Unstable Hemoglobin Disease

Unstable hemoglobin disease results from amino-acid substitutions in alpha or beta globin chains that destabilize the hemoglobin molecule; it denatures and precipitates as intracellular Heinz bodies (denatured globin inclusions), called unstable hemoglobin.

Common Symptoms

Hemolytic anemia; jaundice; splenomegaly; dark urine

Causes and Pathogenesis

Point mutations in globin genes alter amino-acid sequence, destabilizing heme-globin contact. Oxidant stress triggers inclusion formation, RBC-membrane damage and hemolysis.

Treatment

Supportive therapy; avoid oxidant drugs

Preventive Measures

Avoid sulfonamides, antimalarials and other oxidant drugs; folate supplementation; genetic counseling.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.