title: Immunoparesis in newly diagnosed AL amyloidosis is a marker for response and survival.
abstract: Immunoparesis is an adverse prognostic marker in plasma cell proliferative disorders. Its impact in AL amyloidosis has not been explored in depth. Newly diagnosed AL amyloidosis patients (n=998) were evaluated for immunoparesis by two methods. The first method was qualitative, considering the number of suppressed uninvolved immunoglobulins below the lower limit of normal (LLN) (none, partial, all). The second method was quantitative, assessing the average relative difference (ARD) of the uninvolved immunoglobulins from the LLN. Patients with suppression of all the uninvolved immunoglobulins were less likely to achieve very good partial response (VGPR) or better to first-line treatment (44%) compared with patients with partial suppression (68%) or preserved uninvolved immunoglobulins (64%; P<0.0001). In addition, patients with suppression of all the uninvolved immunoglobulins had a shorter survival compared with the respective comparators (median 18 vs 54 vs 52 months; P<0.0001). In the quantitative method, patients with a negative ARD were less likely to achieve VGPR or better (48%) and had a shorter survival (median 24 months) compared with patients with a positive ARD (69%, 57 months, respectively; P<0.0001). In a multivariate analysis for survival, both assessment methods retained an independent impact. Significant immunoparesis has a negative impact on response and survival in newly diagnosed AL amyloidosis.
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: E
authors.initials: E
authors.lastname: Muchtar
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: A
authors.initials: A
authors.lastname: Dispenzieri
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: S K
authors.initials: SK
authors.lastname: Kumar
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: F K
authors.initials: FK
authors.lastname: Buadi
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: M Q
authors.initials: MQ
authors.lastname: Lacy
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: S
authors.initials: S
authors.lastname: Zeldenrust
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: S R
authors.initials: SR
authors.lastname: Hayman
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.|Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.
authors.forename: N
authors.initials: N
authors.lastname: Leung
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: T V
authors.initials: TV
authors.lastname: Kourelis
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: W
authors.initials: W
authors.lastname: Gonsalves
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.|Hospitalist services, Essentia Health St. Joseph's Hospital, Brainerd, MN, USA.
authors.forename: R
authors.initials: R
authors.lastname: Chakraborty
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: S
authors.initials: S
authors.lastname: Russell
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: D
authors.initials: D
authors.lastname: Dingli
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: J A
authors.initials: JA
authors.lastname: Lust
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Y
authors.initials: Y
authors.lastname: Lin
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: P
authors.initials: P
authors.lastname: Kapoor
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: R
authors.initials: R
authors.lastname: Go
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: R A
authors.initials: RA
authors.lastname: Kyle
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: S V
authors.initials: SV
authors.lastname: Rajkumar
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: M A
authors.initials: MA
authors.lastname: Gertz
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000276:immunology* / Q000401:mortality; D015415:Biomarkers; D005260:Female; D006801:Humans; D007108:Immune Tolerance; D007136:Immunoglobulins; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D015996:Survival Rate
chemical_list: D015415:Biomarkers; D007136:Immunoglobulins