Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents with progressive exertional dyspnea and non-productive cough. History significant for biopsy-proven Erdheim-Chester Disease (ECD). Symptoms include [fatigue/chest pain/weight loss]. No history of smoking or occupational lung exposure. Current functional status is [NYHA Class I-IV].
Clinical Examination Findings
Pulmonary exam reveals [bilateral fine inspiratory crackles/decreased breath sounds at bases]. Cardiac exam shows [regular rate and rhythm/signs of right heart strain]. Skin exam notes [xanthelasma/papular lesions]. Skeletal exam reveals [tenderness over long bones]. O2 saturation is [X]% on room air.
Treatment Protocol
Initiation of targeted therapy with [BRAF inhibitor e.g., Vemurafenib / MEK inhibitor e.g., Cobimetinib] as per molecular profiling. Pulmonary rehabilitation initiated. Monitoring for treatment-related toxicities including [QT prolongation/dermatologic reactions]. Serial PFTs and HRCT chest scheduled for [X] months.