Development of rapidly progressive liver light chain deposition under VAD chemotherapy in multiple myeloma.
Light chain deposition disease (LCDD) is a multisystemic disorder seen in the setting of plasma cell dyscrasias. The histological characteristic of this disorder is the deposition of a homogeneous, granular, slightly eosinophilic and non-Congophilic material that shows immunostaining for monoclonal light chains (kappa or gamma), while in primary amyloidosis (AL) the proteinaceous substance is fibrillar and Congo red positive. In contrast with AL, the light chain in LCDD is usually of the kappa-type. Renal involvement, resulting in nephrotic syndrome, is usually the prominent feature of LCDD. Patients with this disease may also have heart, liver or other organ involvement, mimicking the picture of primary systemic amyloidosis. However, liver failure has rarely been described in patients with LCDD. A patient with myeloma-associated LCDD who developed rapidly progressive liver kappa light chain deposition with fatal outcome after undergoing the first cycle of vincristine/doxorubicin/dexamethasone chemotherapy is reported.
Samanez
César
C
Department of Oncology, Instituto de Enfermedades Neoplásicas, Surquillo, Perú.
Domingo
Abel
A
Cibeira
M Teresa
MT
Miquel
Rosa
R
Soler
Manel
M
Bladé
Joan
J
D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D000970:Antineoplastic Agents / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism*; D008099:Liver / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D051437:Renal Insufficiency / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D013577:Syndrome; D014750:Vincristine / Q000008:administration & dosage
D000682:Amyloid; D000893:Anti-Inflammatory Agents; D000970:Antineoplastic Agents; D007145:Immunoglobulin kappa-Chains; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin