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AL amyloidosis treatment

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https://www.readbyqxmd.com/read/29991549/multisystem-amyloidosis-as-the-unifying-diagnosis-for-constipation-collapse-and-cardiomyopathy
#1
Michael McFarlane, Alexander Bashford, Shatrughan Sah, Ben R Disney
Amyloidosis a rare disorder characterised by the deposition of amyloid protein aggregates in different organ systems throughout the body with resulting functional impairment of affected organs. It can present with localised or multisystemic deposits. Diagnosis is often delayed due to the non-specific nature of the symptoms. We present the case of a 59-year-old man with a 12-month history of non-specific symptoms. Investigations revealed Helicobacter pylori positive gastritis. Blood tests showed only a normocytic anaemia and thrombocytopaenia...
July 10, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29957672/light-chain-amyloidosis-presenting-as-bilateral-external-auditory-canal-obstructing-masses
#2
Shenon Sethi, Agne Paner, Tochukwu Okwuosa, Danny Jandali, Sumeet Dua, Richard M Wiet, Ritu Ghai
: A morbidly-obese 57-year-old diabetic and hypertensive man with chronic kidney disease, diastolic heart failure, and bilateral hearing loss was found to have soft tissue masses/densities in the lateral aspect of both auditory canals on local examination and on imaging. He underwent biopsies of both ear canal masses and histologic examination revealed amyloid deposits in the dermis. These deposits were confirmed as AL (amyloid light chain) kappa-type amyloid by laser mass spectrometry. A systemic work-up showed plasma cell dyscrasia with 9% kappa light chain restricted plasma cells in the bone marrow as well as amyloid deposits on a kidney biopsy...
June 27, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29954616/-clinical-radiological-pathological-features-treatment-and-follow-up-of-periocular-and-or-orbital-amyloidosis-report-of-6-cases-and-literature-review
#3
A Martel, A Oberic, A Moulin, N Tieulie, M Hamedani
PURPOSE: To assess demographic, clinical, radiological, pathological features, treatment and follow-up of periocular or/and orbital amyloidosis. PATIENTS AND METHODS: We conducted an observational retrospective monocentric study from January 2004 to April 2017 in patients diagnosed with histologically proven periocular or/and orbital amyloidosis. RESULTS: Six patients were included (2 females, 4 males). Mean age was 76.8 years (range 66-88 years)...
June 25, 2018: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29952963/beh%C3%A3-et-disease-associated-with-gastrointestinal-amyloidosis-manifested-as-hematochezia-a-case-report
#4
Shuzo Sato, Makiko Yashiro, Naoki Matsuoka, Satoshi Kawana, Tomoyuki Asano, Hiroko Kobayashi, Kazuhiro Tasaki, Hiroshi Watanabe, Yuko Hashimoto, Kiyoshi Migita
RATIONALE: Behçet disease (BD) is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, ocular lesions, and skin lesions. Complication of amyloidosis in patients with BD is rare. Here, we report a case of BD with immunoglobulin light chain (AL)-amyloidosis manifested as hematochezia. PATIENT CONCERNS: A 61-year-old man developed sudden hematochezia due to bleeding from multiple small colonic ulcers; AL-amyloid deposition was found on immunohistochemical examination of biopsy specimen of colonic ulcer...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29951831/novel-approaches-for-the-management-of-al-amyloidosis
#5
REVIEW
Nisha S Joseph, Jonathan L Kaufman
PURPOSE OF REVIEW: Light-chain-associated (AL) amyloidosis is a rare disease with a poor prognosis. However, we have made recent strides in more accurate diagnosis and effective treatment. Here, we discuss the most recent updates and advancements during the past year in the diagnosis, prognostication, and management of AL amyloidosis both in the upfront and relapsed setting. RECENT FINDINGS: New imaging modalities, such as cardiac magnetic resonance (CMR) and use of fluorine-labeled radiotracers, are emerging as an important diagnostic tool in conjunction with biomarkers in the diagnosis, prognosis, and monitoring of the effects of therapy...
June 2018: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/29933072/modified-high-dose-melphalan-and-autologous-stem-cell-transplantation-for-al-amyloidosis-experience-in-334-patients
#6
Vina P Nguyen, Heather Landau, Karen Quillen, Dina Brauneis, Anthony C Shelton, Lisa Mendelson, Hafsa Rahman, J Mark Sloan, Shayna Sarosiek, Vaishali Sanchorawala
High dose melphalan and autologous stem cell transplantation (HDM/SCT) has been used in patients with light chain (AL) amyloidosis for over two decades now with durable responses, prolonged survival, and decreasing treatment related mortality. Historically patients with poorer baseline functional status, advanced age, renal compromise and cardiac involvement have been treated with a risk-adapted modified conditioning dose of melphalan (mHDM) of 100-140 mg/m2 prior to SCT. Due in part to these baseline characteristics, patients receiving mHDM/SCT have had poorer outcomes compared to patients receiving full dose of melphalan at 200 mg/m2 ...
June 19, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29933071/autologous-stem-cell-transplant-for-al-amyloidosis-patients-aged-70-to-75
#7
M Hasib Sidiqi, Mohammed A Aljama, Eli Muchtar, Francis K Buadi, Rahma Warsame, Martha Q Lacy, Angela Dispenzieri, David Dingli, Nelson Leung, Wilson I Gonsalves, Prashant Kapoor, Taxiarchis V Kourelis, William J Hogan, Shaji K Kumar, Morie A Gertz
Autologous stem cell transplantation has been used in treatment for AL amyloidosis for over two decades and generally reserved for patients younger than 70 years of age. Herein we report on outcomes of ASCT in a cohort of patients with AL amyloidosis aged 70 years or older. Between August of 2002 and April of 2017, 34 patients aged 70 years or older, with biopsy proven AL amyloidosis, received an autologous stem cell transplant at the Mayo Clinic Rochester. 70% (n=24) of patients were transplanted within 6 months of diagnosis and 74% (n=25) received reduced intensity conditioning with melphalan <200mg/m2...
June 19, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29890159/ocular-adnexal-amyloidosis-a-mass-spectrometric-analysis
#8
Alexander D Blandford, Sari Yordi, Saloni Kapoor, Gabrielle Yeaney, Claudiu V Cotta, Jason Valent, Julian D Perry, Arun D Singh
PURPOSE: Ocular adnexal amyloidosis (OAA) may represent localized manifestation of an underlying systemic process. Accurate identification of the amyloid fibrils can guide the systemic evaluation and treatment. The aim of this study was to characterize subtypes of OAA using immunohistochemistry and mass spectrometric analysis and to correlate with ocular involvement and systemic association. DESIGN: Retrospective case series. METHODS: Review of patients with OAA subtyped by immunohistochemistry and mass spectrometric analysis at the Cleveland Clinic from June 1995 to June 2017...
June 8, 2018: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/29889595/external-beam-radiation-for-localized-periocular-amyloidosis-a-case-series
#9
Thomas S Copperman, Minh Tam Truong, John L Berk, Rachel K Sobel
PURPOSE: To evaluate the effectiveness of low dose external beam radiation therapy to halt progression of localized periocular light chain (AL) amyloidosis, a clonal plasma cell disorder. METHODS: This is a retrospective review of patients referred to a tertiary care center for external beam radiation treatment of biopsy proven localized periocular light chain amyloidosis. The primary outcome measure was clinical disease stability at one year following radiation therapy as evidenced by slit lamp exam and external photography...
June 11, 2018: Orbit
https://www.readbyqxmd.com/read/29795248/immunoglobulin-light-chain-amyloidosis-diagnosis-and-treatment-algorithm-2018
#10
REVIEW
Morie A Gertz
Immunoglobulin light chain amyloidosis (AL) should be considered in any patient that presents to a cancer care provider with nephrotic range proteinuria, heart failure with preserved ejection fraction, non-diabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea. More importantly, patients being monitored for smoldering multiple myeloma and a monoclonal gammopathy of undetermined significance (MGUS) are at risk for developing AL amyloidosis. MGUS and myeloma patients that have atypical features, including unexplained weight loss; lower extremity edema, early satiety, and dyspnea on exertion should be considered at risk for light chain amyloidosis...
May 23, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29791180/-heart-transplantation-and-follow-up-treatment-with-al-amyloidosis-in-5-patients
#11
Zdeněk Adam, Eva Ozábalová, Petr Němec, Helena Bedáňová, Milan Kuman, Jan Krejčí, Lenka Špinarová, Víta Žampachová, Zdeňka Čermáková, Luděk Pour, Marta Krejčí, Viera Sanecká, Martin Štork, Tomáš Pika, Jan Straub, Dagmar Adamová, Yvetta Stavařová, Zdeněk Král, Jiří Mayer
The prognosis for patients with cardiac impairment due to AL-amyloid deposition and severe cardiac insufficiency is poor, with a survival median in the order of months. The classical treatment of AL-amyloidosis in combination with cardiac insufficiency is very poorly tolerated and the treatment of such patients is associated with considerably higher mortality than among other patients with AL-amyloidosis. If, however, patients with an isolated or another dominating cardiac impairment, without severe damage to other organs and tissues, have a heart transplant performed, their cardiovascular condition will significantly improve as a result, along with their ability to tolerate any kind of treatment for AL-amyloidosis including that using high-dose chemotherapy with a transplant of autologous hematopoietic stem cells...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29791070/role-of-oral-examination-in-newly-diagnosed-multiple-myeloma-patients-a-safe-and-simple-way-to-detect-light-chain-amyloidosis
#12
Merav Leiba, Suha Jarjoura, Waseem Abboud, Arnon Nagler, Ran Yahalom, Adrian Duek, Noam Yarom
OBJECTIVE: Up to 30% of multiple myeloma (MM) patients have subclinical amyloid deposits. These patients are under-recognized and are more susceptible to drug toxicity, bleeding and death. Early diagnosis and adjustment of treatment are crucial. Biopsies of oral mucosa might be a potentially useful diagnostic tool. The objective of the present study was to assess the prevalence and characteristics at presentation of oral amyloidosis in a large cohort of MM patients. METHODS: The prevalence and characteristics of oral amyloidosis in a large cohort of MM patients who were referred for oral evaluation before and during bisphosphonate therapy were assessed, retrospectively...
May 23, 2018: Oral Diseases
https://www.readbyqxmd.com/read/29748430/epidemiology-of-al-amyloidosis-a-real-world-study-using-us-claims-data
#13
Tiffany P Quock, Tingjian Yan, Eunice Chang, Spencer Guthrie, Michael S Broder
Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States. Using claims databases from years 2007 to 2015, adults ≥18 years old with AL amyloidosis were included if they had (1) at least 1 inpatient or 2 outpatient claims consistent with AL amyloidosis and (2) received 1 AL-specific treatment. Prevalence was calculated as the number of AL patients divided by the number of enrollees on June 30th of each calendar year...
May 22, 2018: Blood Advances
https://www.readbyqxmd.com/read/29709420/outcome-of-patients-with-cardiac-amyloidosis-admitted-to-an-intensive-care-unit-for-acute-heart-failure
#14
Thomas d'Humières, Damien Fard, Thibaud Damy, Francois Roubille, Arnaud Galat, Huy-Long Doan, Leopold Oliver, Jean-Luc Dubois-Randé, Pierre Squara, Pascal Lim, Julien Ternacle
BACKGROUND: The outcome of cardiac amyloidosis (CA) has been reported mainly in stable populations; limited data are available in patients referred for acute heart failure (AHF) to an intensive cardiac care unit (ICCU). AIMS: To address the characteristics and outcomes of patients with confirmed CA admitted to an ICCU for AHF and then to identify the predictors of evolution to cardiogenic shock. METHODS: All patients with CA referred to an ICCU for AHF between 2009 and 2015 were included...
April 27, 2018: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/29706127/utility-of-the-18-f-florbetapir-positron-emission-tomography-in-systemic-amyloidosis
#15
Jaume Mestre-Torres, Carles Lorenzo-Bosquet, Gemma Cuberas-Borrós, Mercedes Gironella, Roser Solans-Laque, Andreu Fernández-Codina, Segundo Bujan-Rivas, Joan Castell-Conesa, Fernando Martínez-Valle
Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using 18 F-florbetapir. We hypothesize that combining whole body PET/CT with 18 F-Florbetapir can be useful to characterize the burden of the disease. We included 25 patients, 13 of them with different types of amyloidosis, and 12 with Alzheimer's disease as controls...
April 28, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29696194/rationale-and-design-of-dual-study-doxycycline-to-upgrade-response-in-light-chain-al-amyloidosis-dual-a-phase-2-pilot-study-of-a-two-pronged-approach-of-prolonged-doxycycline-with-plasma-cell-directed-therapy-in-the-treatment-of-al-amyloidosis
#16
Anita D'Souza, Kathryn Flynn, Saurabh Chhabra, Binod Dhakal, Mehdi Hamadani, Kirsten Jacobsen, Marcelo Pasquini, Dorothee Weihrauch, Parameswaran Hari
Light chain (AL) amyloidosis is a plasma cell neoplasm associated with insoluble fibril deposition from clonal immunoglobulin chains systemically. The disease is associated with high early mortality and morbidity owing to advanced organ deposition as well as lack of proven de-fibrillogenic therapies. Pre-clinical and retrospective clinical data suggests that doxycycline has benefit in AL amyloidosis. The ongoing DUAL study is a single center, open label, phase 2 study in which patients with AL amyloidosis who are undergoing clone-directed therapy for the underlying neoplasm with oral doxycycline given for 1 year to test the hypothesis that prolonged doxycycline use will be safe, feasible, and lead to reduced early mortality in systemic AL amyloidosis and hasten organ amyloid response...
December 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/29692592/revisiting-renal-amyloidosis-with-clinicopathological-characteristics-grading-and-scoring-a-single-institutional-experience
#17
Abhiram Kalle, Archana Gudipati, Sree Bhushan Raju, Karthik Kalidindi, Swarnalatha Guditi, Gangadhar Taduri, Megha S Uppin
INTRODUCTION: Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Renal biopsy helps in diagnosis and also predicting the clinical course by applying scoring and grading to the biopsy findings. MATERIALS AND METHODS: The study includes all cases of biopsy-proven renal amyloidosis from January 2008 to May 2017...
April 2018: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/29660165/usefulness-of-gastroduodenal-biopsy-in-the-differential-diagnosis-of-systemic-ah-amyloidosis-from-systemic-al-amyloidosis
#18
Shojiro Ichimata, Mikiko Kobayashi, Hisashi Shimojo, Nagaaki Katoh, Masahide Yazaki, Hiroyuki Kanno
AIMS: The aim of this study is to examine the usefulness of gastroduodenal biopsy for the detection of immunoglobulin (Ig) heavy-chain amyloid deposition. Ig heavy-chain amyloidosis (AH amyloidosis) is Ig-related amyloidosis classified together with Ig light-chain amyloidosis (AL amyloidosis). Compared with AL amyloidosis, patients with AH amyloidosis exhibit a better prognosis and they may not need an aggressive treatment. Thus, the accurate diagnosis is essential for management of Ig-related amyloidosis patients...
April 16, 2018: Histopathology
https://www.readbyqxmd.com/read/29651545/extracardiac-18-f-florbetapir-imaging-in-patients-with-systemic-amyloidosis-more-than-hearts-and-minds
#19
T Wagner, J Page, M Burniston, A Skillen, J C Ross, R Manwani, D McCool, P N Hawkins, Ashutosh D Wechalekar
PURPOSE: 18 F-Florbetapir has been reported to show cardiac uptake in patients with systemic light-chain amyloidosis (AL). This study systematically assessed uptake of 18 F-florbetapir in patients with proven systemic amyloidosis at sites outside the heart. METHODS: Seventeen patients with proven cardiac amyloidosis underwent 18 F-florbetapir PET/CT imaging, 15 with AL and 2 with transthyretin amyloidosis (ATTR). Three patients had repeat scans. All patients had protocolized assessment at the UK National Amyloidosis Centre including imaging with 123 I-serum amyloid P component (SAP)...
July 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29627865/advances-in-treatment-of-cardiac-amyloid
#20
REVIEW
Cherie N Dahm, R Frank Cornell, Daniel J Lenihan
Systemic amyloidosis represents a complex group of diseases with a common feature characterized by misfolded autologous proteins depositing into tissues or organs throughout the body. Light chain amyloidosis (AL) and transthyretin (TTR) amyloid are the two most prevalent forms of this disease that commonly results in cardiac amyloidosis. In both of these conditions, the myocardium is a frequent site of infiltration and end-organ involvement often with devastating consequences. With cardiac amyloidosis becoming an increasingly identified disease that has previously been under-recognized, the purpose of this comprehensive review is to focus on the diagnosis and treatment of these two types of cardiac amyloidosis including a contemporary update on currently available therapies being investigated in clinical trials...
April 7, 2018: Current Treatment Options in Cardiovascular Medicine
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