Aggio M C
Servicio de Hematología y Hemoterapia, Hospital Dr. José Penna, Bahía Blanca, Argentina.
Rev Invest Clin. 1990 Apr-Jun;42(2):142-4.
Some enlarged spleens do not seem to be related with known pathogenetic mechanisms (passive congestion, functional workload, malignant infiltration and inflammatory or storage disorders). Non-tropical idiopathic splenomegaly (Dacie's syndrome) is a form of hypersplenism of unknown origin that evolves into a non-Hodgkin lymphoma, after a variable interval, in 20% of the patients. Tropical idiopathic splenomegaly (or hyperreactive malarial splenomegaly) develops when a chronic malarial challenge triggers an abnormal immunological response consisting in decreased suppressor T lymphocytes and increased amounts of circulating immunoglobulin M and immunocomplexes, which are cleared by the splenic macrophages. This peculiar response to malaria seems to be linked to particular HLA antigens. Other confusing splenomegalies are seen in Felty's syndrome, in populations subjected to recurrent infections, and in some families. Overlapping findings and diseases suggest chronic antigenic stimulation as a common feature, with diverse responses depending on the host. A small percentage (probably less than 3%) of normal individuals has minimal splenomegaly without any clinical significance.
一些脾脏肿大似乎与已知的发病机制（被动性充血、功能性负荷、恶性浸润以及炎症或贮积性疾病）无关。非热带特发性脾肿大（达西综合征）是一种病因不明的脾功能亢进形式，在20%的患者中，经过一段可变的间隔时间后会演变成非霍奇金淋巴瘤。热带特发性脾肿大（或高反应性疟疾性脾肿大）是在慢性疟疾感染引发异常免疫反应时发生的，该反应表现为抑制性T淋巴细胞减少，循环免疫球蛋白M和免疫复合物数量增加，这些物质由脾巨噬细胞清除。这种对疟疾的特殊反应似乎与特定的人类白细胞抗原（HLA）有关。在费尔蒂综合征、反复感染人群以及一些家族中也可见到其他令人困惑的脾肿大情况。重叠的发现和疾病提示慢性抗原刺激是一个共同特征，只是宿主的反应各不相同。一小部分（可能不到3%）正常个体有轻微脾肿大，但无任何临床意义。
