title: Uninvolved immunoglobulins predicting hematological response in newly diagnosed AL amyloidosis.
abstract: Immunoparesis serves as a marker for elevated risk for progression in plasma cell proliferative disorders. However, the impact of immunoparesis in AL amyloidosis has not been addressed. Immunoparesis was defined qualitatively as any decrease below the low reference levels of the uninvolved immunoglobulins and quantitatively, as the relative difference between the uninvolved immunoglobulins and the lower reference values. Forty-one newly diagnosed AL amyloidosis patients were included. Sixty-six percent of patients had a suppression of the uninvolved immunoglobulins. The median relative difference of the uninvolved immunoglobulins was 18% above the low reference levels [range (-71%)-210%]. Ninety percent of the patients were treated with novel agents-based regimens, mostly bortezomib-containing regimens. Nineteen percent of the patients did not attain response to first line treatment. Patients with relative difference of uninvolved immunoglobulins below -25% of the low reference levels were less likely to respond to first line treatment compared to patients with a relative difference of -25% and above [odds ratio for no response vs. partial response and better 30 [(95% CI 4.1-222.2), P=0.0004]. Patients who failed first line treatment were successfully salvaged with lenalidomide-based treatment. Immunoparesis, if assessed quantitatively, may serve as a predictor of response in AL amyloidosis patients treated with bortezomib-containing regimens.
authors.affiliation: Institute of Hematology, Davidoff Cancer Center, Beilinson Hospital, Rabin Medical Centre, Petah-Tikva, Israel; Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel. Electronic address: muchtare@gmail.com.
authors.forename: Eli
authors.initials: E
authors.lastname: Muchtar
authors.affiliation: Institute of Hematology, Davidoff Cancer Center, Beilinson Hospital, Rabin Medical Centre, Petah-Tikva, Israel; Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Hila
authors.initials: H
authors.lastname: Magen
authors.affiliation: Institute of Hematology, Davidoff Cancer Center, Beilinson Hospital, Rabin Medical Centre, Petah-Tikva, Israel; Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Gilad
authors.initials: G
authors.lastname: Itchaki
authors.affiliation: Institute of Hematology, Davidoff Cancer Center, Beilinson Hospital, Rabin Medical Centre, Petah-Tikva, Israel; Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Amos
authors.initials: A
authors.lastname: Cohen
authors.affiliation: Immunology Laboratory, Rabin Medical Centre, Petah-Tikva, Israel.
authors.forename: Ra'ama
authors.initials: R
authors.lastname: Rosenfeld
authors.affiliation: Statistical Unit, Research Department, Rabin Medical Centre, Petah-Tikva, Israel.
authors.forename: Tzippy
authors.initials: T
authors.lastname: Shochat
authors.affiliation: Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel; Cardiology Department, Rabin Medical Centre, Petah-Tikva, Israel.
authors.forename: Ran
authors.initials: R
authors.lastname: Kornowski
authors.affiliation: Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel; Cardiology Department, Rabin Medical Centre, Petah-Tikva, Israel.
authors.forename: Zaza
authors.initials: Z
authors.lastname: Iakobishvili
authors.affiliation: Institute of Hematology, Davidoff Cancer Center, Beilinson Hospital, Rabin Medical Centre, Petah-Tikva, Israel; Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Pia
authors.initials: P
authors.lastname: Raanani
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000188:drug therapy* / Q000276:immunology* / Q000401:mortality; D000069286:Bortezomib / Q000627:therapeutic use; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis / Q000096:biosynthesis; D000075363:Immunoglobulin Light-chain Amyloidosis; D007136:Immunoglobulins / Q000032:analysis / Q000096:biosynthesis*; D007155:Immunologic Factors / Q000627:therapeutic use*; D053208:Kaplan-Meier Estimate; D000077269:Lenalidomide; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007147:Immunoglobulin Light Chains; D007136:Immunoglobulins; D007155:Immunologic Factors; D013792:Thalidomide; D000069286:Bortezomib; D000077269:Lenalidomide
keywords: AL amyloidosis; Bortezomib; Immunoparesis; Lenalidomide; Uninvolved immunoglobulins; response