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AL amyloidosis treatment

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https://www.readbyqxmd.com/read/30232705/chronic-intestinal-pseudo-obstruction-due-to-al-amyloidosis-a-case-report-and-literature-review
#1
Tomoya Iida, Daisuke Hirayama, Gota Sudo, Kei Mitsuhashi, Hisayoshi Igarashi, Kentaro Yamashita, Hiroo Yamano, Hiroshi Nakase
A 59-year-old woman presented to our hospital with a 6-month history of nausea, weight loss, and abdominal distension. Physical examination revealed abdominal distension without tenderness, and edema, numbness, and multiple peripheral neuropathy in the limbs. Blood test results showed anemia, hypoproteinemia, and hypoalbuminemia. Immunoelectrophoresis detected kappa-type Bence-Jones protein in both the serum and urine. Bone marrow examination did not reveal an increase of plasma cells. Computed tomography showed intestinal distension and retention of intestinal contents...
September 19, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/30228867/recent-advances-in-understanding-and-treating-immunoglobulin-light-chain-amyloidosis
#2
REVIEW
Talha Badar, Anita D'Souza, Parameswaran Hari
Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical conditions, AL amyloidosis is often diagnosed late and results in dismal outcomes. Early referral to a specialized center with expertise in management of AL amyloidosis is always recommended. The availability of sensitive biomarkers and novel therapies is reforming our approach to how we manage AL amyloidosis...
2018: F1000Research
https://www.readbyqxmd.com/read/30223349/therapies-for-cardiac-light-chain-amyloidosis-an-update
#3
Alberto Aimo, Gabriele Buda, Marianna Fontana, Andrea Barison, Giuseppe Vergaro, Michele Emdin, Giampaolo Merlini
Light-chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. This serious disorder is characterized by the presence of a clone of bone marrow plasma cells that produces monoclonal light chains (LCs) of the κ or predominantly λ type. These amyloidogenic LCs undergo extracellular misfolding and aggregation into proteotoxic soluble oligomers and amyloid fibrils that deposit within tissues. The lethal consequences of AL amyloidosis are due to the toxic products (the LCs) and not to the malignant behaviour of the plasma cell clone...
November 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/30169860/the-clinical-implication-of-monoclonal-gammopathies-monoclonal-gammopathy-of-undetermined-significance-and-monoclonal-gammopathy-of-renal-significance
#4
Krzysztof Batko, Jolanta Malyszko, Artur Jurczyszyn, David H Vesole, Morie A Gertz, Xavier Leleu, Anna Suska, Marcin Krzanowski, Wladyslaw Sulowicz, Jacek S Malyszko, Katarzyna Krzanowska
Monoclonal gammopathy of renal significance (MGRS) has introduced a new perspective to several well-known disease entities impacting nephrology, haematology and pathology. Given the constantly changing disease spectrum of these entities, it is clinically imperative to establish diagnostic and treatment pathways supported by evidence-based medicine. MGRS is a disease of the kidney, secondary to plasma cell clonal proliferation or immune dysfunction, requiring therapeutic intervention to eradicate the offending clone...
August 28, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/30108642/trifascicular-block-as-primary-presentation-of-the-cardiac-amyloidosis-a-rare-case-report
#5
Mohsen Yaghubi, Hossein Dinpanah, Fahimeh Ghanei-Motlagh, Samaneh Kakhki, Reza Ghasemi
BACKGROUND: Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected presentation. CASE REPORT: A 70-year-old man, with a complaint of severe weakness, lightheadedness, and lower limb paresthesia, was admitted to the emergency department. Electrocardiography revealed right bundle branch block and Trifascicular block...
March 2018: ARYA Atherosclerosis
https://www.readbyqxmd.com/read/30108065/treatment-of-al-amyloidosis-with-bendamustine-a-study-of-122-patients
#6
Paolo Milani, Stefan Schönland, Giampaolo Merlini, Christoph Kimmich, Andrea Foli, Tobias Dittrich, Marco Basset, Carsten Müller-Tidow, Tilmann Bochtler, Giovanni Palladini, Ute Hegenbart
No abstract text is available yet for this article.
August 14, 2018: Blood
https://www.readbyqxmd.com/read/30101047/portal-hypertension-and-a-stiff-liver
#7
Felicia D'Alitto, Amina Scherz, Cristina Margini, Hendrik Von Tengg-Kobligk, Matteo Montani, Thomas Pabst, Annalisa Berzigotti
Portal hypertension (PH) is a common clinical syndrome leading to severe complications. In the western world, about 90% of cases of PH are due to liver cirrhosis, and thanks to the availability of ultrasound elastography methods, this diagnosis is usually confirmed at bedside. We report a case of a patient presenting with PH and ascites initially suspected of suffering from liver cirrhosis. The finding of large hepatomegaly and a massive increase in liver stiffness prompted us to perform a liver biopsy. This revealed no fibrosis, but diffuse primary amyloidosis (AL amyloidosis)...
June 8, 2018: Curēus
https://www.readbyqxmd.com/read/30095161/real-world-outcomes-of-pomalidomide-for-treatment-of-relapsed-light-chain-amyloidosis
#8
Faye A Sharpley, Richa Manwani, Shameem Mahmood, Sajitha Sachchithanantham, Helen Lachmann, Julian Gilmore, Carol Whelan, Phillip Hawkins, Ashutosh Wechalekar
Pomalidomide is a next-generation immunomodulatory agent with activity in relapsed light chain (AL) amyloidosis, but real world outcomes are lacking. We report the experience of the UK National Amyloidosis Centre. All patients with AL amyloidosis treated with pomalidomide between 2009 and 2017 were included. Data was collected on treatment toxicity and clonal response. Survival was calculated by the Kaplan-Meier method and outcomes reported on an intent-to-treat (ITT) basis. A total of 29 patients treated with pomalidomide were identified...
August 10, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30079070/cd38-as-immunotherapeutic-target-in-light-chain-amyloidosis-and-multiple-myeloma-association-with-molecular-entities-risk-survival-and-mechanisms-of-upfront-resistance
#9
Anja Seckinger, Jens Hillengass, Martina Emde, Susanne Beck, Christoph Kimmich, Tobias Dittrich, Michael Hundemer, Anna Jauch, Ute Hegenbart, Marc-Steffen Raab, Anthony D Ho, Stefan Schönland, Dirk Hose
Monoclonal antibodies against the cell surface antigen CD38, e.g., isatuximab, daratumumab, or Mor202, have entered the therapeutic armamentarium in multiple myeloma due to single agent overall response rates of 29 vs. 36 vs. 31%, effectivity in combination regimen, e.g., with lenalidomide or bortezomib plus dexamethasone, and tolerable side effects. Despite clinical use, many questions remain. In this manuscript, we address three of these: first, upfront CD38 target-expression in AL-amyloidosis, monoclonal gammopathy of unknown significance (MGUS), asymptomatic, symptomatic, and relapsed multiple myeloma...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/30049825/cardiovascular-adverse-events-in-modern-myeloma-therapy-incidence-and-risks-a-review-from-the-european-myeloma-network-emn-and-italian-society-of-arterial-hypertension-siia
#10
REVIEW
Sara Bringhen, Alberto Milan, Claudio Ferri, Ralph Wäsch, Francesca Gay, Alessandra Larocca, Marco Salvini, Evangelos Terpos, Hartmut Goldschmidt, Michele Cavo, Maria Teresa Petrucci, Heinz Ludwig, Holger W Auner, Jo Caers, Martin Gramatzki, Mario Boccadoro, Hermann Einsele, Pieter Sonneveld, Monika Engelhardt
Cardiovascular disease in patients with multiple myeloma may derive from factors unrelated to the disease (age, diabetes, dyslipidemia, obesity, prior cardiovascular diseases), related to the disease (cardiac AL-amyloidosis, hyperviscosity, high-output failure, arteriovenous shunting, anemia, renal dysfunction) and/or related to anti-myeloma treatment (anthracyclines, corticosteroids, alkylating agents, immunomodulatory drugs, proteasome inhibitors). Good knowledge of cardiovascular events, effective dose reductions, prevention and management of early and late cardiovascular side effects of chemotherapeutic agents are essential in current clinical practice...
September 2018: Haematologica
https://www.readbyqxmd.com/read/30042220/al-amyloidosis-presenting-with-limb-girdle-myopathy
#11
Naveed Malek, Dominic G O'Donovan, Hadi Manji
An elderly Caucasian man presented with a 10-month history of proximal myopathy and dysphagia. His serum creatine kinase (CK) was elevated at 877 U/L (normal 40-320) and electromyography confirmed a myopathic process. Blood and urine tests suggested myeloma; bone marrow examination showed 30% plasma cells and stained positive for amyloid. The muscle biopsy was initially reported as normal but in the light of the bone marrow report, the biopsy specimen was stained for amyloid, which was positive. We diagnosed systemic amyloidosis causing a myopathy and have started treatment for myeloma...
July 24, 2018: Practical Neurology
https://www.readbyqxmd.com/read/30040145/immunoglobulin-light-chain-amyloidosis-2018-update-on-diagnosis-prognosis-and-treatment
#12
Morie A Gertz
DISEASE OVERVIEW: Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and "atypical multiple myeloma." DIAGNOSIS: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for diagnosis...
July 24, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/30038381/european-myeloma-network-recommendations-on-diagnosis-and-management-of-patients-with-rare-plasma-cell-dyscrasias
#13
REVIEW
Maria Gavriatopoulou, Pellegrino Musto, Jo Caers, Giampaolo Merlini, Efstathios Kastritis, Niels van de Donk, Francesca Gay, Ute Hegenbart, Roman Hajek, Sonja Zweegman, Benedetto Bruno, Christian Straka, Meletios A Dimopoulos, Hermann Einsele, Mario Boccadoro, Pieter Sonneveld, Monika Engelhardt, Evangelos Terpos
The introduction of novel agents in the management of multiple myeloma and related plasma cell dyscrasias has changed our treatment approaches and subsequently the outcome of patients. Due to current advances, the European Myeloma Network updated the diagnostic and therapeutic recommendations for patients with Waldenström's macroglobulinemia (WM), AL-amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), POEMS syndrome, and primary plasma cell leukemia. For patients with WM, the combination of rituximab with chemotherapy remains the treatment cornerstone, while the Bruton-tyrosine kinase inhibitor ibrutinib has been introduced and approved for relapsed/refractory disease...
September 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/30033840/daratumumab-for-the-treatment-of-al-amyloidosis
#14
M Hasib Sidiqi, Morie A Gertz
Autologous stem cell transplantation (ASCT) has been used as treatment for immunoglobulin light-chain (AL) amyloidosis for over two decades with improving outcomes; however, the majority of patients are not candidates for this therapy at diagnosis. Novel agents such as immunomodulatory drugs, proteasome inhibitors, and immunotherapy with monoclonal antibodies targeting CD38 have been adopted from the multiple myeloma spheres with encouraging results. Herein, we discuss the role of daratumumab, a monoclonal antibody to CD38, in the treatment of AL amyloidosis...
July 22, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/30032640/outcomes-of-patients-with-al-amyloidosis-and-low-serum-free-light-chain-levels-at-diagnosis
#15
Vina P Nguyen, Allison Rosenberg, Lisa M Mendelson, Raymond L Comenzo, Cindy Varga, Vaishali Sanchorawala
Serum free light chains (sFLC) are independent prognostic markers of disease in light chain (AL) amyloidosis, and are used in the haematologic response criteria for treatment. However, up to 20% of patients have low sFLCs at diagnosis, with a difference between involved and uninvolved free light chains (dFLC) of less than 50 mg/L, making responses to treatment difficult to evaluate. In order to characterize this distinct subgroup of patients, we retrospectively analyzed 123 AL amyloidosis patients with dFLC <50 mg/L who were diagnosed between 2002 and 2013...
July 21, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30030328/mr-neurography-biomarkers-to-characterize-peripheral-neuropathy-in-al-amyloidosis
#16
Jennifer Kollmer, Markus Weiler, Jan Purrucker, Sabine Heiland, Stefan O Schönland, Ernst Hund, Christoph Kimmich, John M Hayes, Tim Hilgenfeld, Mirko Pham, Martin Bendszus, Ute Hegenbart
OBJECTIVE: To detect, localize, and quantify peripheral nerve lesions in amyloid light chain (AL) amyloidosis by magnetic resonance neurography (MRN) in correlation with clinical and electrophysiologic findings. METHODS: We prospectively examined 20 patients with AL-polyneuropathy (PNP) and 25 age- and sex-matched healthy volunteers. After detailed neurologic and electrophysiologic testing, the patient group was subdivided into mild and moderate PNP. MRN in a 3...
August 14, 2018: Neurology
https://www.readbyqxmd.com/read/29991549/multisystem-amyloidosis-as-the-unifying-diagnosis-for-constipation-collapse-and-cardiomyopathy
#17
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
#18
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...
September 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
#19
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 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
#20
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)
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