Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

Table 1

Studies on pulmonary Langerhans cell histiocytosis with extrapulmonary manifestations.

Authors	Number of pts.	Pathological confirmation of PLCH	Extrapulmonary sites	Treatment	Outcome
Vassallo; et al. [5, 11]	17	Surgical or transbronchoscopic lung biopsy	Pituitary = 9, bone = 7, skin = 4, lymph node or liver = 4	Prednisone + chemotherapy (vinblastine 7 pts., methotrexate, cyclophosphamide, etoposide, and cladribine 2 pts., respectively	Mixed results with improvement and refractory cases-overall survival shorter than for aged-matched healthy individuals
Karpathiou et al. [12]	1	Thoracoscopic biopsy	Bone: right humerus-biopsy revealed LCH	Smoking cessation	Resolution of the bone without intervention and once patient stopped smoking pulmonary manifestation resolved
Shih et al. [13]	1	Lung biopsy	Bone: skull and one rib discovered on imaging using Technetium-99m	Not reported	Unknown
Nakamura et al. [14]	1	Open lung biopsy	Pituitary: panhypopituitary confirmed on endocrinology testing	Smoking cessation and subsequently methylprednisolone pulse therapy	Disappearance of the pulmonary findings, but patient remained with panhypopituitary dysfunction
Medoff et al. [15]	1	Video assisted surgical biopsy	Bone: left scapular spine and left femur	Smoking cessation + corticosteroid injections in the bone lesions	Almost complete resolution on CT chest 3 months later; bone X-ray 9 months later showed healing with callus formation