Postiglione K, Ferris R, Jaffe J P, Stroncek D
Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, USA.
Am J Hematol. 1995 Aug;49(4):336-40. doi: 10.1002/ajh.2830490412.
Thymoma has been associated with a variety of autoimmune disorders. We report a case of agranulocytosis and anemia in a 68-year-old woman with a spindle cell thymoma. She was unresponsive to treatment with antibiotics, granulocyte-colony stimulating factor (G-CSF), prednisone, and high-dose intravenous immunoglobulin. Serial bone marrow examinations on this therapy showed progression from a cellular marrow with mild myeloid and erythroid hyperplasia and lymphocytosis, to granulocyte aplasia and severe erythroid hypoplasia. Her serum contained granulocyte-specific antibodies and inhibited the growth in culture of her own marrow cells and marrow cells from a normal donor. An IgG fraction from her serum also inhibited the growth of marrow cells. Although the patient's spindle cell thymoma was surgically removed, she remained neutropenic. She was treated with six plasma exchanges followed by 1,000 milligrams of intravenous cyclophosphamide 2 days after the final plasma exchange and daily G-CSF. Three weeks later her peripheral blood showed marked leukocytosis with pronounced neutrophilia and a left shift. Although her agranulocytosis resolved, she died of fungal sepsis. This case demonstrates that aggressive plasma exchange and immunosuppressive therapy may benefit patients with agranulocytosis associated with thymoma.
胸腺瘤与多种自身免疫性疾病有关。我们报告一例68岁患有梭形细胞胸腺瘤的女性发生粒细胞缺乏症和贫血的病例。她对抗生素、粒细胞集落刺激因子（G-CSF）、泼尼松和大剂量静脉注射免疫球蛋白治疗均无反应。在这种治疗过程中进行的系列骨髓检查显示，骨髓从有轻度髓系和红系增生及淋巴细胞增多的细胞性骨髓，进展为粒细胞缺乏和严重红系发育不全。她的血清中含有粒细胞特异性抗体，并抑制她自身骨髓细胞以及来自正常供体的骨髓细胞在培养中的生长。她血清中的IgG组分也抑制骨髓细胞的生长。尽管患者的梭形细胞胸腺瘤通过手术切除，但她仍为中性粒细胞减少。她接受了6次血浆置换治疗，在最后一次血浆置换后2天给予1000毫克静脉注射环磷酰胺，并每日给予G-CSF。三周后，她的外周血显示明显的白细胞增多，伴有显著的中性粒细胞增多和核左移。尽管她的粒细胞缺乏症得到缓解，但她死于真菌败血症。该病例表明，积极的血浆置换和免疫抑制治疗可能对与胸腺瘤相关的粒细胞缺乏症患者有益。
