Hopital Avicenne, 93000 Bobigny, France.
Haematologica. 2011 Dec;96(12):1866-9. doi: 10.3324/haematol.2011.050005. Epub 2011 Sep 20.
Patients with essential thrombocythemia often complain of various subjective neurological symptoms. This prospective study aims to assess their incidence and response to therapy. Among 37 consecutive patients with essential thrombocythemia, 11 presented with neurological symptoms. Among them 4 had thrombotic events, 7 complained of transient or fluctuating subjective symptoms, and one had both. Brain magnetic resonance imagery failed to detect any substratum in patients with subjective symptoms. JAK2V617F mutation was found in 9 of 11 patients with neurological symptoms versus 14 of 26 patients without symptoms. Ten patients received low-dose aspirin for these symptoms: complete resolution was observed in 3, improvement with persisting episodes in 2, and resistance to aspirin in 2 patients, in whom addition of cytoreductive therapy became necessary to resolve those disabling symptoms. In this prospective cohort, 30% of patients with essential thrombocythemia presented neurological symptoms. Aspirin was fully efficient in only 30% of cases. JAK2V617F mutation could be a risk factor for such symptoms.
原发性血小板增多症患者常诉有各种主观神经系统症状。本前瞻性研究旨在评估其发生率及对治疗的反应。37 例连续原发性血小板增多症患者中，11 例有神经系统症状。其中 4 例有血栓事件，7 例有短暂或波动的主观症状，1 例兼有这两种情况。脑磁共振成像未能发现有任何基础病变存在于有主观症状的患者中。有神经系统症状的 11 例患者中有 9 例存在 JAK2V617F 突变，而无症状的 26 例患者中有 14 例存在 JAK2V617F 突变。10 例患者因这些症状接受了低剂量阿司匹林治疗：3 例完全缓解，2 例持续发作但有改善，2 例对阿司匹林耐药，这 2 例患者需要添加细胞减少治疗才能缓解这些致残症状。在本前瞻性队列中，30%的原发性血小板增多症患者有神经系统症状。阿司匹林在仅有 30%的病例中完全有效。JAK2V617F 突变可能是这些症状的一个危险因素。
