Hematologic and renal improvement of monoclonal immunoglobulin deposition disease after treatment with bortezomib-based regimens.
Leuk Lymphoma
; 58(8): 1832-1839, 2017 08.
Article
in En
| MEDLINE
| ID: mdl-27967286
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 m2 and 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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Paraproteinemias
/
Immunoglobulin Heavy Chains
/
Immunoglobulin Light Chains
/
Hematologic Diseases
/
Kidney Diseases
Type of study:
Diagnostic_studies
/
Prognostic_studies
Limits:
Aged
/
Aged80
/
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
Leuk Lymphoma
Journal subject:
HEMATOLOGIA
/
NEOPLASIAS
Year:
2017
Document type:
Article
Affiliation country:
Greece