Chronic eosinophilic leukemia-not otherwise specified has a poor prognosis with unresponsiveness to conventional treatment and high risk of acute transformation
Chronic eosinophilic leukemia-not otherwise specified has a poor prognosis with unresponsiveness to conventional treatment and high risk of acute transformation
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DOI：
10.1002/ajh.23193
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发表时间：
2012-06-01
影响因子：
12.8
通讯作者：
Kyrcz-Krzemien, Slawomira
中科院分区：
文献类型：
--
作者：
Helbig, Grzegorz;Soja, Anna;Kyrcz-Krzemien, Slawomira
慢性嗜酸性粒细胞白血病-未另行说明（CEL-NOS）是一种罕见的疾病，具有嗜酸性粒细胞增多和血液或骨髓原始细胞数量增加（< 20%）或嗜酸性粒细胞克隆性的证据。我们评估了10例CEL-NOS患者的临床结局。纳入了7名男性和3名女性，中位年龄为62岁（范围：23-73岁）。诊断时中位白细胞计数为33.4 3109/l（范围：9.3-175.0），中位嗜酸性粒细胞计数为15.6 3109/l（范围：1.5-136.0）。中位血红蛋白和血小板分别为11.0 g/dl（范围：8.3-13.3）和158 3 109/l（范围：31.0-891.0）。临床表现包括脾肿大（n 5 7）、肝肿大（n 56）、心力衰竭（n 5 2）和肺浸润（n 5 1）。整个队列从诊断到死亡的中位生存期为22.2个月（范围，2.2-186.2）。10例研究患者中有5例在诊断后中位数20个月（范围，1.6-41.9）后发生急性转化（AT）。末次随访时无AT患者存活。AT至死亡的中位时间为2个月（范围，1.0-6.1）。在5例未发生AT的患者中，3例死于活动性疾病。两名患者完全缓解，第一次接受异基因干细胞移植前强化诱导化疗，第二个仍然对伊马替尼与羟基脲。除了后者患者，伊马替尼在我们的研究人群中无效。CEL-NOS是一种罕见的侵袭性疾病，具有高AT率和对常规治疗的抵抗力。
Chronic eosinophilic leukemia-not otherwise specified (CEL-NOS) is a rare disorder with hypereosinophilia and an increased number of blood or marrow blast (< 20%) or an evidence of eosinophil clonality. We evaluated the clinical outcome of 10 patients with CEL-NOS. Seven males and three females at a median age of 62 years (range, 23–73) were included. The median leukocyte count at diagnosis was 33.4 3109/l (range, 9.3–175.0) with a median eosinophil count of 15.6 3 109/l (range, 1.5–136.0). Median hemoglobin and platelets were 11.0 g/dl (range, 8.3–13.3) and 158 3 109/l (range, 31.0–891.0), respectively. Clinical manifestations included splenomegaly (n 5 7), hepatomegaly (n 56), cardiac failure (n 5 2), and lung infiltrations (n 5 1). Median survival from diagnosis to death for entire cohort was 22.2 months (range, 2.2–186.2). Five of the 10 studied patients developed acute transformation (AT) after median of 20 months from diagnosis (range, 1.6–41.9). None of patients with AT is alive at the time of last follow-up. Median time from AT to death was 2 months (range, 1.0–6.1). Among five patients who did not develop AT, three died in active disease. Two patients are alive in complete remission; first underwent allogeneic stem-cell transplantation preceding by intensive induction chemotherapy; the second remains on imatinib with hydroxyurea. Except the latter patient, imatinib was ineffective in our study population. CEL-NOS is a rare and aggressive disease with high rate of AT and resistance to conventional treatment.