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Heavy Lifting: Nomenclature and Novel Therapy for Gamma Heavy Chain Disease and Other Heavy Chain Disorders
- Source :
- Clinical lymphoma, myelomaleukemia. 20(8)
- Publication Year :
- 2019
-
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.
- Subjects :
- Bendamustine
Male
Cancer Research
medicine.medical_treatment
Hematopoietic stem cell transplantation
030204 cardiovascular system & hematology
Immunoglobulin light chain
03 medical and health sciences
0302 clinical medicine
Medicine
Humans
Gamma Heavy Chain Disease
business.industry
Bortezomib
Hematology
Middle Aged
medicine.disease
Heavy chain disease
Oncology
030220 oncology & carcinogenesis
Cancer research
Immunoglobulin heavy chain
Rituximab
Female
business
medicine.drug
Heavy Chain Disease
Subjects
Details
- ISSN :
- 21522669
- Volume :
- 20
- Issue :
- 8
- Database :
- OpenAIRE
- Journal :
- Clinical lymphoma, myelomaleukemia
- Accession number :
- edsair.doi.dedup.....70d5f7444be2a276c676da6b333fcdb6