Sickle cell, G6PD Deficiency and Megaloblastic anaemia

Red blood cell physiology in Sickle-Cell Anaemia

Deformed, less flexible red blood cells

Acute complication of sickle-cell anaemia

Sickle-cell crisis: restricted blood supply to organs

Treatment for sickle-cell crisis

Hospitalisation → fluid replacement, analgesia, treat any infections

Long-term complications of sickle-cell anaemia

Anaemia. Leg ulcers. Renal failure. More susceptible to infections.

Treatment for haemolytic anaemia

Folic acid supplementation

Drug used to reduce frequency of painful sickle-cell crises and reduce transfusion requirements

Hydroxycarbamide

Full name of G6PD Deficiency

Glucose-6-phosphate dehydrogenase deficiency

Populations & gender where G6PD deficiency is more common

Common in Africa and Asia. More common in males.

People with G6PD deficiency are more susceptible to developing which type of anaemia?

Acute haemolytic anaemia

Drugs with definitive risk of haemolysis in most G6PD-deficient individuals

Dapsone and other sulfones. Fluoroquinolones. Nitrofurantoin. Quinolones. Sulfonamides. Primaquine

Drugs with possible risk of haemolysis in some G6PD-deficient individuals

Aspirin. Chloroquine. Menadione. Quinine. Sulfonylureas.

(Remember Quinine’s MACS)

Two causes of megaloblastic anaemia

Malabsorption of Vitamin B12. Folate deficiency.

Treatment for megaloblastic anaemia caused by Vitamin B12 deficiency

Hydroxocobalamin at intervals of up to 3 months. Frequent IM injections initially to replenish stores → maintenance

Left of daily folic acid supplementation in megaloblastic anaemia caused by folic acid deficiency

4 months

Treatment of megaloblastic anaemia in emergencies

Administer both (hydroxocobalamin and folic acid) while waiting for results

Drug to avoid giving alone if the cause of megaloblastic anaemia is undiagnosed and why

Don’t give folic acid alone if undiagnosed → can cause neuropathy

Risk factors for neural tube defects

Smoking. Sickle-cell anaemia. Diabetes. Obesity. Anti-epileptic drugs. Antimalarial drugs.