Dr. T. Tóth szerk.: Studia historico-anthropologica (Anthropologia Hungarica 21. Budapest, 1990)

1. Geographie distribution Thalassaemia major (Cooley-anaemia) and thalassaemia minor developed in the Northern Mediterranean region and they are endemic there (ANGEL 1966, 1967). They are quite common in Italy, in Greece, in Turkey and in Bulgaria. Their frequencies decrease in parallel with the growth of the distance from this area. No Cooley-anaemia was reported with Hungarian citizens during the last 60 years (HOLLÂN 1973). (COOLEY described this disease in 1925.) Sickle-cell anaemia (drepanocytaemia, haemoglobin C disease) is characteristic for black African peoples. 5-10% of the black populations of Middle-, East- and West-Africa have sickle-shaped red blood cells and 10% of them is affected with severe anaemia. None of these diseases occurred in pre-Columbian America and in the northern parts of Eurasia. We may practically disregard these clinical patterns in the differential diagnosis of Hungarian skeletal material as only sporadic isolated cases or disease affected families were found in Hungary. Up to the present only a single Cooley-anaemia case came to light from a Roman cemetery in Pécs (SZÁLA11986). 2. Family relations Congenital haemolithic anaemias may have dominant (Cooley-anaemia, sickle-cell anaemia, elliptocytosis familiáris) or recessive inheritance. The hereditary character of the basic disease must be considered when family relations can be established within a cemetery, and PH occurs cumulatively in the members of a given family. Pyruvat-kinase enzyme deficiency symptom is also a hereditary disease, it is very rare in the recent Hungarian population but we must not disregard it when analyzing skeletalized material. When this enzyme deficiency is cumulatively present in a family, at least half of the children becomes afflicted. Due to enzyme deficiency anaemia PH develops in them. PH occurs almost as a rule with each individual whose anaemia was caused by pyruvat-kinase enzyme deficiency but postcranial alterations are usually absent in these cases. 3. Immunological examinations Haemoglobin's protein component (apohaemoglobin) has two different chains (alpha and beta). The two chains have different immunological character and both are antigenic. Therefore, not only the quality but the quantity of fossil bone's haemoglobin content can be detected by immunochemical methods (ASCENZI & al. 1985). Even the haeomoglobin content of 15-20,000 years old bone material can be determined. The haemoglobin content of bones is proportional to the grade Of erythropoesis. Therefore, an increased haemoglobin content may indicate hyperactive erythropoesis even before their morphological manifestation on bones. 4. Trace element analysis of bones Iron deficiency anaemia is probably the most frequent etiology of PH. The iron content of bones could be an excellent marker of iron deficiency. Though soil can affect the iron content of fossil bones trace element analysis is convenient to determine the etiology of PH on bones from the same mie'roenvironment. Atomic absorption spectrophotometric analysis of normal bones always detected iron contents larger than 800 ppm while iron deficiency samples always had smaller than 700 ppm values (usually 200-400 ppm) (FORNICARI & al. 1981). Iron deficiency anaemia can be excluded as an etiological factor when gross morphology indicates the presence of PH but the bone has normal iron contents. Larger than normal iron contents could indicate even a hyperglobulia based on congenital vitium and polycythemia vera.