Mottaz P, Becot-Bretez F, Jeanneau R, Vignon G, Bertin A, Carrere F, Augereau P F, Aucher P, Lellouche F
Service de médecine interne, centre hospitalier de Royan, 2, rue de St-Sordelin, 17640 Vaux-sur-Mer, France.
Service de médecine polyvalente, centre hospitalier de St-Jean-d'Angély, 18, avenue du Port, 17400 St-Jean-d'Angély, France.
Rev Med Interne. 2017 Nov;38(11):774-777. doi: 10.1016/j.revmed.2017.06.002. Epub 2017 Jun 28.
Myeloid sarcomas are uncommon proliferations of immature myeloid cells occurring in any extramedullary organ. We report here two cases of myeloid sarcomas in patients with, respectively, a polycythemia vera and a myelodysplastic syndrome.
The first is an 81-year-old woman who presented with osteolytic lesions. Diagnosis has been highlighted using anatomopathological study after bone marrow biopsy, but it was delayed because of a very localized basin lesion and few positive myeloid markers. The second patient is an 86-year-old man who presented with pancytopenia and several lymph nodes. Lymph node cytology failed because of the rarity of blast cells. Diagnosis was done after anatomopathological study on lymph node biopsy which revealed a localized form of myeloid sarcoma.
The diagnosis of myeloid sarcoma must be considered when unusual tumors occur in patients with a chronic myeloid disease. In that case, therapeutic options are those of an acute myeloid leukemia.
髓系肉瘤是发生于任何髓外器官的未成熟髓系细胞的罕见增殖性病变。我们在此报告两例髓系肉瘤患者，分别患有真性红细胞增多症和骨髓增生异常综合征。
第一例是一名81岁女性，表现为溶骨性病变。经骨髓活检后的解剖病理学研究明确了诊断，但由于盆腔病变非常局限且髓系标记物阳性较少，诊断有所延迟。第二例患者是一名86岁男性，表现为全血细胞减少和多处淋巴结肿大。由于原始细胞罕见，淋巴结细胞学检查未能明确诊断。经淋巴结活检的解剖病理学研究后确诊为局限性髓系肉瘤。
当慢性髓系疾病患者出现不寻常肿瘤时，必须考虑髓系肉瘤的诊断。在这种情况下，治疗方案与急性髓系白血病相同。
