AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome.

Blood
Ashutosh D WechalekarJulian D Gillmore

Abstract

AL amyloidosis associated with immunoglobulin M (IgM) paraproteinemia is rare. We report 103 consecutive such patients evaluated at the National Amyloidosis Centre (London, United Kingdom) between 1988 and 2006. Renal, cardiac, and lymph node amyloid was present in 53%, 35%, and 21% of patients, respectively, at presentation and 2 or more organs were involved in 54%. Seventy-three percent had an abnormal bone marrow infiltrate (lymphoid in 87%). The median IgM paraprotein was 8 g/L and serum free light chain (FLC) ratio was abnormal in 77 (88%) of 87. The abnormal FLC component was more than 100 mg/L in only 31% cases. Thirty-two percent achieved a partial hematologic response to treatment with no complete responders, and there appeared to be a greater response to combination regimens than single-agent oral alkylators (59% vs 20%, respectively; P = .003). Four achieved amyloidotic organ responses; organ function remained stable in 68%. None with lymph node involvement showed nodal improvement. Median overall survival was 49 months. AL amyloidosis with IgM paraproteinemia represents a distinctive subset of patients with AL amyloidosis who have a wider variety of underlying clonal disorders (often lymphoid) than AL in general, ha...Continue Reading

References

Aug 23, 1990·The New England Journal of Medicine·P N HawkinsM B Pepys
Apr 1, 1983·Annals of Internal Medicine·M A GertzP R Greipp
Jan 1, 1980·Archives of Internal Medicine·R C Bone
Dec 1, 1982·American Journal of Clinical Oncology·M M OkenP P Carbone
Jul 1, 1993·The American Journal of Medicine·M A DimopoulosM J Keating
May 1, 1993·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·M A GertzP Noel
Jul 31, 1998·Journal of Oral Pathology & Medicine : Official Publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology·E W OdellC A White
Oct 19, 1999·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·M SetoguchiT Ishihara
Sep 28, 2000·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·S DacicS A Yousem
Jul 31, 2001·Journal of Clinical Pathology·J K LimM A Gertz
Dec 12, 2001·British Journal of Haematology·R García-SanzUNKNOWN Spanish Group for the Study of Waldenström Macroglobulinaemia and PETHEMA (Programme for the Study and Treatment of Haematol
Jun 7, 2002·The New England Journal of Medicine·Helen J LachmannPhilip N Hawkins
Nov 1, 2002·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Katrin WiekerAstrid Franke
Jan 11, 2003·Clinical Lymphoma·Meletios A DimopoulosJohn Christakis
Apr 30, 2003·Seminars in Oncology·Morie A Gertz, Robert A Kyle
Dec 26, 2003·The New England Journal of Medicine·Michel AttalUNKNOWN InterGroupe Francophone du Myélome
Jan 14, 2004·British Journal of Haematology·A D CohenR L Comenzo
Apr 22, 2005·American Journal of Clinical Pathology·Pei LinL Jeffrey Medeiros
Oct 4, 2005·Biochimica Et Biophysica Acta·Laura ObiciGiampaolo Merlini
Mar 31, 2006·The New England Journal of Medicine·Robert A KyleL Joseph Melton
Jul 15, 2006·Leukemia & Lymphoma·Maria ValenteMorie A Gertz
Jul 26, 2006·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Athanasios AnagnostopoulosSergio A Giralt
Aug 30, 2006·British Journal of Haematology·Robert A Kyle, S Vincent Rajkumar
Jan 4, 2008·The New England Journal of Medicine·Shaji KumarMorie A Gertz

❮ Previous
Next ❯

Citations

Feb 9, 2012·Annals of Hematology·Tao ChanYok-Lam Kwong
Nov 18, 2008·Zeitschrift für Rheumatologie·S Ladner-Merz, U Müller-Ladner
Jul 21, 2009·Blood·Raymond L Comenzo
Feb 6, 2014·Haematologica·Shameem MahmoodAshutosh Wechalekar
Apr 30, 2015·Case Reports in Hematology·Binod DhakalAnita D'Souza
Apr 6, 2012·Expert Review of Hematology·Pierre Morel, Giampaolo Merlini
Oct 22, 2015·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Moussa SissokoJ Mark Sloan
Nov 20, 2009·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Ko-Ichi TazawaShu-Ichi Ikeda
Oct 23, 2015·British Journal of Haematology·Efstathios Kastritis, Meletios A Dimopoulos
Apr 14, 2009·Clinical Lymphoma & Myeloma·Giovanni PalladiniGiampaolo Merlini
Sep 28, 2010·American Journal of Hematology·David TelioVishal Kukreti
May 9, 2013·British Journal of Haematology·Anita D'SouzaMorie A Gertz
May 20, 2015·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Sophie ChauvetGuy Touchard
Feb 18, 2014·British Journal of Haematology·Roger G OwenUNKNOWN British Committee for Standards in Haematology
Dec 15, 2010·Seminars in Nephrology·Pierre RoncoPierre Aucouturier
Apr 2, 2011·Clinical Lymphoma, Myeloma & Leukemia·Giovanni PalladiniGiampaolo Merlini
Apr 2, 2011·Clinical Lymphoma, Myeloma & Leukemia·Morie A GertzSuzanne R Hayman
Mar 12, 2013·Clinical Lymphoma, Myeloma & Leukemia·Giovanni Palladini, Giampaolo Merlini
Nov 9, 2016·Best Practice & Research. Clinical Haematology·Paolo Milani, Giampaolo Merlini
Dec 23, 2016·Expert Opinion on Pharmacotherapy·Giulia ZumboMarianna Fontana
Apr 27, 2016·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Sajitha SachchithananthamAshutosh D Wechalekar
Sep 15, 2017·Respiration; International Review of Thoracic Diseases·Yurdagül UzunhanDominique Valeyre
Jan 9, 2013·British Journal of Haematology·Moshe E Gatt, Giovanni Palladini
Nov 13, 2019·Blood·Marco BassetGiovanni Palladini
Dec 24, 2010·Current Opinion in Oncology·Giovanni Palladini, Giampaolo Merlini
Jan 27, 2016·Clinical Chemistry and Laboratory Medicine : CCLM·Peter Mollee, Giampaolo Merlini
Aug 16, 2011·The Journal of Immunology : Official Journal of the American Association of Immunologists·Salar N KhanMarko Radic
Oct 14, 2018·Leukemia·Saurabh ZanwarPrashant Kapoor
Jul 8, 2020·Cancers·Louis-Pierre GirardSanjay de Mel
Dec 15, 2020·Curēus·Karim BayanzayAlissa Holman
Jul 29, 2009·Current Opinion in Pulmonary Medicine

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.