Heavy Lifting: Nomenclature and Novel Therapy for Gamma Heavy Chain Disease and Other Heavy Chain Disorders |
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| Institution: | 1. Department of Biomedical and Clinical Sciences, University of Milan, Luigi Sacco Hospital Milan, Milan, Italy;2. Department of Hematology, Cambridge Institute for Medical Research, Cambridge University, Cambridge, UK;3. Fondazione Matarelli Milano, Milan, Italy;1. Department of Internal Medicine, King Edward Medical University, Lahore, Pakistan;2. Department of Internal Medicine, Rawalpindi Medical University, Rawalpindi, Pakistan;3. Mary Babb Randolph Cancer Center, West Virginia University Health Sciences Center Morgantown, WV;4. Department of Hematology, Medical Oncology, Taussig Cancer Center, Cleveland Clinic, Cleveland, OH;5. Department of Internal Medicine, John H. Stroger Jr. Hospital of Cook County, Chicago, IL;6. Department of Internal Medicine, Zucker School of Medicine Mather Hospital, Port Jefferson, NY;7. Department of Internal Medicine, Allegheny Health Network, Pittsburgh, PA;1. Plasma Cell Disorders Division, Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute/Atrium Health, Charlotte, NC;2. Department of Translational Research, Levine Cancer Institute, Atrium Health, Charlotte, NC;1. M.P. Shah Government Medical College, Jamnagar, Gujarat, India;2. Department of Pathology, Vanderbilt University Medical Center, Nashville, TN;3. Department of Hematology-Oncology, Vanderbilt University Medical Center, Nashville, TN;1. Department of Pathology, George Washington University, Washington, DC;2. Department of Pathology, Veterans Health Administration, Washington, DC;3. Department of Hematology and Oncology, Veterans Health Administration, Washington, DC |
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| Abstract: | Heavy chain disorders are rare B-cell disorders and include heavy chain disease, heavy chain deposition disease, and heavy chain amyloidosis. These disorders share the pathognomonic finding of a truncated immunoglobulin heavy chain without an associated light chain in the serum or urine in the case of heavy chain disease or in the tissues in the case of heavy chain deposition disease and heavy chain amyloidosis but are clinically distinct entities. The clinical recognition and systematic approaches to these disorders are challenging because of the rarity of the diseases, lack of consensus on treatment approaches, and minimal data with novel therapy. Herein we present a review of the literature and 5 consecutive cases at a single institution of gamma heavy chain disease and heavy chain deposition disease treated with novel agents including regimens of CRd (cyclophosphamide, lenalidomide, and dexamethasone), CyBorD (cyclophosphamide, bortezomib, and dexamethasone), R-CVP (rituximab, cyclophosphamide, vincristine, and dexamethasone), BR (bendamustine and rituximab), V-EPOCH (bortezomib, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin), and autologous hematopoietic stem cell transplantation. |
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| Keywords: | Heavy chain amyloid Heavy chain deposition disease |
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