[Lymphomatoid granulomatosis, polymorphic reticulosis and angioimmunoblastic lymphadenopathy with pulmonary...

This study attempts to demonstrate that typical lymphomatoid granulomatosis, polymorphic reticulosis and angioimmunoblastic lymphadenopathy with pulmonary involvement, are three related anatomo-clinical conditions. Three groups of patients with clinical and pathological signs corresponding to each of these three conditions are studied. In group A, the clinical, radiographic and histologic data for 11 patients presenting "classical lymphomatoid granulomatosis" are reported. In this group, all subjects presented a poor general condition, with fever and immunological disorders (hypo- or hypergammaglobulinemia, often monoclonal gammopathy). Chest X-rays always showed bilateral, nodular, poorly defined lung infiltrates. Most patients have had rash and/or dermal infiltrates and ENM involvement. Some of them developed liver and/or spleen, and/or lymph node enlargement. Whatever the localization of the lesions, there were angiocentric polymorphic granulomas with atypical cells spread between lymphocytes, plasma cells and rare polymorphonuclear leucocytes. In group B, the clinical, radiographic and biological data for 4 patients presenting a so-called polymorphic reticulosis are reported. All subjects presented ENM involvement; only one had a poor general condition and hypogammaglobulinemia. Only one patient presented a lung involvement. Histologically the lesions were angiocentric granulomas composed of numerous lymphocytes, plasma cells intermingled with some polymorphonuclears and numerous characteristic large atypical cells. In group C, the clinical, radiographic and biological data for 3 out of 26 cases diagnosed as angioimmunoblastic lymphadenopathy (AIL) are summarized. These 3 cases were selected because of presence of lung involvement; all three had a typical AIL with fever, poor general condition, rash, hemolytic anemia (Coombs +) and lymph node enlargement. All of them presented bilateral, nodular, poorly defined lung infiltrates. Lymph node lesions were typical for AIL: destruction of follicular structure, vascular proliferation and numerous immunoblasts. Lesions in other organs (mainly lung, dermal and ENM lesions) were characterized by angiocentric granulomas containing numerous blast cells. The biological behavior with eventual development of a monoclonal gammopathy, associated or not with a malignant immunoblastic lymphoma, as well as resemblance of clinical and histological manifestations during the course of the disease, suggest that these three conditions represent variants of a basically identical entity.