NDLI: Successful Immunomodulatory Therapy in Castleman Disease with Paraneoplastic Pemphigus Vulgaris

Content Provider	Springer Nature Link
Author	Miltenyi, Zsofia Toth, Judit Gonda, Andrea Tar, Ildiko Remenyik, Eva Illes, Arpad
Copyright Year	2008
Abstract	Castleman disease is a rare lymphoproliferative disorder. The clinical signs and symptoms of the disease are primarily mediated by cytokines, especially interleukin-6. We presented the case of a young female. In May 2004, a 30-year-old otherwise healthy looking woman presented with oral ulcerations resistant to topical and systemic antibiotic and antimycotic treatment. Bullous mucosal lichen or pemphigus vulgaris were suspected. Histological examination and direct and indirect immunofluorescence confirmed the diagnosis of pemphigus. Search for neoplasm revealed a retroperitoneal Castleman tumour sized 15 × 6 × 5 cm in the abdominal MRI. The tumour was a bleeder, so the removal was partial. Histological examination showed hyalin hypervascular Castleman disease. Considering her young, fertile age and the multicentric Castleman disease, non-cytostatic immunomodulatory therapy was started including steroid, cyclosporine-A and thalidomide treatment. The control abdominal CT showed a small residual tumour on the bladder. The residual tumour was removed in repeated surgery. At this time the histological examination showed transient type tumour between plasma cell and vascular variant. Currently, i.e. 4 years after the onset of the disease. $^{18}$FDG PET/CT examination showed low metabolic active mass in the right iliacal region, but our patient had no symptoms or complaints. She is on 200 mg thalidomide a day and no tumour progression can be seen. Castleman disease can be successfully treated with non-cytostatic immunomodulatory therapy.
Starting Page	375
Ending Page	381
Page Count	7
File Format	PDF
ISSN	12194956
Journal	Pathology & Oncology Research
Volume Number	15
Issue Number	3
e-ISSN	15322807
Language	English
Publisher	Springer Netherlands
Publisher Date	2008-12-10
Publisher Place	Dordrecht
Access Restriction	Subscribed
Subject Keyword	Castleman disease Immunmodulatory PET/CT Biomedicine general Immunology Pathology Oncology Cancer Research
Content Type	Text
Resource Type	Article
Subject	Cancer Research Pathology and Forensic Medicine Oncology

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