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
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

Essentials of diagnosis & typical features

-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

- 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
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

Essentials of diagnosis & typical features

- 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
Essentials of diagnosis & typical features
- 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