hyperchromic
Contoh : lihat berikut

Morphologic classification of anemias
Type MCV MCHC Common cause

________________________________________________________
Macrocytic anemia increased normal Vitamin B12 deficiency
Folic acid deficiency
Microcytic anemia
- hypochromic decreased decreased Iron deficiency
Thalassemia
- normochromic decreased normal Spherocytosis
or normal
Normocytic anemia normal normal Aplastic anemia
- normochromic Chronic renal failure
Some hemolytic
anemia
HEMOLYTIC ANEMIA
1.CONGENITAL HEMOLYTIC ANEMIA
2.AQUIRED HEMOLYTIC ANEMIA

1.CONGENITAL HEMOLYTIC ANEMIAS :
A.RED CELL MEMBRANE DEFECTS

A.1. Hereditary Spherocytosis
Essentials of diagnosis & typical features
 Anemia and jaundice
 Slpenomegaly
 Positive family history of anemia, jaun-
dice or gallstones.
 spherocytosis with reticulosytes
 osmotic fragilility
 negative coombs test
Spherocytosis
A.2. Hereditary Elliptocytosis
 autosomal dominant inheritance

 most are asymptomatic
 elevated reticulosyte
 jaundice and splenomegaly
No treatment is indicated :
- folate suplementation
- splenectomy
Elliptocytosis
Poikilocytosis, Bentuk cel tak sama
B. HEMOGLOBINOPATHIES
CLASSIFIED INTO TWO MAJOR GROUPS
1. THALASSEMIAS
Quantitative deficiencies in the production
of globin chains
2. HEMOGLOBINS DISORDER
Structural abnormalities of globin chains
B.1. - THALASSEMIA
ESSENTIALS OF DIAGNOSIS &

TYPICAL FEATURES :
- African, Mediteranian, Middle Eastern,

Chinese, or Southeast Asian ancestry
- Microcytic, hypochromic anemia of variable

severity
- Hemoglobin Bart’s detected by neonatal

screening.
Table 1. The - thalassemias
HEMOGLOBIN ELECTROPHORESIS
USUAL - GENE CLINICAL
GENOTYPES NUMBER FEATURES
BIRTH > 6 MO
 /  4 NORMAL NORMAL NORMAL
SILENT
- /  3 0 – 3% Hb Bart’s NORMAL
CARRIER
-- /  or - THAL
2 2 – 10% Hb Bart’s NORMAL
- / - TRAIT
Hb H Hb H
-- / - 1 15 – 30% Hb Bart’s
DISEASE PRESENT
FETAL
-- / -- 0 > 75% Hb Bart,s -
HYDROPS
Thalassemia _alpha
Teardrop
Differential Diagnosis :
Thalassemia trait :
DD/ - Iron Deficiencies Anemia
- -thalassemia minor
Hb H disease :
DD/ - other hemolytic anemias
Hydrops fetalis :
DD/ - hydrops due to other causes of
anemia such as isoimmunization
Treatment
-thalassemia trait require no treatment
Hb H disease should receive folic acid

avoid the same oxidant drugs
Transfusions may be required
Splenectomy
Genetic counseling and prenatal diagnosis

B.2. -Thalassemia
-thalassemia minor
- Normal neonatal screening test
- African, Mediterranean, Middle Eastern or
Asian ancestry
- Mild microcytic, hypochromic anemia
- No respon to iron therapy
- Elevated level of Hb A2
-thalassemia major :
- Neonatal screening shows Hb F only

- Mediterranean, Middle Eastern, or Asian
ancestry
- Severe microcytic, hypochromic anemia with
marked hepatoslenomegaly
Symptoms and Signs
-thalassemia minor are asymptomatic

-thalassemia major are normal at birth but
develop significant anemia during the 1st year
Thalassemia_beta
Thalassemia minor
Thalassemia major
Differential Diagnosis
-thalassemia minor :
DD/ - Iron Deficiencies Anemia
- -thalassemia
-thalassemia major is rarely confused with

other disorders.
Treatment
• -thalassemia minor requires no specific

theraphy
• -thalassemia major :
- Chronic transfusion with iron chelation

-- [‘desferal’ pembuang zat besi]
- Stem cell transplantation
B.3. Sickle cell disease
Essentials of diagnosis & typical feature
• Neonatal screening test with Hb FS, FCS, or FSA
• African, Mediterranean, middle eastern, Indian,
Caribbean ancestry
• Anemia, Elevated Reticulocyte, jaundice
• Recurrent episodes of musculoskeletal or abdo
minal pain
• Hb electrophoresis with Hb S and F; Hb S and C
or Hb S, A and F with S>A
• Splenomegaly in early childhood with later disap
perance
• High risk of bacterial sepsis
Table 2. Common clinical manifestation of SCD
Acute Chronic
Children Bacterial sepsis or Functional asplenia
Meningitis Delayed growth and
Splenic sequestration Development
Aplastic crisis A vascular necrosis of
Vaso-occlusive events The hip
Dactylitis Hypothenuria
Bone infarction Cholelithiasis
Acute chest syndrome
Stroke
Priapism
Sickle cell - bentuk sabit
Sickle cell
B.4. Sickle Cell Trait
Identification : Hb electrophoresis
shows 60% Hb A and 40% Hb S
There is no anemia and hemolysis
Physical examination is normal
Life expectancy is normal

B.5. Hb C Disorders
Detected by neonatal screening
Hb C Trait : no symptoms
target cells  blood smear
Hb C : Mild microcytic hemolytic anemia

Splenomegaly
prominent target cells
Gallstones
Aplastic crises
B.6 Hb E Disorders
Second most common
Frequency > 10% in Thailand and Cambodia
30 million people have Hb E trait in Southeast
Asia
Mild Anemia
Microcytosis and some target cells
B.7 Other Hb pathies

Hb D, Hb G, Hb D and S
Hb C
Hb H
Target cell
C. Disorders of Red Cell metabolism
C.1. Glucose-6-phosphate Dehydrogenase
(G6PD) Deficiency
- African, Mediterranean or Asian ancestry

- Neonatal hyperbilirubinemia
- Sporadic hemolysis  infection, oxidant drugs
fava beans
- X- linked inheritance.
Table 3. Some common drugs and chemicals
that can induce hemolytic anemia
Acetanilide Niridazole
Doxorubicin Nitrofurantoin
Furazolidone Phenazopyridine
Methylene blue Primaquine
Nalidixic acid Sulfamethoxazole
N Eng J Med 1991; 324;171

C.2 Pyruvate Kinase Deficiency
Autosomal recessive
Most common in northern Europeans
Chronic hemolytic anemia
Jaundice, hydrops fetalis, neonatal death
Echinocytes on blood smear
Low levels of pyruvate kinase activity
2. Acquired Hemolytic Anemia
A. Autoimmune Hemolytic Anemia
- Pallor, fatique,jaundice, dark urine
- Slenomegaly common
- Positive combs test
- Reticulocytosis & spherocytosis
Treatment
- Prednisone
- IVIG
- Immunosuppressive agent
Autoimmune Hemolytic Anemia
B. Nonimmune Acquired Hemolytic Anemia
- Hepatic disease
- Renal disease  HUS
- A microangiopathic hemolytic  DIC
- Kasabach – Merritt syndrome
TKS

Anemia: Prof. Dr. Rafita Ramayati, Spa (K) Prof. Dr..Rusdidjas, Spa (K) Dr. Oke Rina Ramayani, Spa BGN Ilmu Kes. Anak Fk-Usu

Uploaded by

Copyright:

Available Formats

Anemia: Prof. Dr. Rafita Ramayati, Spa (K) Prof. Dr..Rusdidjas, Spa (K) Dr. Oke Rina Ramayani, Spa BGN Ilmu Kes. Anak Fk-Usu

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Anemia: Prof. Dr. Rafita Ramayati, Spa (K) Prof. Dr..Rusdidjas, Spa (K) Dr. Oke Rina Ramayani, Spa BGN Ilmu Kes. Anak Fk-Usu

Uploaded by

Copyright:

Available Formats

ANEMIA

Prof. dr. Rafita Ramayati,SpA(K)

1-3 days 18.5(14.5) 56(45) 108(95)

0.5-2 yrs 12(10.5) 36(33) 78(70)

Contoh : lihat berikut

Type MCV MCHC Common cause

1.CONGENITAL HEMOLYTIC ANEMIA

2.AQUIRED HEMOLYTIC ANEMIA

A.RED CELL MEMBRANE DEFECTS

 autosomal dominant inheritance

CLASSIFIED INTO TWO MAJOR GROUPS

ESSENTIALS OF DIAGNOSIS &

- African, Mediteranian, Middle Eastern,

- Microcytic, hypochromic anemia of variable

- Hemoglobin Bart’s detected by neonatal

 /  4 NORMAL NORMAL NORMAL

Hb H disease should receive folic acid

Transfusions may be required

Genetic counseling and prenatal diagnosis

Essentials of diagnosis & typical features

- Neonatal screening shows Hb F only

Symptoms and Signs

-thalassemia minor are asymptomatic

-thalassemia major is rarely confused with

• -thalassemia minor requires no specific

- Chronic transfusion with iron chelation

There is no anemia and hemolysis

Physical examination is normal

Life expectancy is normal

Hb C : Mild microcytic hemolytic anemia

B.7 Other Hb pathies

Essentials of diagnosis & typical features

- African, Mediterranean or Asian ancestry

N Eng J Med 1991; 324;171

You might also like

Pfad - The Proxy pFad of © 2024 Garber Painting. All rights reserved.