Generalized cutis laxa and fibrillar glomerulopathy resulting from IgG Deposition in IgG-lambda Monoclonal Gammopathy: pulmonary hemorrhage during stem cell mobilization and complete hematological response with bortezomib and dexamethasone therapy.
The case of a 52-years-old man with generalized acquired cutis laxa associated with IgG-lambda monoclonal gammopathy and nephrotic syndrome with renal failure (due to fibrillar glomerulopathy resulting from IgG deposition) is reported. A peripheral blood autologous stem cell transplant was planned, but the procedure was complicated by severe pulmonary hemorrhage during stem cells mobilization with granulocyte colony-stimulating factor (G-CSF). Treatment with bortezomib and dexamethasome was subsequently started and a complete hematological response was achieved. Finally, the complete hematological response with the disappearance of the toxic M-protein allows the possibility of a long-term benefit with a kidney transplant followed by an autologous bone marrow transplant.
Fernández de Larrea
Carlos
C
Department of Hematology, Hospital Clinic de Barcelona, Institute d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Rovira
Montserrat
M
Mascaró
José M
JM
Torras
Albert
A
Solé
Manel
M
Lloreta
Josep
J
Serra
Núria
N
Cibeira
Maria Teresa
MT
Bladé
Joan
J
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001897:Boronic Acids / Q000627:therapeutic use*; D000069286:Bortezomib; D003483:Cutis Laxa / Q000150:complications*; D003907:Dexamethasone / Q000627:therapeutic use*; D016179:Granulocyte Colony-Stimulating Factor / Q000008:administration & dosage; D006470:Hemorrhage / Q000150:complications*; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D007674:Kidney Diseases / Q000150:complications*; D007678:Kidney Glomerulus / Q000473:pathology*; D008168:Lung / Q000098:blood supply*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D011719:Pyrazines / Q000627:therapeutic use*
D000970:Antineoplastic Agents; D001897:Boronic Acids; D007074:Immunoglobulin G; D011719:Pyrazines; D016179:Granulocyte Colony-Stimulating Factor; D000069286:Bortezomib; D003907:Dexamethasone