Paediatrics: Red blood cell fragmentation

Red blood cell fragmentation

Causes

·Microangiopathic haemolytic anaemias (MAHA): includes—HUS, TTP, giant capillary haemangioma (Kasabach Merritt syndrome), and DIC.

·Infection: e.g. meningococcal, pneumococcal, malaria (black water fever- intravascular haemolysis), viral haemorrhagic fevers, Clostridium perfringens.

·Burns.

·Mechanical: e.g. prosthetic heart valves, March haemoglobinuria.

·Hereditary acanthocytosis: rare genetic condition of abetalipoproteinaemia with mental retardation, ataxia, retinitis pigmentosa, and steatorrhoea.

·Envenomation from several of the worlds venomous snakes, spiders, etc.

Clinical features

Depend on underlying cause and severity of anaemia.

Laboratory investigations

·Hb d.

·Blood film: reticulocytes, nucleated RBC ii, RBC fragmentation, shistocytes, irregularly contracted cells, microspherocytes, acanthocytes.

·Possible platelets ‘fall’ or clotting prolongation with consumption.

·In malaria, visible parasites on thick/thin blood film.

Treatment

·Treat underlying disease.

·Correct haematological abnormalities, e.g. blood +/– platelet transfusion, fresh frozen plasma to correct clotting abnormalities.

Give iron or folate supplements if required.