Fuentes A, Feliu E, Blade J, Campo E, Ribera J M, Cervantes F, Rozman C
Servicio de Hematología y Hemoterapia, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona.
Med Clin (Barc). 1996 Feb 24;106(7):263-6.
Two female patients of 54 and 48 years, respectively, with pancytopenia and splenomegaly of probable immune origin are reported. The first presented anemic syndrome and fever, and the second bleeding. In both cases large sized splenomegaly was found on physical examination. Serologic and immunologic studies excluded chronic infection or systemic autoimmune disease. Bone marrow biopsy showed hematopoietic hyperplasia with lymphoid nodules and reticulin fibrosis. Treatment with glucocorticoids and splenectomy was performed in one case and only glucocorticoids were administered in the other case with the cytopenias disappearing and the bone marrow becoming normocellular. Reactive follicular hyperplasia was observed in the white pulp of the spleen in one of the patients, as were abundant macrophagic activity in the red pulp, and myeloid metaplasia. The patients remain asymptomatic at 4 and 12 years of follow up. The origin of the process was attributed probably to an immune etiology and differential diagnosis with idiopathic myelofibrosis and non tropical idiopathic splenomegaly must be considered.
报告了两名分别为54岁和48岁的女性患者,她们患有全血细胞减少症和可能由免疫因素引起的脾肿大。第一名患者表现为贫血综合征和发热,第二名患者有出血症状。在这两个病例中,体格检查均发现脾脏肿大。血清学和免疫学研究排除了慢性感染或全身性自身免疫性疾病。骨髓活检显示造血增生伴淋巴小结和网状纤维组织增生。其中一例患者接受了糖皮质激素治疗和脾切除术,另一例仅给予糖皮质激素治疗,血细胞减少症消失,骨髓恢复正常细胞状态。在其中一名患者的脾脏白髓中观察到反应性滤泡增生,红髓中巨噬细胞活性丰富,还有髓外化生。在4年和12年的随访中,患者均无症状。该病程的起源可能归因于免疫病因,必须考虑与特发性骨髓纤维化和非热带特发性脾肿大进行鉴别诊断。