Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support.
In a patient with nephrotic syndrome, renal biopsy revealed AL amyloid deposits. Monoclonal lambda light chains were identified in serum and urine. A low percentage of monoclonal plasma cells was detected in the bone marrow. The patient received four cycles of VAD and subsequent high-dose chemotherapy (HDCT) with melphalan (200 mg/m2) followed by autologous peripheral blood stem cell transplantation. Proteinuria rapidly diminished during chemotherapy. Three months after HDCT, the patient has no edema, and no signs of plasma cell dyscrasia are currently detectable. Using VAD before starting HDCT may improve the condition of patients with amyloidosis and reduce transplantation-related morbidity and mortality.
Sezer
O
O
Universitätsklinikum Charité, Department of Internal Medicine, Haematology/Oncology, Humboldt University, Berlin, Germany.
Schmid
P
P
Shweigert
M
M
Heider
U
U
Eucker
J
J
Harder
H
H
Sinha
P
P
Radtke
H
H
Possinger
K
K
D000686:Amyloidosis / Q000150:complications*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003907:Dexamethasone / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008558:Melphalan / Q000627:therapeutic use*; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D012008:Recurrence; D014182:Transplantation, Autologous; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D008558:Melphalan