Sickle Cell Anemia

15 Apr, 2022

Dr. Rajesh Bollam

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Sickle cell anemia : one of the common consults in the OPD
Clinical features :
Anemia
Shortness of breath
Infection
Crisis
Gall stones
Leg ulcers
Brain stroke
Investigation :
CBP ( peripheral smear) shows sickle cells
Anemia
Sickling test ( screening test )
Hb electrophoresis
Management
Hydration ( IV fluids )
Oxygen
Pain control ( especially in vasoocclusive crisis )
Blood transfusion ( hb to maintain around 9 )
Treat precipitating factors
Specific Treatment
1) Hydroxyurea ( increase HBF )
2 ) Endari ( Oral L glutamine ) ..for pain crisis
3) Adakveo ( Crizalizumab ) ... ( Monoclonal antibody which binds to P- selectin , blocking it's interaction with PSGL 1 on neutrophils and monocytes . )
By inhibiting platelet aggregation maintains normal blood flow and thus minimises sickle cell related pain crisis
4)Voxelotor ( sickle cell heamoglobin polymerization inhibitors )
5)CRISPR gene therapy
6)Bone marrow transplant


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