title: Hematologic and renal improvement of monoclonal immunoglobulin deposition disease after treatment with bortezomib-based regimens.
abstract: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by non-organized immunoglobulin-fragments along renal basement membranes with subsequent organ deterioration. Treatment is directed against the immunoglobulin-producing clone. We treated 18 MIDD patients with bortezomib-based regimens (12 received bortezomib-dexamethasone, 6 bortezomib-dexamethasone with cyclophosphamide). Eleven (61%) patients achieved a hematologic response, but only 6 (33.3%) reached to a complete (CR) or very good partial response (VGPR). Regarding renal outcomes 77.8 and 55.6% had ≥30 and ≥50% reduction of proteinuria, respectively, but 33.3% ended up in end-stage renal disease (ESRD). Among patients with CR or VGPR, median eGFR improvement was 7.7 ml/min/1.73 m2and none progressed to ESRD, but no significant renal recovery was observed in patients achieving a partial response or less, with 50% progressing to dialysis. Pretreatment eGFR seems to influence renal prognosis. Bortezomib-based treatment is considered an effective approach in MIDD and reaching to a deep hematologic response (≥VGPR) conditionally controls further renal declining.
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Dimitrios C
authors.initials: DC
authors.lastname: Ziogas
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Efstathios
authors.initials: E
authors.lastname: Kastritis
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Evangelos
authors.initials: E
authors.lastname: Terpos
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Maria
authors.initials: M
authors.lastname: Roussou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Magdalini
authors.initials: M
authors.lastname: Migkou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Maria
authors.initials: M
authors.lastname: Gavriatopoulou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Despoina
authors.initials: D
authors.lastname: Spanomichou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Evangelos
authors.initials: E
authors.lastname: Eleutherakis-Papaiakovou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Despoina
authors.initials: D
authors.lastname: Fotiou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Panagiotidis
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Eftychia
authors.initials: E
authors.lastname: Kafantari
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Erasmia
authors.initials: E
authors.lastname: Psimenou
authors.affiliation: b Department of Nephrology and Renal Transplantation Unit , Laiko General Hospital , Athens , Greece.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Boletis
authors.affiliation: c Renal Unit, Attikon General Hospital , National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Demetrios V
authors.initials: DV
authors.lastname: Vlahakos
authors.affiliation: d Department of Histopathology , National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Hariklia
authors.initials: H
authors.lastname: Gakiopoulou
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Charis
authors.initials: C
authors.lastname: Matsouka
authors.affiliation: a Department of Clinical Therapeutics , Alexandra General Hospital, National and Kapodistrian University of Athens, School of Medicine , Athens , Greece.
authors.forename: Meletios A
authors.initials: MA
authors.lastname: Dimopoulos
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D015415:Biomarkers; D001706:Biopsy; D000069286:Bortezomib / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D006402:Hematologic Diseases / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000378:metabolism*; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism* / Q000503:physiopathology; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D000069286:Bortezomib
keywords: ESRD; MIDD; bortezomib; proteinuria; renal response