Isolated Cardiac Involvement in Rosai-Dorfman-Destombes Disease Causing Obstructive Shock.

ABSTRACT:

Background

Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder that classically presents with painless massive lymphadenopathy; however, extranodal disease can occur.

Case summary

A 63-year-old patient was incidentally found to have a soft tissue mass in the left atrium on computed tomography imaging during work-up for atrial flutter. After open surgery to obtain tissue diagnosis, the patient developed shock requiring venovenous extracorporeal membrane oxygenation support. The tissue biopsy revealed the diagnosis of RDD, and the patient received rituximab, which led to substantial clinical improvement.

Discussion

We report a case of isolated cardiac RDD resulting in severe cardiac obstruction. Cardiac RDD is very rare, and a histopathologic diagnosis

SUBMITTER: Marinkovic A 

PROVIDER: S-EPMC12539452 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature