Bittencourt Henrique, Teixeira Junior Antonio Lucio, Glória Ana Beatriz Firmato, Ribeiro Ana Flávia Leonardi Tiburcio, Fagundes Evandro Maranhão
Hematology and Oncology Service, Centro Hospitalar Universitário Sainte-Justine, Universidade de Montreal, Montreal, AC, Canada.
Rev Bras Hematol Hemoter. 2011;33(6):478-80. doi: 10.5581/1516-8484.20110126.
Acute promyelocytic leukemia is potentially a highly curable type of leukemia that usually presents with pancytopenia, coagulopathies and bleeding. We describe a case of an unusual presentation of acute promyelocytic leukemia. A 53 year-old male was admitted complaining of pain and weakness in his legs. He presented at examination a spastic paraparesis with a sensitive level at the eighth thoracic medullar (T8) segment. Magnetic resonance imaging showed a posterolateral extradural mass from T6 through T8 segments with medullar compression. A complete blood count showed anemia, thrombocytopenia and the presence of promyelocytes and blasts. Marrow examination was compatible with the diagnosis of acute promyelocytic leukemia by cytogenetics and polymerase chain reaction for the PML-RARα gene. He was treated with all-trans-retinoic acid therapy plus daunorubicin and presented an all-trans-retinoic acid syndrome. Despite hematological remission, the patient presented neurologic deterioration and had to be treated with radiotherapy (total dose 3000 cGy) of the extradural lesion. The patient evolved with severe sepsis and died without any recovery from his neurologic deficit. Extramedullary infiltration is a very rare complication in acute promyelocytic leukemia. Most cases are related to relapse after initial treatment with all-trans-retinoic acid. The skin and the central nervous system are the most frequently involved sites. This is possibly the first case reported of this condition in which the patient had a symptomatic extradural mass.
急性早幼粒细胞白血病是一种潜在的高治愈率白血病类型，通常表现为全血细胞减少、凝血功能障碍和出血。我们描述了一例急性早幼粒细胞白血病的不寻常表现病例。一名53岁男性因腿部疼痛和无力入院。检查时发现痉挛性截瘫，感觉平面位于胸髓第8节段（T8）。磁共振成像显示T6至T8节段后外侧硬膜外肿块并伴有脊髓受压。全血细胞计数显示贫血、血小板减少以及早幼粒细胞和原始细胞的存在。骨髓检查通过细胞遗传学和PML-RARα基因的聚合酶链反应，与急性早幼粒细胞白血病的诊断相符。他接受了全反式维甲酸治疗加柔红霉素治疗，并出现了全反式维甲酸综合征。尽管血液学缓解，但患者出现神经功能恶化，不得不接受硬膜外病变的放射治疗（总剂量3000 cGy）。患者病情进展为严重脓毒症，最终死于神经功能缺损且未恢复。髓外浸润是急性早幼粒细胞白血病中非常罕见的并发症。大多数病例与全反式维甲酸初始治疗后的复发有关。皮肤和中枢神经系统是最常受累的部位。这可能是首例报道的患者出现有症状硬膜外肿块的这种情况。
