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Ending chemotherapy Al Amyloidosis

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Renal impairment (RI) is a common complication of multiple myeloma (MM), which is presented as chronic kidney disease (CKD) or acute kidney injury (AKI). The typical pathological feature is cast nephropathy. Presently international system staging (ISS) is used in evaluating MM. Although the classic Durie-Salmon staging system could be still used in clinical practice, it may miss out some patients with renal impairment. For evaluations of RI in MM patients with CKD, it's recommended to assess the estimated glomerular filtration rate (eGFR) by creatinine based formula CKD-epidemiology collaboration (EPI) or modification of diet in renal disease(MDRD) and to stage the renal injuries according to 2013 Kidney Disease Improving Global Outcomes (KDIGO) CKD guidelines...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Jolanta Małyszko, Klaudia Kozłowska, Jacek Stanisław Małyszko
Amyloidosis is the general term describing the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins. There are multiple different human protein precursors of amyloid fibrils. Amyloid deposits are stained using Congo Red and show typical apple-green birefringence in polarized microscopy. Nowadays, a novel technique LMD/MS technique or laser microdissection combined with mass spectrometry help to diagnose amyloidosis. Amyloidosis of the kidney is typically classified as being either one of two types: AL or AA...
March 2017: Advances in Medical Sciences
M K Davis, P Kale, M Liedtke, S Schrier, S Arai, M Wheeler, R Lafayette, T Coakley, R M Witteles
We conducted a review of patients undergoing heart transplantation (HT) at our institution for amyloid cardiomyopathy (ACM) between 2008 and 2013. Complete follow-up was available for all patients. Nineteen patients with ACM underwent HT during the study period, accounting for 9.4% of all HT performed at our institution during this period. Amyloid subtype was light chain (AL) in 9 patients and transthyretin (ATTR) in 10 (2 wild-type, 7 familial, 1 unknown). Eight of nine patients with AL amyloidosis began chemotherapy prior to HT, six have resumed chemotherapy since HT, and five have undergone autologous stem cell transplantation...
March 2015: American Journal of Transplantation
Lauren Gray Gilstrap, Emily Niehaus, Rajeev Malhotra, Van-Khue Ton, James Watts, David C Seldin, Joren C Madsen, Marc J Semigran
BACKGROUND: Orthotopic heart transplant (OHT), followed by myeloablative chemotherapy and autologous stem cell transplant (ASCT), has been successful in the treatment of amyloid light-chain (AL) cardiac amyloidosis. The purpose of this study was to identify predictors of survival to OHT in patients with end-stage heart failure due to AL amyloidosis and compare post-OHT survival of cardiac amyloid patients with survival of other cardiomyopathy patients undergoing OHT. METHODS: From January 2000 to June 2011, 31 patients with end-stage heart failure secondary to AL amyloidosis were listed for OHT at Massachusetts General Hospital...
February 2014: Journal of Heart and Lung Transplantation
Z Adam, J Krejčí, M Krejčí, P Němec, L Spinarová, V Zampachová, Z Cermáková, T Pika, L Pour, Z Kořístek, M Tomíška, P Szturz, Z Král, J Mayer
UNLABELLED: Severe damage to the heart caused by AL amyloid deposits is a contraindication of high-dose chemotherapy with autologous haematopoietic stem cell transplantation. Severe heart damage caused by AL amyloid results in frequent life-threatening complications, even during the course of the classical chemotherapy treatment and it often makes keeping to the treatment schedule impossible. Scheduling heart transplantation before the treatment of AL amyloidosis will significantly improve the patients overall condition and enable them to undergo the intensive AL amyloidosis treatment with the hope that a long-term complete remission may be achieved...
February 2013: Vnitr̆ní Lékar̆ství
J H Pinney, H J Lachmann, P T Sattianayagam, S D J Gibbs, A D Wechalekar, C P Venner, C J Whelan, J A Gilbertson, D Rowczenio, P N Hawkins, J D Gillmore
Renal transplantation remains contentious in patients with systemic amyloidosis due to the risk of graft loss from recurrent amyloid and progressive disease. Outcomes were sought among all patients attending the UK National Amyloidosis Centre who received a renal transplant (RTx) between January 1978 and May 2011. A total of 111 RTx were performed in 104 patients. Eighty-nine percent of patients with end-stage renal disease (ESRD) due to hereditary lysozyme and apolipoprotein A-I amyloidosis received a RTx...
February 2013: American Journal of Transplantation
Dorota Rowczenio, Ahmet Dogan, Jason D Theis, Julie A Vrana, Helen J Lachmann, Ashutosh D Wechalekar, Janet A Gilbertson, Toby Hunt, Simon D J Gibbs, Prayman T Sattianayagam, Jenny H Pinney, Philip N Hawkins, Julian D Gillmore
The phenotype of hereditary apolipoprotein A-I amyloidosis is heterogeneous with some patients developing extensive visceral amyloid deposits and end-stage renal failure as young adults and others having only laryngeal and/or skin amyloid, which may be of little clinical consequence. Clinical management and prognosis of patients with systemic amyloidosis depend entirely on correct identification of the fibril protein, such that light chain amyloidosis (AL, previously referred to as "primary"), the most frequently diagnosed type, is treated with chemotherapy, which has absolutely no role in hereditary apolipoprotein A-I amyloidosis...
October 2011: American Journal of Pathology
Jennifer H Pinney, Helen J Lachmann, Loveleen Bansi, Ashutosh D Wechalekar, Janet A Gilbertson, Dorota Rowczenio, Prayman T Sattianayagam, Simon D J Gibbs, Emanuela Orlandi, Nancy L Wassef, Arthur R Bradwell, Philip N Hawkins, Julian D Gillmore
PURPOSE: Chemotherapy in AL (primary or light chain) amyloidosis is associated with improved survival, but its effect on renal outcome has not been examined systematically. The purpose of this study was to evaluate the effect of chemotherapy on clinical outcome among patients with renal AL amyloidosis. PATIENTS AND METHODS: We evaluated factors influencing survival among 923 patients with renal AL amyloidosis observed during a 21-year period, including 221 patients who became dialysis dependent...
February 20, 2011: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Falk-Udo Sack, Arnt Kristen, Hartmut Goldschmidt, Philipp A Schnabel, Thomas Dengler, Achim Koch, Matthias Karck
OBJECTIVE: Cardiac amyloidosis (CA) is associated with a poor prognosis and a survival rate of less than 30% 2 years after clinical manifestation. Considered as a semi-malignant disease, CA is often a contraindication for HTx; however, depending on the type of CA, there are excellent treatment regimes that can be combined with HTx. In AL-amyloidosis, chemotherapy and stem cell transplantation are necessary and in TTR-amyloidosis, where the liver is the source of the pathologic protein, liver transplantation is recommended after HTx...
February 2008: European Journal of Cardio-thoracic Surgery
F Bergesio, A M Ciciani, M Manganaro, G Palladini, M Santostefano, R Brugnano, A M Di Palma, M Gallo, A Rosati, P L Tosi, M Salvadori
BACKGROUND: Few data are available from large population-based studies on survival and renal outcome of patients with renal involvement and different types of systemic amyloidosis. METHODS: Two hundred and ninety of over 373 patients affected from systemic amyloidosis with renal involvement diagnosed in Italy between January 1995 and December 2000 were followed from diagnosis to death or until the last available clinical control. Eighty-three patients were excluded from analysis either because the amyloid type remained undetermined or they were lost at follow-up...
March 2008: Nephrology, Dialysis, Transplantation
Arnaud Jaccard, Philippe Moreau, Veronique Leblond, Xavier Leleu, Lotfi Benboubker, Olivier Hermine, Christian Recher, Bouchra Asli, Bruno Lioure, Bruno Royer, Fabrice Jardin, Frank Bridoux, Bernard Grosbois, Jérome Jaubert, Jean-Charles Piette, Pierre Ronco, Fabrice Quet, Michel Cogne, Jean-Paul Fermand
BACKGROUND: High-dose chemotherapy followed by autologous hematopoietic stem-cell transplantation has been reported to provide higher response rates and better overall survival than standard chemotherapy in immunoglobulin-light-chain (AL) amyloidosis, but these two strategies have not been compared in a randomized study. METHODS: We conducted a randomized trial comparing high-dose intravenous melphalan followed by autologous hematopoietic stem-cell rescue with standard-dose melphalan plus high-dose dexamethasone in patients with AL amyloidosis...
September 13, 2007: New England Journal of Medicine
Nelson Leung, Matthew D Griffin, Angela Dispenzieri, Eric N Haugen, James M Gloor, Thomas R Schwab, Stephen C Textor, Martha Q Lacy, Mark R Litzow, Fernando G Cosio, Timothy S Larson, Morie A Gertz, Mark D Stegall
Primary systemic amyloidosis (AL) is characterized by multiorgan deposition of monoclonal immunoglobulin light chain. Renal involvement is common and impaired kidney function is associated with reduced median survival. Autologous stem cell transplantation (SCT) for AL achieves superior response rates compared to chemotherapy alone but patients with end-stage renal disease (ESRD) may be excluded from consideration. A treatment approach consisting of living donor kidney transplantation (LDKTx) followed by autologous SCT was developed for AL with ESRD...
July 2005: American Journal of Transplantation
Martha Skinner, Vaishali Sanchorawala, David C Seldin, Laura M Dember, Rodney H Falk, John L Berk, Jennifer J Anderson, Carl O'Hara, Kathleen T Finn, Caryn A Libbey, Janice Wiesman, Karen Quillen, Niall Swan, Daniel G Wright
BACKGROUND: AL amyloidosis is a fatal disease resulting from tissue deposition of amyloid fibrils derived from monoclonal immunoglobulin light chains. Treatment with oral chemotherapy is minimally effective. OBJECTIVE: To test survival and organ response in a large sample of patients treated with high-dose intravenous melphalan (100 to 200 mg/m2) and autologous blood stem-cell transplantation. DESIGN: 8-year longitudinal analysis of clinical effectiveness...
January 20, 2004: Annals of Internal Medicine
R Zenhäusern, A Tobler, L Leoncini, O M Hess, P Ferrari
Amyloidosis (AL) is a rapidly fatal plasma cell dyscrasia causing progressive multiorgan failure. Recently, substantial improvement of survival was reported following high-dose chemotherapy with peripheral blood stem cell (PBSC) rescue. We describe a patient with AL with severe cardiac and renal involvement who received high-dose melphalan followed by fractioned autologous PBSC transplantation (455 ml on day 1 and 350 ml on day 2). Immediately after the second infusion of the PBSCs, life-threatening cardiac arrhythmias occurred and, despite intensive treatment, the patient died less than 24 h later...
September 2000: Annals of Hematology
J J Montseny, D Kleinknecht, A Meyrier, P Vanhille, P Simon, A Pruna, D Eladari
BACKGROUND: The prognosis of monoclonal gammopathies with multiple myeloma and renal involvement is poor, and the indication for renal replacement therapy is controversial. Few studies address the value of renal histology for determining prognosis according to initial pathology findings. METHODS: We studied the course of 118 patients with multiple myeloma according to renal biopsy lesions. The monoclonal component was identified and quantified in serum and urine...
June 1998: Nephrology, Dialysis, Transplantation
D J Goldsmith, D Sandooran, C D Short, N P Mallick, R W Johnson
AL-amyloidosis has a poor prognosis, typically with cardiac or renal failure ensuing some months after diagnosis. However, sporadically there have been reports of long-term survivors, either with unusual manifestations of amyloidosis, or after concerted chemotherapy to suppress the overt or occult pathological monoclonal plasma cell population responsible for the elaboration of immunoglobulin light chains. We report the case of a 46-year-old man who has survived 21 years after the histological diagnosis of renal amyloidosis was made, after he had presented with severe nephrotic syndrome...
August 1996: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
C Duvic, B Viron, C Michel, F Mignon
Nowadays, maintenance dialysis can be proposed to patients suffering from myeloma with end-stage chronic renal failure. We report here data from eight patients dialysed either by hemo- (6) or peritoneal dialysis (2), together with chemotherapy in half of them. Six patients died; the longest survival has been about 6 years. The main cause of morbidity was sepsis, especially in peritoneal dialysis patients; therefore we now favour hemodialysis in patients exposed to aggressive chemotherapy. We think dialysis justified in all cases, including those with high tumor mass, in order to expect the effect of chemotherapy; then, provided good response to drugs, further survival can be consistently improved...
1993: La Revue de Médecine Interne
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