Introduction
Hyperhemolysis syndrome (HS), a potentially life-threatening condition,
was first described in sickle cell disease but was subsequently seen in
other hematological conditions such as thalassemia, myelofibrosis and
lymphoma1. This syndrome is characterised by the
occurrence of severe anemia with posttransfusion hemoglobin levels being
lower than pretransfusion levels2. Intravascular
destruction of both host and transfused red blood cells occurs as
evident by the presence of positive hemolytic markers such as indirect
hyperbilirubinemia, elevated lactate dehydrogenase (LDH),
hemoglobinuria, severe reticulocytopenia and decreased serum
haptoglobin. HS is categorised into acute and delayed forms. The acute
form occurs within the first 7 days of red cell transfusion meanwhile
the delayed form occurs more than 7 days post red cell
transfusion3. HS rarely recurs. Here, we describe the
possible mechanisms involved in a transfusion dependent beta thalassemia
major patient presenting with acute hyperhemolysis syndrome after red
cell transfusion.