Michiels J J, Van Genderen P J
European Working Group on Myeloproliferative Disorders, Goodheart Institute, Rotterdam, The Netherlands.
Semin Thromb Hemost. 1997;23(3):295-301. doi: 10.1055/s-2007-996102.
In addition to the criteria of the Polycythemia Vera Study Group, positive markers for essential thrombocythemia (ET) include spontaneous BFU-E, splenomegaly, and megakaryocyte morphology in bone marrow smears and biopsy material. The hematologic features of 11 reported cases of ET in childhood showed platelet counts in excess of 1000 x 10(9)/L in all, slight leukocytosis in 8, and splenomegaly in 9. The presenting thrombohemorrhagic manifestations in 8 symptomatic cases were microcirculatory disturbances and transient neurologic ischemic attacks in 2, recurrent mucocutaneous bleedings in 6, and priapism in 1. There are no reports of ET in childhood complicated by microcirculatory disturbances at platelet counts below 1000 x 10(9)/L. Anagrelide and alpha-interferon, which are non-leukemogenic agents for the reduction of platelet counts, may become the treatment of choice in childhood ET. Anagrelide is tolerated better than alpha-interferon. The potential leukemogenic drugs hydroxyurea and busulfan should be used cautiously and withheld as long as possible.
除真性红细胞增多症研究组的标准外，原发性血小板增多症（ET）的阳性标志物还包括自发的爆式红系集落形成单位（BFU-E）、脾肿大以及骨髓涂片和活检材料中的巨核细胞形态。11例儿童ET报告病例的血液学特征显示，所有病例血小板计数均超过1000×10⁹/L，8例有轻度白细胞增多，9例有脾肿大。8例有症状病例出现的血栓出血表现为：2例有微循环障碍和短暂性神经缺血发作，6例有反复的黏膜皮肤出血，1例有阴茎异常勃起。尚无儿童ET在血小板计数低于1000×10⁹/L时并发微循环障碍的报告。阿那格雷和α干扰素是降低血小板计数的非致白血病药物，可能成为儿童ET的治疗选择。阿那格雷的耐受性优于α干扰素。潜在的致白血病药物羟基脲和白消安应谨慎使用，并尽可能避免使用。
