Arterial elastorrhexis in β‐thalassaemia intermedia, sickle cell thalassaemia and hereditary spherocytosis

European Journal of Haematology - Tập 67 Số 3 - Trang 135-141 - 2001
K. Tsomi1, Markissia Karagiorga‐Lagana2, Fotis Karabatsos2, Christina Fragodimitri2, C. Van Vliet‐Konstantinidou3, Evangelos Premetis4, Alexandra Stamoulakatou4
1Department of Nephropathology, Evangelismos General Hospital, Athens;
2Thalassaemia Unit;
3Department of Pathology
4Laboratory of Haematology, Aghia Sophia Children's Hospital, Athens, Greece

Tóm tắt

Abstract: Arterial and stromal elastorrhexis, an elastic tissue disorder, was recently described in β‐thalassaemia major. Histopathological material from 10 patients with thalassaemia intermedia, 14 with sickle cell thalassaemia and 18 with hereditary spherocytosis was examined in order to investigate the specificity of the arteriopathy. Histological re‐examination was made in a total of 42 spleens with parasplenic lymph nodes in 14 cases, 26 surgical liver biopsies and 16 gallbladders with associated regional lymph nodes. Arteriopathy, qualitatively similar to that seen in β‐thalassaemia major, was found in up to 90% of extrasplenic muscular arteries. Elastorrhexis lesions were also found in intrasplenic arteries and in stromal elastic tissue of spleens and parasplenic lymph nodes, in the absence of tissue iron overload. The arteriopathy appears in the first decade of life even in spleens of normal weight, and seems unrelated to the severity of permanent anaemia. It is suggested that patients suffering from hereditary chronic haemolytic diseases are subject to an elastic tissue disorder which is similar to hereditary pseudoxanthoma elasticum, the earliest and most frequent manifestation of which is arterial elastorrhexis of muscular extrasplenic arteries.

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Thông tin xuất bản

European Journal of Haematology

Tập 67 Số 3

135-141

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