Abstract
IgM-associated AL amyloidosis is rare and may represent a distinct entity. Sixty (7%) of 868 consecutive AL patients referred to our center had an IgM monoclonal protein. They were significantly older than non-IgM patients (median, 67 years vs. 62 years), had a higher frequency of lymph-node involvement (25% vs. 2%) and significantly lower median proteinuria (1.2 g/24h vs. 3.4 g/24h), N-terminal pro-natriuretic peptide type-B (1177 ng/L vs. 2135 ng/L) and troponin I (0.02 ng/mL vs. 0.05 ng/mL). In IgM patients, κ light-chains were more frequent (42% vs. 23%) and the involved free light-chain concentration was lower (median 63 mg/L vs. 182 mg/L). Serum albumin and NT-proBNP were independent prognostic determinants. Response to treatment improved survival. The 14 patients who received melphalan/dexamethasone showed a 64% hematologic (complete remissions, 29%) and a 43% organ response rate. IgM-associated AL amyloidosis is a distinct entity, with less advanced organ dysfunction. Treatment with melphalan/ dexamethasone might be effective in these patients.
| Original language | English |
|---|---|
| Pages (from-to) | 80-83 |
| Number of pages | 4 |
| Journal | Clinical Lymphoma and Myeloma |
| Volume | 9 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - 2009 |
Fingerprint
Keywords
- Dexamethasone
- Melphalan
- Prednisone
- Prognosis
- Response
- Waldenström
- Waldenström's macroglobulinemia
ASJC Scopus subject areas
- Cancer Research
- Oncology
- Hematology
Cite this
AL amyloidosis associated with IgM monoclonal protein : A distinct clinical entity. / Palladini, Giovanni; Russo, Paola; Bosoni, Tiziana; Sarais, Gabriele; Lavatelli, Francesca; Foli, Andrea; Bragotti, Letizia Zenone; Perfetti, Vittorio; Obici, Laura; Bergesio, Franco; Albertini, Riccardo; Moratti, Remigio; Merlini, Giampaolo.
In: Clinical Lymphoma and Myeloma, Vol. 9, No. 1, 2009, p. 80-83.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - AL amyloidosis associated with IgM monoclonal protein
T2 - A distinct clinical entity
AU - Palladini, Giovanni
AU - Russo, Paola
AU - Bosoni, Tiziana
AU - Sarais, Gabriele
AU - Lavatelli, Francesca
AU - Foli, Andrea
AU - Bragotti, Letizia Zenone
AU - Perfetti, Vittorio
AU - Obici, Laura
AU - Bergesio, Franco
AU - Albertini, Riccardo
AU - Moratti, Remigio
AU - Merlini, Giampaolo
PY - 2009
Y1 - 2009
N2 - IgM-associated AL amyloidosis is rare and may represent a distinct entity. Sixty (7%) of 868 consecutive AL patients referred to our center had an IgM monoclonal protein. They were significantly older than non-IgM patients (median, 67 years vs. 62 years), had a higher frequency of lymph-node involvement (25% vs. 2%) and significantly lower median proteinuria (1.2 g/24h vs. 3.4 g/24h), N-terminal pro-natriuretic peptide type-B (1177 ng/L vs. 2135 ng/L) and troponin I (0.02 ng/mL vs. 0.05 ng/mL). In IgM patients, κ light-chains were more frequent (42% vs. 23%) and the involved free light-chain concentration was lower (median 63 mg/L vs. 182 mg/L). Serum albumin and NT-proBNP were independent prognostic determinants. Response to treatment improved survival. The 14 patients who received melphalan/dexamethasone showed a 64% hematologic (complete remissions, 29%) and a 43% organ response rate. IgM-associated AL amyloidosis is a distinct entity, with less advanced organ dysfunction. Treatment with melphalan/ dexamethasone might be effective in these patients.
AB - IgM-associated AL amyloidosis is rare and may represent a distinct entity. Sixty (7%) of 868 consecutive AL patients referred to our center had an IgM monoclonal protein. They were significantly older than non-IgM patients (median, 67 years vs. 62 years), had a higher frequency of lymph-node involvement (25% vs. 2%) and significantly lower median proteinuria (1.2 g/24h vs. 3.4 g/24h), N-terminal pro-natriuretic peptide type-B (1177 ng/L vs. 2135 ng/L) and troponin I (0.02 ng/mL vs. 0.05 ng/mL). In IgM patients, κ light-chains were more frequent (42% vs. 23%) and the involved free light-chain concentration was lower (median 63 mg/L vs. 182 mg/L). Serum albumin and NT-proBNP were independent prognostic determinants. Response to treatment improved survival. The 14 patients who received melphalan/dexamethasone showed a 64% hematologic (complete remissions, 29%) and a 43% organ response rate. IgM-associated AL amyloidosis is a distinct entity, with less advanced organ dysfunction. Treatment with melphalan/ dexamethasone might be effective in these patients.
KW - Dexamethasone
KW - Melphalan
KW - Prednisone
KW - Prognosis
KW - Response
KW - Waldenström
KW - Waldenström's macroglobulinemia
UR - http://www.scopus.com/inward/record.url?scp=67649556687&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=67649556687&partnerID=8YFLogxK
U2 - 10.3816/CLM.2009.n.021
DO - 10.3816/CLM.2009.n.021
M3 - Article
C2 - 19362981
AN - SCOPUS:67649556687
VL - 9
SP - 80
EP - 83
JO - Clinical Lymphoma and Myeloma
JF - Clinical Lymphoma and Myeloma
SN - 1557-9190
IS - 1
ER -