SICKLE CELL DISEASE

Aditya edit Master subtitle style Click toRangga Fandiarta 1301-1210-0019

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DEFINITION
Autosomal disorder RBC rigid, irregular and sickle shaped. RBC rigidity vascular trombus complication. RBC rigidity hemolytic anemia

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EPIDEMIOLOGY
High

prevalence in person of central african origin Other ethnic : iltaly, greece, turkey, saudi arabia, india Occur in about one of every 400 afroamerican infant.

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ETIOLOGY-PATHOGENESIS

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CLINICAL MANIFESTATION
VASOOCCLUSIO N SCD HEMOLITIC CRISIS

SPLEEN CHRONIC STROK CONGESTI PAIN E ON ACUTE DACTYLITI SPLEENIC PRIAPISM S SEQUESTRA TION FUNCTIONAL ACS ASPLENIA SEPSIS
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ACUTE SPLEENIC SEQUESTRATION

Life

threatening complication Occur as early as 5 week of age. Spleenomegaly Hypovolemia shock Aplastic crisis usually caused by parvo virus Therapy

Maintenance hemodynamic stability : isotonic fluid and blood transfusion Prophylactic spleenectomy the only effective strategy.

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FUNCTIONAL ASPLENIA
Occur

as early as 6 month Abnormal immune function Complication : bacterial sepsis

Streptococcus pneumonia Haemophilus influenza B Prophylactic oral penicilin at least until 5 years old (125 mg twice daily up to 3 years, then 250 mg twice daily) Fever IV cephalosporin 3 generation.

Therapy

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CHRONIC PAIN
Abrupt,

unremitting, precipitating cause : physical stress, dehidration, infection, hypoxia. Dactylitis (Hand-Foot syndrome)

Occur in 50% of children by 2 years of age. Bilateral / unilateral swelling Therapy : analgetic (acetaminophen) Unilateral dactylitis DD/ osteomyelitis Penile erection longer than 30 minutes Therapy : sitz bath or pain medication, aspiration of blood in corpora cavernosa, irrigation with dilute epinephrine. 4/19/12

Priapism

 Acute

Chest Syndrome

Fever, pleuritic chest pain, acute pulmonary infiltrates with hypoxemia. Caused by pulmonary infection, infarction or fat embolism from ischemic bone marrow. CXR : lung consolidation, pleural effusion. Therapy :
Oxygen administration Blood transfusion Empirical antibiotic Pain control

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 Hydroxyurea

myelosupressive agent, the only effective drug proven to reduce painful syndrome Treatment begin in infancy may preserve spleen function Monitor toxicity.

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STROKE
Neurologic

deficit CT scan : cerebral infarction Therapy :

Oxygen administration Blood transfusion, erythrocytophresis

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LABORATORY FINDING
Baseline

Hb : 7-10 mg/dL Reticulocyte increase Anemia : normocytic or macrocytic BS : sickle cell with numerous target cell Hemoglobin electrophoresis sufficient to confirm diagnosis.

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PROGNOSIS
Neonatal

screening combined with comprehensive management reduced mortality.

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