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Autologous stem cell transplant amyloidosis

S Rosengren, U-H Mellqvist, H Nahi, K Forsberg, S Lenhoff, O Strömberg, L Ahlberg, O Linder, K Carlson
High-dose melphalan and autologous stem cell transplantation (HDM/ASCT) is widely used in immunoglobulin light chain (AL) amyloidosis, but the benefit is debated mainly because of the high treatment-related mortality (24% in a randomised study comparing HDM/ASCT with oral melphalan/dexamethasone). We report here on the long-term outcome of all patients treated with HDM/ASCT for AL amyloidosis in Sweden between 1994 and 2009. Seventy-two patients were treated at eight Swedish centres. Median follow-up was 67...
October 3, 2016: Bone Marrow Transplantation
Toshiharu Ueno, Keiichi Sumida, Junichi Hoshino, Tatsuya Suwabe, Koki Mise, Ryo Hazue, Noriko Hayami, Rikako Hiramatsu, Masahiro Kawada, Aya Imafuku, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Keiichi Kinowaki, Kenichi Ohashi, Takeshi Fujii, Aya Nishida, Yoshifumi Ubara
A 57-year-old Japanese woman with a 5-year history of rheumatoid arthritis (RA) was admitted to our hospital for an evaluation of nephrotic range proteinuria (4.8 g/day). A renal biopsy led to the diagnosis of amyloidosis according to strong positivity for Congo red staining and the detection of microfibrillar structures on electron microscopy that were negative for AA and positive for kappa light chain. Combination therapy with high-dose melphalan and autologous stem cell transplantation was performed according to the regimen for AL amyloidosis...
2016: Internal Medicine
Hans L A Nienhuis, Johan Bijzet, Bouke P C Hazenberg
BACKGROUND: Amyloidosis has been a mystery for centuries, but research of the last decennia has clarified many of the secrets of this group of diseases. A protein-based classification of amyloidosis helps to understand problems that were part of the obsolete clinical classification in primary, secondary, and familial amyloidosis. All types of amyloid are secondary to some underlying precursor-producing process: each type is caused by a misfolded soluble precursor protein that becomes deposited as insoluble amyloid fibrils...
April 2016: Kidney Diseases
Xiang-Hua Huang, Zhi-Hong Liu
BACKGROUND: Amyloidosis includes a group of diseases characterized by the extracellular deposition of various fibrillary proteins that can autoaggregate in a highly abnormal fibrillary conformation. The amyloid precursor protein of systemic light-chain (AL) amyloidosis is comprised of monoclonal light chains that are due to plasma cell dyscrasia. The clinical presentation of patients with AL amyloidosis varies from patient to patient. Current treatment strategies target the clone in order to decrease the production of the pathologic light chains...
April 2016: Kidney Diseases
Taxiarchis V Kourelis, Samih H Nasr, Angela Dispenzieri, Shaji K Kumar, Morie A Gertz, Fernando C Fervenza, Francis K Buadi, Martha Q Lacy, Stephen B Erickson, Fernando G Cosio, Prashant Kapoor, John A Lust, Suzanne R Hayman, Vincent Rajkumar, Steven R Zeldenrust, Stephen J Russell, David Dingli, Yi Lin, Wilson Gonsalves, Elizabeth C Lorenz, Ladan Zand, Robert A Kyle, Nelson Leung
Recent reports suggest that deep hematologic responses to chemotherapy are associated with improved renal outcomes in monoclonal immunoglobulin deposition disease (MIDD). Here we describe the long term outcomes and identify prognostic factors after first line treatment of the largest reported series of patients with MIDD. Between March 1992 and December 2014, 88 patients with MIDD were seen at Mayo Clinic, MN. Renal responses were defined using criteria used for light chain amyloidosis (AL) or those used by the IMWG...
August 8, 2016: American Journal of Hematology
Rajshekhar Chakraborty, Eli Muchtar, Morie A Gertz
The heart and the kidneys are the most commonly involved organs in systemic amyloidosis. Cardiac involvement is associated with an increased morbidity, treatment intolerance, and poorer overall survival. The most common types of amyloidosis that are associated with cardiac involvement include light chain (AL) amyloidosis and transthyretin (TTR) amyloidosis (both mutant and wild type). The traditional first-line treatment for AL amyloidosis includes alkylator-based chemotherapy or high-dose melphalan followed by autologous stem cell transplantation (ASCT)...
August 6, 2016: Current Heart Failure Reports
Eli Muchtar, Angela Dispenzieri, Shaji K Kumar, David Dingli, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Nelson Leung, Rajshekhar Chakraborty, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz
Clinical tools to guide in the appropriate treatment selection in immunoglobulin light chain (AL) amyloidosis are not well developed. We evaluated the response and outcome for various regimens at first-line treatment (n=681) and first progression (n=240) stratified by the immunoparesis status at diagnosis. Immunoparesis was assessed by the average relative difference of the uninvolved immunoglobulins, classifying patients into a negative average relative difference (i.e. significant immunoparesis) or a positive average relative difference (no/modest immunoparesis)...
September 2016: Haematologica
Shojiro Haji, Junichi Kiyasu, Yoshimichi Tachikawa, Jiro Toyonaga, Motohiko Ikeda, Mariko Tsuda, Yasuhiro Tsukamoto, Mitsuo Kozuru, Yuji Yufu
Light-chain deposition disease (LCDD) is a rare plasma cell neoplasm that secretes an abnormal immunoglobulin light chain, which is deposited in tissues, leading to organ dysfunction. Spontaneous splenic rupture is a rare and life-threatening complication of treatment with granulocyte colony-stimulating factor (G-CSF). Herein, we describe spontaneous splenic rupture after the administration of lenograstim to a patient with LCDD undergoing autologous stem cell transplantation (ASCT). The patient was successfully treated by transcatheter embolization of the splenic artery, and long-term stringent complete remission was attained...
June 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Martha Grogan, Morie Gertz, Arleigh McCurdy, Lindsey Roeker, Robert Kyle, Sudhir Kushwaha, Richard Daly, Joseph Dearani, Richard Rodeheffer, Robert Frantz, Martha Lacy, Suzanne Hayman, Christopher McGregor, Brooks Edwards, Angela Dispenzieri
AIM: To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis. METHODS: The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness > 15 mm, ejection fraction < 40%. Selection guidelines for heart transplant included age < 60 years, absence of multiple myeloma and significant extra-cardiac organ involvement...
June 24, 2016: World Journal of Transplantation
Yi L Hwa, Shaji K Kumar, Morie A Gertz, Martha Q Lacy, Francis K Buadi, Taxiarchis V Kourelis, Wilson I Gonsalves, S Vincent Rajkumar, Ronald S Go, Nelson Leung, Prashant Kapoor, David Dingli, Robert A Kyle, Stephen Russell, John A Lust, Suzanne R Hayman, Yi Lin, Steven Zeldenrust, Angela Dispenzieri
There is no consensus on whether patients with immunoglobulin light chain amyloidosis (AL) should receive induction therapy prior to an autologous stem cell transplant (ASCT). This study investigated the relationships between baseline bone marrow plasmacytosis (BMPC), cardiac staging, and pre-transplant induction in AL patients. All patients who received ASCT for AL within 12 months of diagnosis were included. Patient characteristics and outcomes were abstracted. Univariate and multivariate modeling was performed...
October 2016: American Journal of Hematology
Christopher Dittus, Nsabimana Uwumugambi, Fangui Sun, J Mark Sloan, Vaishali Sanchorawala
The prognosis in light chain (AL) amyloidosis has been linked to several variables, which are primarily related to end-organ damage. Recently, bone marrow plasma cell (BMPC) burden >10% has also been described as an adverse prognostic factor. We reviewed data pertaining to 546 patients with AL amyloidosis who underwent high-dose melphalan (HDM) and stem cell transplantation (SCT) to determine if BMPC > 10% was a negative prognostic factor. Of these patients, 445 had a BMPC burden ≤ 10% and 101 had a BMPC burden > 10%...
September 2016: Biology of Blood and Marrow Transplantation
Tilmann Bochtler, Ute Hegenbart, Christina Kunz, Axel Benner, Christoph Kimmich, Anja Seckinger, Dirk Hose, Hartmut Goldschmidt, Martin Granzow, Peter Dreger, Anthony D Ho, Anna Jauch, Stefan O Schönland
Cytogenetic aberrations detected by interphase fluorescence in situ hybridization (iFISH) of plasma cells are routinely evaluated as prognostic markers in multiple myeloma. This long-term follow-up study aimed to assess the prognosis of systemic light chain amyloidosis (AL) patients treated with high-dose melphalan (HDM) chemotherapy and autologous stem cell transplantation, depending on iFISH results. Therefore, we analyzed a consecutive cohort of 123 AL patients recruited from 2003 to 2014. HDM was safe, with only 1 of 123 patients dying as a result of treatment-related mortality, and effective, with a complete remission (CR) rate of 34%...
July 28, 2016: Blood
Mayada Elnegouly, Katja Specht, Heinz Zoller, Edouard Matevossian, Florian Bassermann, Andreas Umgelter
 Hepatic involvement in AL amyloidosis may present as acute liver failure. Historically, liver transplantation in these cases has achieved poor outcomes due to progress of amyloidosis and non-hepatic organ damage. In the era of bortezomib treatment, the prognosis of AL amyloidosis has been markedly improved and may also result in better post-transplant outcomes. We present a case of isolated acute liver failure caused by AL amyloidosis, bridged to transplantation with bortezomib and treated with sequential orthotopic liver transplantation (OLT) and autologous stem cell transplantation...
July 2016: Annals of Hepatology
S Y Lee, R S Meehan, D C Seldin, J M Sloan, K Quillen, A Shelton, D Brauneis, V Sanchorawala
High-dose melphalan with stem cell transplantation (HDM/SCT) extends survival and induces hematologic and clinical responses in patients with light chain (AL) amyloidosis. Eighty percent of melphalan is bound to plasma proteins (60% albumin-bound). We hypothesized that patients with profound hypoalbuminemia have a greater free melphalan fraction and more toxicity. Patients with AL amyloidosis treated with HDM/SCT between 2011 and 2014 with severe hypoalbuminemia (SH), defined as serum albumin ⩽2 g/dL were studied retrospectively...
May 16, 2016: Bone Marrow Transplantation
Morie A Gertz, Martha Q Lacy, Angela Dispenzieri, Francis K Buadi, David Dingli, Suzanne R Hayman, Shaji K Kumar, Nelson Leung, John Lust, S Vincent Rajkumar, Stephen J Russell, Vera J Suman, Jennifer G Le-Rademacher, William J Hogan
BACKGROUND: Autologous stem cell transplantation (SCT) is a common management strategy for select patients with immunoglobulin light-chain amyloidosis, but no trials have documented improved overall survival. METHODS: Eighty-nine patients with biopsy-proven immunoglobulin light-chain amyloidosis were allowed to select treatment with melphalan plus dexamethasone (n = 34) or SCT (n = 55); all patients were transplant eligible. Treatment preference resulted in imbalanced study arms...
July 15, 2016: Cancer
J Li, J Feng, X X Cao, C L Zhang, K N Shen, X F Huang, C L Zhang, M H Duan, W Zhang, T N Zhu, H C Cai, L Zhang, H Cai, D B Zhou
OBJECTIVE: To evaluate the efficacy and safety of autologous peripheral blood hematopoietic stem cell transplantation (ASCT) for patients with primary light chain (AL) amyloidosis. METHODS: Clinical data, hematological and organ response, safety and survival status of 31 patients with AL amyloidosis who had received ASCT from January 2009 to June 2015 were retrospectively analyzed. RESULTS: Among 31 patients, there were 18 males and 13 females with the median age of 55 (range, 43-66) years old...
March 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Frank Bridoux, Vincent Javaugue, Jean Paul Fermand, Arnaud Jaccard
No abstract text is available yet for this article.
August 2016: Nephrology, Dialysis, Transplantation
J R Passweg, H Baldomero, P Bader, C Bonini, S Cesaro, P Dreger, R F Duarte, C Dufour, J Kuball, D Farge-Bancel, A Gennery, N Kröger, F Lanza, A Nagler, A Sureda, M Mohty
A record number of 40 829 hematopoietic stem cell transplantation (HSCT) in 36 469 patients (15 765 allogeneic (43%), 20 704 autologous (57%)) were reported by 656 centers in 47 countries to the 2014 survey. Trends include: continued growth in transplant activity, more so in Eastern European countries than in the west; a continued increase in the use of haploidentical family donors (by 25%) and slower growth for unrelated donor HSCT. The use of cord blood as a stem cell source has decreased again in 2014...
June 2016: Bone Marrow Transplantation
Pankaj Mathur, Hakan Paydak, Sharmilan Thanendrarajan, Frits van Rhee
Atrial fibrillation (AF) is the most common cardiac arrhythmia and is associated with significant morbidity and mortality worldwide. In addition to well-established risk factors, cancer has been increasingly associated with the development of AF. Its increased occurrence in those with hematologic malignancies has been attributed to chemotherapeutic agents and autologous hematopoietic stem cell transplantation (AHSCT). Recently, a few studies have attempted to define the etiopathogenesis of AF in hematologic malignancies...
February 2016: Clinical Lymphoma, Myeloma & Leukemia
Talha Badar, Amanda Megan Cornelison, Nina D Shah, Qaiser Bashir, Simrit Parmar, Krina Patel, Chitra Hosing, Uday Popat, Donna M Weber, Sheeba K Thomas, Jatin J Shah, Robert Z Orlowski, Richard E Champlin, Muzaffar H Qazilbash
Cardiac involvement in systemic light chain amyloidosis (AL) is generally associated with a worse outcome, especially if other organs are also involved. We sought to determine whether concurrent cardiac and renal involvement were associated with a worse outcome than either organ alone. We identified 129 patients with AL, who received high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (auto-HCT) at our institution between 1997 and 2014. Ninety-nine patients had either renal (group 1: n = 62, 62%), cardiac (group 2: n = 20, 20%), or both cardiac and renal (group 3: n = 17, 17%) involvement...
October 2016: European Journal of Haematology
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