Publication Date:
2014-12-06
Description:
Lytic bone involvement in primary systemic (AL) amyloidosis is uncommon. To-date, there is no systematic study of bone metabolism in patients with AL amyloidosis. For this reason, we evaluated prospectively 102 patients with previously untreated AL amyloidosis who were diagnosed between January 2000 and June 2008 in the Plasma Cell Dyscrasias Unit of the Department of Clinical Therapeutics (Athens, Greece). All patients had histologically confirmed AL amyloidosis, while the definition of organ involvement, hematological and organ response and progression were based on consensus criteria. The levels of the following bone remodeling indices were measured before the administration of any kind of therapy: i) osteoclast regulators: soluble receptor activator of nuclear factor-kappaB ligand (sRANKL), osteoprotegerin (OPG), chemokine C-C motif ligand 3 (CCL-3, previously known as MIP-1alpha) and osteopontin; ii) bone resorption markers: C- and N- telopeptide of type-1 collagen (CTX and NTX, respectively) and tartrate resistant acid phosphatase type-5b; iii) bone formation markers: bone-alkaline phosphatase and osteocalcin. The results were compared with those of 35 age- and gender-matched healthy controls, 40 patients with monoclonal gammopathy of undetermined significance (MGUS) and 35 newly diagnosed, untreated patients with symptomatic multiple myeloma (MM). The median age of AL patient was 65 years (range: 39-80 years); 43% were males; 60% had heart involvement, 73% renal involvement, 11% liver involvement and 40% had peripheral/autonomous nerve system involvement; 67% of patients had two or more organs involved; 16% had a baseline serum creatinine 〉2 mg/dl. The median serum albumin was 3.2 g/dl, the median serum β2-microglobulin was 1.8 mg/L and the median 24h urine protein was 3350 mg/24h. Median NT-proBNP was 1954 pg/ml; 26%, 41% and 33% of patients were Mayo stage -1, -2 and -3, respectively. None of AL patients had lytic bone lesions in plain radiography or other features suggestive of MM, such as hypercalcemia, significant anemia unrelated to renal impairment or predominant Bence-Jones proteinuria. Bone resorption (assessed by CTX or NTX) in AL-patients was increased (p
Print ISSN:
0006-4971
Electronic ISSN:
1528-0020
Topics:
Biology
,
Medicine
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