Topical nitrogen mustard ointment with occlusion for Langerhans' cell histiocytosis of the scalp.

BACKGROUND: Topical nitrogen mustard solution has been used as an effective alternative to corticosteroids for the treatment of cutaneous eruptions of Langerhans' cell histiocytosis (LCH). When used as an ointment under occlusion, nitrogen mustard may still be effective and possess less risk of unwanted side effects. METHODS: A patient with scalp LCH was treated topically with nitrogen mustard ointment 0.01% under occlusion. RESULTS: The lesions cleared in 3 weeks without irritation. CONCLUSION: Topical nitrogen mustard ointment 0.01% under occlusion is a well-tolerated, non-irritating treatment for scalp LCH.

Increased interleukin 5 production in eosinophilic Sézary syndrome: regulation by interferon alfa and interleukin 12.

BACKGROUND: Peripheral eosinophilia occurs in a small subpopulation of patients with cutaneous T-cell lymphoma (CTCL) and denotes a poor prognosis. Clinical studies have suggested that the Sézary cell is a T(H)2 type helper T cell that produces cytokines that enhance the differentiation and activation of eosinophils. Interferon alfa (IFN-alpha) and interleukin 12 are effective therapeutic agents for CTCL and other hematologic disorders. OBJECTIVE: Our purpose was to determine the inhibitory activity of IFN-alpha and IL-12 on IL-5 production in vitro by peripheral blood mononuclear cells (PBMCs) obtained from patients with CTCL and eosinophilia. METHODS: Suppression of IL-5 production by IFN-alpha and IL-12 was assessed by comparing IL-5 production by PBMCs from patients with Sézary syndrome and eosinophilia when cultured alone or in the presence of either IFN-alpha or IL-12. RESULTS: A marked increase in IL-5 production by PBMCs from patients with Sézary syndrome and eosinophilia was observed. IL-5 production was markedly reduced when PBMCs were exposed to IFN-alpha or IL-12. CONCLUSION: These results suggest that IFN-alpha and perhaps IL-12 may produce a therapeutic response in patients with CTCL and eosinophilia through direct suppression of IL-5 production by malignant Sézary cells.

Extracorporeal photochemotherapy does not suppress T- or B-cell responses to novel or recall antigens.

BACKGROUND: Extracorporeal photopheresis (ExP) is an effective therapy for several conditions including cutaneous T-cell lymphoma, scleroderma, and allograft rejection. Experimental animal models suggest that ExP may induce antigen-specific immunosuppression. OBJECTIVE: Our purpose was to determine the effect of photopheresis on humoral and cell-mediated immunity in human subjects. METHODS: Recall and primary immune responses of patients with scleroderma receiving monthly ExP treatments were assessed by delayed type hypersensitivity skin tests, T-cell proliferative responses after immunizations with tetanus toxoid and keyhole limpet hemocyanin, and serum antibody titers against common viral pathogens. RESULTS: After 6 months of ExP, viral antibody titers and delayed type hypersensitivity responses were not significantly different from baseline values in all 7 patients tested. T-cell responses to tetanus toxoid remained normal in 3 of 3 patients tested for a minimum of 6 months after booster immunization. Immunization with the protein antigen keyhole limpet hemocyanin after initiation of ExP therapy resulted in sustained T-cell proliferative responses up to 6 months in 3 of 3 patients. CONCLUSION: These results, along with the observation of no increased incidence of opportunistic infections or neoplasms, suggest that ExP is not broadly immunosuppressive and does not prevent primary responses to vaccination or other antigenic challenges.

Photopheresis: clinical applications and mechanism of action.

Photopheresis is a leukapheresis-based therapy that utilizes 8-methoxypsoralen and ultraviolet A irradiation. Photopheresis is currently available at approximately 150 medical centers worldwide. Recent evidence suggests that this therapy used as a single agent may significantly prolong life, as well as induce a 50%-75% response rate among individuals with advanced cutaneous T cell lymphoma (CTCL). Furthermore, a 20%-25% complete response rate with photopheresis alone, or in combination with other biologic response modifiers, has been obtained at our institution among patients with Sezary syndrome. These complete responses have been characterized by the complete disappearance of morphologically atypical cells from the skin and blood. The use of sensitive molecular techniques has also confirmed the sustained disappearance of the malignant T cell clone from the blood of patients with complete responses. In addition to the treatment of CTCL, numerous reports indicate that photopheresis is a potent agent in the therapy of acute allograft rejection among cardiac, lung, and renal transplant recipients. Chronic graft versus host disease also appears to be quite responsive to photopheresis therapy. Likewise, there may also be a potential role for photopheresis in the therapy of certain autoimmune diseases that are poorly responsive to conventional therapy. The immunologic basis for the responses of patients with these conditions is likely due to the induction of anticlonotypic immunity directed against pathogenic clones of T lymphocytes. Treatment-induced apoptotic death of pathogenic T cells and activation of antigen presenting cells are postulated to have important effects in this therapeutic process.