Thalassemia

Thalassemia are genetic disorders of Hb synthesis in which there is reduced production of one or more chains of Hb. This results in a relative excess production of either a chains or b chains, which without their partner chains are unstable and precipitate in the red cell or their precursors. The inclusion bodies producd by this process increase the rigidity of the red cell and result in its destruction, either in the marrow or the circulation or both. Hence the anaemia of thalassemia results from ineffective erythropoiesis due to intramedullary red cell destruction and a shortened red cell survival caused by haemolysis. The b thalassemias are more often encountered. Their clinical symptoms are different, as for the b thalassemias severe patients will have severe anemia, jaundice, hepatosplenomegaly, disturbances in growth, mental retardation; typical cases shown the flat nose, increased width between eyebrow, protruded facial bones; regenerative and volume of compensatory cells of bone marrow are increased, shrinkage of size of erythrocytes, low colour index, accompanied with sickle cells syndromes; it is associated with inheritance and ontogeny.
The etiology of this disease is not clearly known, the b thalassemias are autosomal codominant traits.
The main diagnose of this disease in based on anemia and jaundice, lab analysis reveals microcytic hypochromic anemia with sickle cells syndromes, increased capillary network of erytrocytes (gelatinous network of fibrous polymers that stiffen the erythrocytes membrane), increase viscosity and cause dehydration due to potassium leakage and calcium influx.
The modern medicine treatments against this disease are transfusion of blood in severe anemic patients and even apply the spleenectomy (removal of spleen).
In chinese medicine, this disease belongs to xu lao (tiredness) and wang dan (jaundice). The patient will experience anemia since young, Qi (energy) is excessive accumulated thus causing imbalance of blood system, moreover the body system will start to become weaker and resulting in Yang cannot generate Yin and one will lack of Jing (essence) of blood; consequently the inner organs cannot adjust with one another and will cause slightly wetness of the blood Jing , then, causing excessive of bile which is shown on the skin as weakness and jaundice; if Yin Yang of blood Qi is not adequate and at the same time is infected by other diseases, therefore will resulted jin xu (symptoms of Qi), shi (symptoms of damp and jaundice) combination of symptoms.