Background: Treatment of multisystem Langerhans cell histiocytosis (LCH) remains difficult. Various regimens of single and multiagent chemotherapy have been used, but a significant proportion of patients fail to respond to treatment.
Procedure: We have evaluated the use of cyclosporine A (CSA) in a controlled group of patients, who had received a systematic primary therapy (LCH-I). Patients received CSA either as a single agent (10 patients) or in combination with vinblastine, etoposide, prednisolone, and/or antithymocyte globulin (16 patients).
Results: Among the total of 26 patients treated, a single patient developed a complete response and three a partial response, whereas 85% (22 patients) had no response to CSA.
Conclusions: CSA is at best of limited value in the treatment of patients with multisystem LCH, particularly those who had progressive disease while receiving chemotherapy.
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Article Synopsis
- Langerhans cell histiocytosis (LCH) is a rare disorder marked by the abnormal buildup of dendritic histiocytes in tissues, often presenting as single- or multi-system diseases, particularly affecting adults.
- In a reported case, a 46-year-old woman experienced back pain, motor and sensory deficits, and was diagnosed with a compression fracture in the spine, alongside signs of central nervous system and salivary gland involvement.
- The patient underwent surgical intervention that included decompression and a biopsy, confirming the presence of LCH, highlighting the complexity and rarity of adult-onset LCH with extensive system involvement.
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