title: Mediastinal Germ Cell Tumor-associated Histiocytic Proliferations Treated With Thalidomide Plus Chemotherapy Followed by Alemtuzumab-containing Reduced Intensity Allogeneic Peripheral Blood Stem Cell Transplantation: A Case Report.
abstract: Mediastinal nonseminomatous germ cell tumor (MNSGCT)-associated histiocytic proliferations are rare and rapidly fatal disorders. Standard treatment modalities have yet to be established.We report a case of MNSGCT-associated hemophagocytic syndrome that evolved into malignant histiocytosis/disseminated histiocytic sarcoma (MH/HS), which was initially treated with intravenous immunoglobulin, corticosteroids, and cyclosporine. Then, thalidomide plus cyclophosphamide, adriamycin, oncovin, prednisolone chemotherapy followed by alemtuzumab-containing reduced-intensity allogeneic peripheral blood stem cell transplantation (PBSCT) was used as salvage therapy.The severe constitutional symptoms and pancytopenia resolved shortly after thalidomide with cyclophosphamide, adriamycin, oncovin, prednisolone. After PBSCT, the patient developed steroid-dependent skin graft-versus-host disease, but maintained a functional life for 1.5 years. Rapid resolution of chronic graft-versus-host disease preceded the fulminant recurrence of hemophagocytic syndrome and MH/HS.Thalidomide plus chemotherapy followed by alemtuzumab-containing reduced intensity allogeneic PBSCT is effective in allaying MNSGCT-associated histiocytic disorders, but does not prevent eventual relapse. However, further posttransplant immune modulation should be developed to completely eradicate the residual MH/HS cells.
authors.affiliation: From the Department of Pharmacy, Koo Foundation Sun Yat-Sen Cancer Center, Taipei, Taiwan (L-HF); Department of Pathology, Koo Foundation Sun Yat-Sen Cancer Center, Taipei, Taiwan (L-SH); Department of Nuclear Medicine, Koo Foundation Sun Yat-Sen Cancer Center, Taipei, Taiwan (P-IL); Department of Internal Medicine, Koo Foundation Sun Yat-Sen Cancer Center, Taipei, Taiwan (W-TC); and Department of Pediatric Hematology and Oncology, Koo Foundation Sun Yat-Sen Cancer Center, Taipei, Taiwan (R-LC).
authors.forename: Li-Hua
authors.initials: LH
authors.lastname: Fang
authors.forename: Li-Sun
authors.initials: LS
authors.lastname: Shih
authors.forename: Pei-Ing
authors.initials: PI
authors.lastname: Lee
authors.forename: Wei-Ting
authors.initials: WT
authors.lastname: Chen
authors.forename: Rong-Long
authors.initials: RL
authors.lastname: Chen
mesh_terms: D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D017809:Fatal Outcome; D054747:Histiocytic Sarcoma / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008479:Mediastinal Neoplasms / Q000150:complications* / Q000188:drug therapy; D009373:Neoplasms, Germ Cell and Embryonal / Q000150:complications* / Q000188:drug therapy; D036102:Peripheral Blood Stem Cell Transplantation; D013792:Thalidomide / Q000627:therapeutic use; D014184:Transplantation, Homologous; D055815:Young Adult
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D000074323:Alemtuzumab; D013792:Thalidomide