[Randall-type monoclonal immunoglobulin deposition disease: From diagnosis to treatment].
Monoclonal immunoglobulin (Ig) deposition disease (MIDD) is a rare complication of plasma cell disorders, defined by linear Congo red-negative deposits of monoclonal light chain (LCDD), heavy chain (HCDD) or both (LHCDD) along basement membranes. MIDD should be suspected in patients presenting with glomerular proteinuria and monoclonal gammopathy, but none of these criteria is necessary for the diagnosis although renal involvement is prominent. Since an abnormal serum κ/λ ratio is found in virtually all MIDD patients, including those with HCDD, serum free light chain assay should be included in the initial workup in patients older than 50 presenting with kidney disease. Bortezomib-based regimens are efficient and well tolerated, resulting in improvement in both renal and global survival, comparatively to historical series. High dose melphalan with autologous stem cell transplantation may be proposed as second line therapy in selected patients. The achievement of hematological response, based on the difference between involved and uninvolved serum free light chains (dFLC), is mandatory. In a recent series, post-treatment dFLC<40mg/L was the major predictive factor of renal response and was associated with improvement of both renal and global survival. In MIDD, bortezomib-based therapy is safe and efficient when introduced early after diagnosis. dFLC response is a favorable prognostic factor for renal survival.
Service de néphrologie, hôpital Necker, 149, rue de sèvres, 75015 Paris, France. Electronic address: camille.cohen@inserm.fr.
Camille
C
Cohen
Service de néphrologie, centre national de référence maladies rares : amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
Vincent
V
Javaugue
Service de néphrologie, centre national de référence maladies rares : amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
Florent
F
Joly
Service d'immuno-hématologie, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Bertrand
B
Arnulf
Service d'immuno-hématologie, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Jean-Paul
JP
Fermand
Service d'hématologie clinique, centre national de référence maladies rares : amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU de Limoges, 2, avenue Martin-Luther-King, 87000 Limoges, France.
Arnaud
A
Jaccard
CNRS UMR 7276, centre national de référence maladies rares : amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, Limoges, 2, avenue Martin-Luther-King, 87000 Limoges, France.
Christophe
C
Sirac
Service de néphrologie, hôpital Necker, 149, rue de sèvres, 75015 Paris, France.
Bertrand
B
Knebelmann
Service de néphrologie, centre national de référence maladies rares : amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
Frank
F
Bridoux
Service de néphrologie, centre national de référence maladies rares : amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
Guy
G
Touchard
D003663:Decision Trees; D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D010265:Paraproteinemias / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*
Bortezomib; Bortézomib; Chaînes légères libres; Gammapathie monoclonale de signification rénale; Immunoglobuline monoclonale; Maladie des dépôts d’immunoglobulines monoclonales; Monoclonal gammopathy of renal significance; Monoclonal immunoglobulin; Monoclonal immunoglobulin deposition disease; Serum free light chains