Successful Treatment of Adult Multisystemic Langerhans Cell Histiocytosis With Psoralen–UV-A, Prednisolone, Mercaptopurine, and Vinblastine

Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare disease with a peak incidence in childhood. There is limited experience with treatment options for adult patients having multisystemic LCH involvement. We report successful treatment of a 70-year-old woman with adult onset of LCH and multisystem disease (diabetes insipidus centralis, bone marrow infiltration, and lung and skin involvement). Observations: A 70-year-old woman with erythematous plaques and papules of the submammary and inguinal skin attended our outpatient clinic and was diagnosed as having LCH. Organ involvement was found in the infundibulum of the pituitary gland, associated with diabetes insipidus centralis, bone marrow infiltration, and several micronodules of the thoracic and lumbar spine and lungs. Based on the Histiocyte Society's LCH-A1 study in adults, the patient was treated for 12 months with a combination of corticosteroids, vinblastine, and mercaptopurine. No major adverse effects were observed. The skin was also treated with a combination of psoralen-UV-A and local corticosteroids. Restaging revealed regression of all clinical symptoms (skin involvement and diabetes insipidus centralis) and regression of organ infiltration (pituitary gland, bone marrow, and lungs). Conclusion: Effective treatment of adult multisystemic LCH disease was achieved using prednisolone, vinblastine, and mercaptopurine, which was well tolerated.

Langerhans cell histiocytosis with multiple spinal involvement

“…Sometimes, the pathologists might be confused by the tiny specimen with both bony destruction and reconstruction [34]. In Brown's study [5], the pathological findings were inconclusive in two, and we had a similar experience.…”

“…Therapy includes observation, immobilization, NSAIDs [7,15,20,28], chemotherapy [12,34], radiotherapy, intralesional infiltration of steroids and surgery [30]. Recent case reports show encouraging results using the tumor necrosis factor-a (TNF-a) antagonist etanercept, the PPAR-c antagonist pioglitazone, 2-Cda22 and the COX-2 antagonist rofecoxib to treat single-system disease [15].…”

“…A search of the MEDLINE databank located 13 studies and totally 50 cases of LCH with multiple spinal lesions [1,3,5,11,13,20,21,23,26,27,[32][33][34][35]. We reported five additional cases and a meta-analysis of all cases was also performed.…”

Antihyperglycemic, Antihyperlipidemic and Antiapoptotic Activities of Micromelum minutum Seeds in Diabetic Rats

“…Therefore when coumarin is supplied from exogenous source as from Micromelum minutum seeds, it is able to do its antioxidant duty and protect the pancreas and decrease serum glucose. These observations were supported by Von Stebut et al [37] who succeeded to obtain a successful treatment of adult multisystemic langerhans cell histiocytosis from coumarin.…”

Successful Treatment of Adult Onset Langerhans Cell Histiocytosis with Multi-Drug Combination Therapy

“…every three weeks and prednisone p.o. 1 mg/kg on days 1-5 every three weeks up to six months) (9). His skin eruptions had completely disappeared by six weeks of treatment.…”