Retroperitoneal nodal aplasia, asplenia, chylous effusion and lymphatic dysplasia: an acquired immunodeficiency syndrome?

Two adult patients (one in Italy and the other in the USA) are described with similar findings of paraaortic nodal aplasia, asplenism, multiple serous and chylous effusions, and retroperitoneal lymphatic dysplasia. Although the clinical courses are incomplete, this unusual constellation of signs in the setting of normal peripheral lymph trunks suggest an acquired rather than inborn anomaly and possibly a variant acquired immunodeficiency syndrome.