High-dose chemotherapy and APSCT as a potential cure for relapsing hemolysing AILD.
Angioimmunoblastic lymphadenopathy with dysproteinemia (or dysgammaglobulinemia) (AILD) is a lymphoproliferative disorder with cytogenetic and molecular abnormalities characteristic of malignant T-cell lymphoma (angioimmunoblastic T-cell lymphoma -- AITL). We report the clinical course of a 58-year-old male patient with unusually aggressive AILD, including severe hemolysis and Guillain-Barré syndrome, who entered complete remission after CHOP therapy, but had a full relapse after 2 months. At relapse, treatment with high-dose chemotherapy followed by autologous peripheral stem cell transplantation (APSCT) with CD34 selected cells was shown to be successful. The patient is alive and disease-free 3 years after diagnosis and 32 months after APSCT. Considering the poor prognosis of the majority of patients with AILD, intensive treatment followed by APSCT, may be a subject for further studies.
Department of Medicine, Division of Hematology, Karolinska Institutet, Huddinge University Hospital, 141 86 Stockholm, Sweden.
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J
Lindahl
E
E
Kimby
B
B
Björkstrand
B
B
Christensson
E
E
Hellström-Lindberg
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D020275:Guillain-Barre Syndrome / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation; D006461:Hemolysis; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000150:complications / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D012074:Remission Induction / Q000379:methods; D014182:Transplantation, Autologous; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone