Symptoms and treatment Flashcards
1/3 makes have jaundice
Majority are asymptomatic
Neonatal hyperbolirubinea
Acute/chronic HA
G6PD
Remove triggers to remove HA by decreasing ROS Chance
Remove fava beans
Remove medication (dapsone)
G6PD
What drug is increases ROS Chance
Dapsone
Jaundice
Splenegamoly
Extramedary hematopoiesis
Hepatosplengamoly
Autoimmune HA
Prednisone
Corticosteroids
Cytotoxic immunosuppresive drugs
Cyclosporine
Splenectomy if corticosteroids dont work 3months
Monoclonal AB
Autoimmune HA
Monoclonal AB treatment for aHA
Campath 1H
Why does harmolysis occur in RBC in HbH disease
Excess of B globin /homotetramer (HbH)
Recognised by spleen and destroyed
Extravascular
Hepatosplenomegaly
Ineffective haemopoirsis
HbH
Treatment generally not required
Antibiotics
Regular folic acid if preganant
Splenectomy
BT
HbH
Vado-occlusive
Severe bone/joint pain
Acute chest syndrome
Dactylitis (children)
Jaundice
Gall stones
Splenegomaly
Sickle cell
Life long prophylactic penicillin with folate replacement
Avoid triggers if trait (altitude.stress)
Blood transfusions +iron chelation
HSCT
Hydroxurea
Mumurs / hamaturia/renal tubular defects /strokes
Sickle cell
What does hydroxurea do
What’s it used in
Increase HbF
Sickle cell
What bacterial infection is common in sickle cell patients
Staphylococcus aureus
Avascular Necrosis if head of femur / growth retardation
Sickle cell
Parlour of mucuos membrane
Conjunctival mucosa
Brittle nails
Ulceration around mouth
Glottitis
Jaundice
IDA