Case Report: Multicentric Reticulohistiocytosis Associated With Posterior Mediastinal Adenosquamous Carcinoma, Antinuclear Antibody Positivity and Lupus Anticoagulant Positivity

Abstract

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of non-Langerhans cell histiocytosis. A number of studies in the literature have documented that it can coexist with malignancy or autoimmune disease, making it difficult to determine the most appropriate therapy. Here, we present a case study of MRH associated with posterior mediastinal adenosquamous carcinoma along with antinuclear antibody positivity and lupus anticoagulant positivity. The patient experienced 6 months of clinical benefit after surgical resection and chemoradiotherapy of the mediastinal malignancy. This case adds to the available literature on multicentric reticulohistiocytosis associated with different types of malignancy and provides supplementary clinical data on the coexistence of this syndrome with malignancy and immune system abnormalities. To the best of our knowledge, this is the first case study describing MRH accompanied by posterior mediastinal adenosquamous carcinoma and lupus anticoagulant positivity. The unknown aetiology and polymorphic clinical presentation of MRH warrants further investigation.

Multicentric Reticulohistiocytosis Presenting As Acute Pericarditis With Moderate-Sized Pericardial Effusion: A Case Report and Review of Multicentric Reticulohistiocytosis Treatment

“…The similarity of clinical manifestations between MRH and rheumatologic conditions is proposed to be secondary to autoimmune or inflammatory pathology. Hence, the treatment with systemic glucocorticoids, methotrexate, bisphosphonates, and biologic agents are being employed with varied outcomes [ 11 ]. Prednisone can be started at a dose of 7.5 - ≤30 mg/day and then tapered to ≤7.5 mg/day based on the disease activity [ 12 ].…”