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AL amyloidosis treatment

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https://www.readbyqxmd.com/read/28808991/light-chain-al-amyloidosis-the-journey-to-diagnosis
#1
Kristen L McCausland, Michelle K White, Spencer D Guthrie, Tiffany Quock, Muriel Finkel, Isabelle Lousada, Martha S Bayliss
BACKGROUND: Light chain (AL) amyloidosis is a rare, complex disease associated with significant morbidity and mortality. Delays in diagnosis are common and may have detrimental consequences on patients' prognosis. Too little is known regarding the patient journey to diagnosis. OBJECTIVE: The objective of this study was to describe the patient-reported journey to a correct diagnosis for AL amyloidosis. METHODS: Using a mixed-methods approach, data were collected from clinician (n = 4) and patient (n = 10) interviews and a survey of community-based patients with AL amyloidosis (n = 341)...
August 14, 2017: Patient
https://www.readbyqxmd.com/read/28808514/nodular-pulmonary-amyloidosis-with-primary-pulmonary-malt-lymphoma-masquerading-as-metastatic-lung-disease
#2
Sunil Upadhaya, Mohd Baig, Basim Towfiq, Samer Al Hadidi
Nodular pulmonary amyloidosis is a very rare form of localized amyloidosis involving the lung, with very little known about its nature. It is usually associated with indolent B cell lymphoproliferative disorder and also connective tissue disorders. No definite treatment guideline exists. Many patients respond to chemotherapy with low risk of progression and a 'wait and watch' strategy is also considered a valid treatment option. In this report the authors present a case of nodular pulmonary amyloidosis with pulmonary mucosa associated lymphoid tissue (MALT) lymphoma that presented with features of metastatic malignant disease and after definitive diagnosis decided not to undergo treatment...
July 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28807981/delineation-of-the-timing-of-second-line-therapy-post-autologous-stem-cell-transplant-in-patients-with-al-amyloidosis
#3
Yi L Hwa, Rahma Warsame, Morie A Gertz, Francis K Buadi, Martha Q Lacy, Shaji K Kumar, David Dingli, Steve R Zeldenrust, Nelson Leung, Suzanne R Hayman, Prashant Kapoor, Wilson I Gonsalves, Taxiarchis V Kourelis, Stephen Russell, Ronald S Go, Miriam A Hobbs, Amie L Fonder, S Vincent Rajkumar, Angela Dispenzieri
Among patients with immunoglobulin light chain (AL) amyloidosis, there is little consensus on when reinstitution of chemotherapy should occur. We conducted a retrospective study to evaluate the patterns of relapse or progression (R/P) and the timing of re-initiating therapy among 235 patients initially treated with ASCT at Mayo Clinic. The median time from ASCT to second-line therapy was 24.3 months. At the time of restarting therapy, median dFLC was 9.9 mg/dL (42% of diagnosis value); 32% had a dFLC< 5mg/dL; and 63% met criteria for organ R/P...
August 14, 2017: Blood
https://www.readbyqxmd.com/read/28754546/comparison-of-different-stem-cell-mobilization-regimen-in-al-amyloidosis-patients
#4
Katharina Lisenko, Patrick Wuchter, Marion Hansberg, Anja Mangatter, Axel Benner, Anthony D Ho, Hartmut Goldschmidt, Ute Hegenbart, Stefan Schönland
High-dose melphalan (HDM) and autologous blood stem cell transplantation (ABSCT) is an effective treatment for transplant-eligible patients with systemic light chain (AL) amyloidosis. Whereas most centers use granulocyte colony-stimulating factor (G-CSF) alone for mobilization of peripheral blood stem cells (PBSC), the application of mobilization chemotherapy might offer specific advantages. We retrospectively analyzed 110 patients with AL amyloidosis who underwent PBSC collection. Major eligibility criteria included age <70 years and cardiac insufficiency NYHA ≤III°...
July 25, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#5
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28725274/news-in-al-amyloidosis-ash-2016-a%C3%A2-rapidly-evolving-field-of-investigation
#6
REVIEW
Hermine Agis
Amyloidosis is a rare but life-threatening protein misfolding disease. The early diagnosis and enrollment of patients into multicentre trials is of great importance, as is the need for intensive collaboration between multiple medical departments and experienced specialists. In the following review, the most interesting abstracts from the annual American Society of Hematology (ASH) meeting in 2016 are presented. The topics include the limitations of established biomarkers in risk assessment and response evaluation, the introduction of a new biomarker, the comparison of different treatment sequences and the efficacy of a multiple drug regimen in light-chain (AL) amyloidosis...
2017: Memo
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#7
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
August 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28699793/predictors-of-early-treatment-failure-following-initial-therapy-for-systemic-immunoglobulin-light-chain-amyloidosis
#8
Nidhi Tandon, Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Morie A Gertz, Martha Q Lacy, Robert A Kyle, Francis K Buadi, David Dingli, Suzanne R Hayman, Amie L Fonder, Miriam A Hobbs, Wilson I Gonsalves, Prashant Kapoor, Yi Lisa Hwa, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, Shaji K Kumar
We analysed factors predicting early treatment failure (ETF), after first-line therapy for light-chain amyloidosis (AL). AL amyloidosis patients seen at Mayo Clinic within 90 days of diagnosis, from 2006 to 2015, excluding those who died within 3 months of initial therapy, were analysed retrospectively. ETF was defined as progression requiring treatment change or death within 12 (ETF12) or 24 (ETF24) months of first-line treatment. Non-ETF included those with a follow-up of more than 12 or 24 months who had progression beyond 12 or 24 months...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28693194/combination-of-lenalidomide-and-low-dose-dexamethasone-therapy-promotes-the-anticoagulant-activity-of-warfarin-in-patients-with-immunoglobulin-light-chain-amyloidosis
#9
Fumiaki Kitazawa, Shin-Ichi Fuchida, Fumitaka Ise, Yoko Kado, Kumi Ueda, Takatoshi Kokufu, Akira Okano, Mayumi Hatsuse, Satoshi Murakami, Yuko Nakayama, Kohji Takara, Chihiro Shimazaki
The present study aimed to evaluate the drug interactions between warfarin and combination chemotherapy with lenalidomide and low-dose dexamethasone in immunoglobulin light-chain (AL) amyloidosis patients with unstable international normalized ratios (INR). The changes to INR values over time in 3 AL amyloidosis patients treated with warfarin and a combination of lenalidomide and low-dose dexamethasone between March 2011 and February 2015 were analyzed retrospectively. The mean INR value was 1.52 prior to the combination chemotherapy, and the value increased 1...
July 2017: Oncology Letters
https://www.readbyqxmd.com/read/28682959/paraneoplastic-neuropathies
#10
Jean-Christophe Antoine, Jean-Philippe Camdessanché
PURPOSE OF REVIEW: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. RECENT FINDINGS: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease...
July 5, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28648829/novel-pharmacotherapies-for-cardiac-amyloidosis
#11
REVIEW
Kevin M Alexander, Avinainder Singh, Rodney H Falk
Amyloidosis refers to a range of protein misfolding disorders that can cause organ dysfunction through progressive fibril deposition. Cardiac involvement often leads to significant morbidity and mortality and increasingly has been recognized as an important cause of heart failure. The two main forms of cardiac amyloidosis, light chain (AL) and transthyretin (ATTR) amyloidosis, have distinct mechanisms of pathogenesis. Recent insights have led to the development of novel pharmacotherapies with the potential to significantly impact each disease...
June 22, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28648672/prognostic-significance-of-interventricular-septal-thickness-in-patients-with-al-amyloidosis
#12
Hyunsoo Cho, Soo-Jeong Kim, Chi Young Shim, Geu-Ru Hong, Jong-Won Ha, Yu Ri Kim, Woo Ick Yang, Haerim Chung, Ji Eun Jang, June-Won Cheong, Yoo Hong Min, Jin Seok Kim
The major prognostic determinant of immunoglobulin light chain (AL) amyloidosis is cardiac involvement. However, the role of interventricular septal thickness (IVST), which reflects the extent of cardiac involvement, remains unclear. Therefore, we analyzed 77 patients with newly diagnosed AL amyloidosis and evaluated the prognostic role of IVST. Fifty patients (64.9%) had cardiac involvement and 17 patients (22.1%) showed IVST >15mm. Among all patients, the revised Mayo Clinic Stage III-IV and IVST >15mm were independently associated with inferior overall survival (OS) in a multivariable analysis...
June 16, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#13
P Patrizia Mangione, Giuseppe Mazza, Janet A Gilbertson, Nigel B Rendell, Diana Canetti, Sofia Giorgetti, Luca Frenguelli, Marco Curti, Tamer Rezk, Sara Raimondi, Mark B Pepys, Philip N Hawkins, Julian D Gillmore, Graham W Taylor, Massimo Pinzani, Vittorio Bellotti
Diagnosis and treatment of systemic amyloidosis depend on accurate identification of the specific amyloid fibril protein forming the tissue deposits. Confirmation of monoclonal immunoglobulin light chain amyloidosis (AL), requiring cytotoxic chemotherapy, and avoidance of such treatment in non-AL amyloidosis, are particularly important. Proteomic analysis characterises amyloid proteins directly. It complements immunohistochemical staining of amyloid to identify fibril proteins and gene sequencing to identify mutations in the fibril precursors...
June 21, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#14
Johanna Denman, Kaveh Manavi, Mark Cook
We present a case of an HIV-positive man with systemic immunoglobulin light chain (AL) amyloid with cardiac involvement. At relapse, he was treated with lenalidomide and dexamethasone having previously developed autonomic neuropathy with bortezomib-based chemotherapy. The patient achieved a serological complete response with symptomatic improvement. After 11 cycles, lenalidomide was discontinued due to extensive ischaemia of the gastrointestinal tract. The patient remains symptomatically stable with normal levels of serum-free light chains 11 months after the treatment was discontinued...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28622303/addition-of-cyclophosphamide-and-higher-doses-of-dexamethasone-do-not-improve-outcomes-of-patients-with-al-amyloidosis-treated-with-bortezomib
#15
E Kastritis, M Gavriatopoulou, M Roussou, D Fotiou, D C Ziogas, M Migkou, E Eleutherakis-Papaiakovou, I Panagiotidis, N Kanellias, E Psimenou, E Papadopoulou, C Pamboucas, E Manios, H Gakiopoulou, A Ntalianis, A Tasidou, S Giannouli, E Terpos, M A Dimopoulos
Bortezomib, in combination with dexamethasone (VD) or with the addition of cyclophosphamide (VCD), is highly effective in patients with amyloid light-chain (AL) amyloidosis. Currently, VCD is considered as a primary regimen for patients with AL, but it is not clear whether the addition of cyclophosphamide to VD further and significantly improves efficacy, given the substantial activity of bortezomib itself. We retrospectively compared the outcomes of 101 patients with AL amyloidosis who received VD (n=59) or VCD (n=42) in two consecutive periods...
June 16, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28616564/regression-of-cardiac-amyloidosis-following-stem-cell-transplantation-a-comparison-between-echocardiography-and-cardiac-magnetic-resonance-imaging-in-long-term-survivors
#16
Benjamin Thomas Fitzgerald, John Bashford, Katrina Newbigin, Gregory Malcolm Scalia
BACKGROUND: AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillary protein in tissue and organs. Untreated median survival is very poor, and even worse with cardiac involvement. Chemotherapy and peripheral blood stem cell transplantation (PBSCT) have been shown to dramatically improve survival, with hematologic remission documented. Regression of cardiac changes has previously been shown, as assessed by echocardiography (TTE) and cardiac magnetic resonance imaging (CMR)...
March 2017: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/28615223/daratumumab-yields-rapid-and-deep-hematologic-responses-in-patients-with-heavily-pretreated-al-amyloidosis
#17
Gregory P Kaufman, Stanley L Schrier, Richard A Lafayette, Sally Arai, Ronald M Witteles, Michaela Liedtke
The majority of patients with immunoglobulin light chain amyloidosis (AL) fail to achieve a complete response (CR) to standard light chain suppressive chemotherapy, and almost all patients eventually experience hematologic relapse and progression of organ involvement; additional well tolerated treatment options are therefore needed. We present our retrospective experience of 25 consecutive previously treated AL patients who received daratumumab, a CD38-directed monoclonal antibody approved for the treatment of multiple myeloma...
June 14, 2017: Blood
https://www.readbyqxmd.com/read/28611935/gastrointestinal-amyloidosis-review-of-the-literature
#18
REVIEW
Kyle Rowe, Jon Pankow, Fredy Nehme, William Salyers
Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. GIA can be acquired or genetic, and most commonly results from chronic inflammatory disorders (AA amyloidosis), hematologic malignancy (AL amyloidosis), and end-stage renal disease (Beta-2 amyloidosis)...
May 8, 2017: Curēus
https://www.readbyqxmd.com/read/28579588/a-case-of-primary-amyloidosis-with-spontaneous-hepatic-rupture
#19
Tsuyoshi Suda, Hikaru Oguri
A 79-year-old man was diagnosed to have primary amyloid light-chain (AL) amyloidosis with associated liver damage and prominent hepatomegaly. He was followed up without any treatment. One year after the diagnosis, he was taken to the hospital with a sudden onset of features of shock. Computed tomography revealed hepatic rupture, and he was treated by emergent transcutaneous arterial embolization. However, the procedure was unable to save his life. AL amyloidosis with prominent hepatomegaly is considered to be a risk factor for spontaneous liver rupture and intra-abdominal hemorrhage...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/28546074/outcomes-from-autologous-hematopoietic-cell-transplantation-versus-chemotherapy-alone-for-the-management-of-light-chain-amyloidosis
#20
Oluchi Oke, Tarsheen Sethi, Stacey Goodman, Sharon Phillips, Ilka Decker, Samuel Rubinstein, Beatrice Concepcion, Sarah Horst, Madan Jagasia, Adetola Kassim, Shelton L Harrell, Anthony Langone, Daniel Lenihan, Kyle T Rawling, David Slosky, Robert Frank Cornell
Light chain amyloidosis (AL) results in tissue deposition of misfolded proteins, causing organ dysfunction. In an era of modern therapies, such as bortezomib, reassessment of the benefit of autologous hematopoietic cell transplantation (AHCT) should be considered. In this study, we compared outcomes between patients with AL receiving chemotherapy alone (CT) and those undergoing AHCT. Seventy-four patients with AL were analyzed retrospectively. Two cohorts of patients were studied, those receiving CT (n = 31) and those undergoing AHCT (n = 43)...
September 2017: Biology of Blood and Marrow Transplantation
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