Original Article2-Chlorodeoxyadenosine Therapy for Disseminated Langerhans Cell Histiocytosis
Section snippets
PATIENTS AND METHODS
Between December 1994 and January 2001, 5 patients with biopsy-proven disseminated LCH were consecutively treated with 2-CDA at the Mayo Clinic in Rochester, Minn. We retrospectively reviewed their records to assess for clinical response to 2-CDA therapy, after obtaining approval from the Mayo Foundation Institutional Review Board. The ages of the 3 men and 2 women ranged from 19 to 81 years, and the median pretreatment duration of disease was 23 months (range, 4-24 months). In one patient,
Case 1
A 38-year-old man presented with a 2-week history of progressive headaches. Magnetic resonance imaging of the brain showed an irregular, enhancing mass involving the right caudate nucleus with considerable surrounding edema associated with mass effect and midline shift (Figure 1). Pathologic review of tumor tissue obtained by stereotactic biopsy confirmed LCH (Figure 2). The patient was given high-dose corticosteroids, subsequently underwent primary external beam radiation therapy (2.035 Gy in
DISCUSSION
For patients with LCH who have refractory or relapsed disease or those with widespread multiorgan involvement and rapid progression of disease, new and effective therapies are necessary.
Among antimetabolites, 2-CDA is novel in that it appears to have activity against both resting and actively dividing lymphocytes.12 Its ability to induce apoptosis in lymphocyte populations with low growth fractions is thought to underlie its substantial clinical activity in indo lent lymphoproliferative
CONCLUSION
The developing opinion is toward the use of 2-CDA for refractory or relapsed LCH or even as first-line therapy for patients with high-risk disease, although the latter practice is not universally accepted because of the potential for acute and long-term toxic effects associated with this agent. 2-Chlorodeoxyadenosine is potently immunosuppressive, and its incorporation into DNA renders it potentially mutagenic.8 Sustained follow-up of patients is required for an accurate assessment of the
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2018, Journal of the American Academy of DermatologyCitation Excerpt :Combination vinblastine/prednisone is largely ineffective in adults, and no chemotherapy protocols have been successful enough in randomized, double-blind trials to enable guideline development for adult LCH.35 Cladribine36 and the MACOP-B (methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin)37 chemotherapy protocol have shown promising results. Ongoing chemotherapeutic trials for pediatric and adult LCH are shown in Supplemental Table III (available at http://www.jaad.org).
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2016, Clinics in Chest MedicineCitation Excerpt :Chemotherapeutic agents have been used in patients with progressive disease or multisystem involvement.50–53 Although few efficacy data are available, in several case reports and small series, cladribine has been reported to induce radiographic improvement or partial clinical remission of both isolated pulmonary and systemic LCH.50–56 Further study of longitudinal toxicity from this treatment is warranted.
Langerhans cell histiocytosis in adults
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