Langerhans cell histiocytosis (LCH) and Rosai Dorfman disease (RD), also known as sinus histiocytosis with massive lymphadenopathy, may cause pain by expansive or erosive lesions in bones. Sometimes patients have pain from residual structural defects in bones that do not heal completely with therapy. It has been observed that teenage and adult LCH/RD patients may have persistent pain when no active lesions can be found by bone scans or standard radiographic techniques. The etiology of this pain is unknown, but one may suspect cytokines, such as tumor necrosis factor alpha, interleukin-1, interferon gamma, or others could be involved.

Clearly, this problem needs to be addressed prospectively as a research question in current and future Histiocyte Society protocols. However, at this time it is a recognized complication of LCH/RD that should be fully evaluated and treated with appropriate anti-pain medications which may include nonsteroidal anti-inflammatory agents or narcotics. This is done most successfully by an established doctor/patient relationship in person, not over the internet.

Consultation with an expert in pain management may be indicated.

Kenneth McClain, MD, PhD

Professor of Pediatrics

Texas Children's Hospital

Houston, Texas

Robert Arceci, MD

Director, Pediatric Oncology

Johns Hopkins Oncology Center

Baltimore, Maryland

Maurizio Arico, MD

Ospedale dei Bambini

Oncoematologia Pediatrica

Palermo, Italy

R. Maarten Egeler, MD, PhD

Leiden University Medical Center

Leiden, The Netherlands