title: High-dose chemotherapy and autologous bone marrow transplantation in relapsing angioimmunoblastic lymphadenopathy with dysproteinemia (AILD).
abstract: Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) or lymphogranulomatosis X is a lymphoproliferative disorder with a histological picture resembling that of reactive lesions but with frequent cytogenetic and molecular abnormalities characteristic of malignant T cell lymphoma. Clinically, the disease runs a fatal course in the majority of patients although occasional spontaneous remissions have been observed. Median survival approaches only 1 year even with the most effective treatment protocols implemented so far. Fewer than 20% of patients survive 5 years after diagnosis and cure seems exceedingly rare. High-dose chemotherapy (HDCT) followed by autologous bone marrow transplantation (ABMT) represents a promising new treatment modality for patients with advanced lymphoma conceivably including AILD. We report the first patient with relapsed AILD successfully treated by HDCT and ABMT. This 21-year-old male is alive and free of disease 27 months after ABMT with a Karnofsky score of 100%.
authors.affiliation: Department of Internal Medicine II, Christian-Albrechts-University Kiel, Germany.
authors.forename: N
authors.initials: N
authors.lastname: Schmitz
authors.forename: E
authors.initials: E
authors.lastname: Prange
authors.forename: T
authors.initials: T
authors.lastname: Haferlach
authors.forename: H
authors.initials: H
authors.lastname: Griesser
authors.forename: R
authors.initials: R
authors.lastname: Sonnen
authors.forename: B
authors.initials: B
authors.lastname: Schlegelberger
authors.forename: S
authors.initials: S
authors.lastname: Claus
authors.forename: H
authors.initials: H
authors.lastname: Löffler
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001796:Blood Protein Disorders / Q000150:complications* / Q000378:metabolism; D016026:Bone Marrow Transplantation; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D004317:Doxorubicin / Q000008:administration & dosage; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000150:complications / Q000378:metabolism / Q000628:therapy*; D008297:Male; D011241:Prednisone / Q000008:administration & dosage; D012008:Recurrence; D014182:Transplantation, Autologous; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000970:Antineoplastic Agents; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone