2,148 results match your criteria Amyloidosis Primary Systemic


Systemic amyloidosis as a cause of chronic diarrhea after gastric surgery. Literature review.

Rev Esp Enferm Dig 2022 Jun 24. Epub 2022 Jun 24.

Cirugía General y del Aparato Digestivo, Hospital Universitario Clínico San Cecilio, España.

Amyloidosis with gastrointestinal involvement is a rare cause of chronic diarrhoea, and should be considered especially in adult patients with intestinal malabsorption and extra GI manifestations. We present the case of a male patient who, after an oncological gastrectomy, presented with chronic diarrhea that did not respond to treatment and, after study, the cause of the diarrhea was finally found. Primary systemic light chain amyloidosis (AL) initially presents as chronic diarrhea and weight loss after gastrectomy. Read More

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Cardiac Amyloidosis.

Authors:
Morie A Gertz

Heart Fail Clin 2022 Jul;18(3):479-488

Department of Medicine, Mayo Clinic Rochester, 200 Southwest First Street, W10, Rochester, MN 55905, USA. Electronic address:

Amyloid deposits are defined by their tinctorial properties. Under the light microscope amyloid deposits are eosinophilic and amorphous when stained with hematoxylin and eosin. With Congo red staining the deposits are positive and under polarized light will exhibit green birefringence. Read More

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[A clinical retrospective analysis of newly diagnosed multiple myeloma patients with systemic light chain amyloidosis].

Zhonghua Xue Ye Xue Za Zhi 2022 Apr;43(4):330-335

Jiangsu Institute of Hematology, National Clinical Research Center for Hematologic Diseases, NHC Key Laboratory of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Suzhou 215000, China.

To analyze the clinical characteristics, treatment response, and prognosis of newly diagnosed symptomatic multiple myeloma (MM) patients with systemic light chain amyloidosis (AL) . The clinical data of 160 patients with newly diagnosed MM treated at the First Affiliated Hospital of Soochow University from January 1, 2017 to October 31, 2018, were retrospectively analyzed. According to the histopathological biopsy results of bone marrow, skin, and other tissues, the patients were divided into two groups according to whether amyloidosis was combined or not, namely, the MM+AL group and the MM group. Read More

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[Value of (11)C-PiB PET/MRI in the evaluation of organ involvement in primary systemic light chain amyloidosis].

Zhonghua Xue Ye Xue Za Zhi 2022 Apr;43(4):316-322

Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

To analyze the value of (11)C-PiB PET/MRI for evaluating organ involvement in patients with primary light chain amyloidosis (pAL) . The clinical data of 20 patients with pAL and 3 healthy volunteers from January 2019 to October 2021 were retrospectively analyzed. The correlation between the organ involvement evaluated by clinical standards and PET/MRI was compared. Read More

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[Primary amyloidoma of the brain].

Arkh Patol 2022 ;84(3):48-51

Burnasyan State Research Federal Medical Biophysical Center, Moscow, Russia.

Primary brain amyloidoma is rare tumor-like lesion characterized by focal accumulation of insoluble fibrillar aggregates of amyloid in brain tissue without systemic amyloidosis. Literature review and a case report of primary brain amyloidoma in 61 years old female are given with a special attention on the complexity of preoperative diagnosis. Read More

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Cutaneous Involvement in Diseases with Plasma Cell Differentiation: Diagnostic Approach.

Curr Oncol 2022 04 24;29(5):3026-3043. Epub 2022 Apr 24.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, Italy.

Neoplasms with plasma cell differentiation may occasionally involve the skin. Cutaneous lesions may represent the first sign of an underlying systemic plasma cell malignancy, such as multiple myeloma, or the skin itself may be the primary site of occurrence of a hematological tumor with plasma cell differentiation. Starting from examples encountered in our daily practice, we discussed the diagnostic approach pathologists and clinicians should use when faced with cutaneous lesions with plasma cell differentiation. Read More

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Primary Systemic Amyloidosis With Cardiac and Renal Involvement.

Cureus 2022 May 21;14(5):e25194. Epub 2022 May 21.

Internal Medicine, Universiti Teknologi MARA, Selangor, MYS.

Systemic amyloidosis is a life-threatening disorder with a poor prognosis. Accurate and early diagnosis of the condition is of paramount importance as early initiation of therapy improves the prognosis and survival rate. A 49-year-old gentleman presented with recurrent right exudative pleural effusion. Read More

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Multidisciplinary supportive care in systemic light chain amyloidosis.

Blood Res 2022 May 20. Epub 2022 May 20.

Department of Hematology-Oncology, Myeloma and Amyloidosis Program, Maroone Cancer Center, Cleveland Clinic Florida, Weston FL, USA.

The immunoglobulin light-chain amyloidosis is a multisystemic disease which manifests by damage to the vital organs by light chain-derived amyloid fibril. Traditionally, the treatment has been directed to the underlying plasma cell clone with or without high dose chemotherapy followed by autologous stem cell transplantation using melphalan based conditioning. Now with the approval of highly tolerable anti-CD38 monoclonal antibody daratumumab based anti-plasma cell therapy in 2021, high rates of hematologic complete responses are possible even in patients who are otherwise deemed not a candidate for autologous stem cell transplantation. Read More

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Eyelid Amyloid Deposition in Systemic Light Chain Amyloidosis.

Actas Dermosifiliogr 2022 03 9;113(3):305. Epub 2022 Feb 9.

Haematology Department, University Hospital Lisbon North, Lisbon, Portugal.

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Adding Cyclophosphamide to Bortezomib and Dexamethasone was not Associated With Improved Outcomes of Patients With Newly Diagnosed Light Chain Amyloidosis: A Retrospective Study.

Clin Lymphoma Myeloma Leuk 2022 Apr 8. Epub 2022 Apr 8.

Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL. Electronic address:

Introduction: Before 2021, the combination of bortezomib, cyclophosphamide, and dexamethasone (VCd) was one of the most used upfront therapy for systemic immunoglobulin light chain (AL) amyloidosis. Recently, daratumumab in combination with VCd resulted in improved outcomes compared to VCd. However, it's still unclear the role of cyclophosphamide in this combination. Read More

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The clinical features and outcomes of systemic light chain amyloidosis with hepatic involvement.

Ann Med 2022 12;54(1):1226-1232

National Clinical Research Center of Kidney Disease, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.

Objectives: To evaluate the clinical characteristics and prognostic factors of hepatic systemic light chain (AL) amyloidosis.

Methods: Eighty-eight patients diagnosed AL amyloidosis with hepatic involvement between June 2004 and January 2019 were analysed retrospectively.

Results: The median age of the patients was 55 years old, and the male to female ratio was 2. Read More

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December 2022

Macroglossia Associated With Primary Systemic Amyloidosis - Case Report With Unusual Clinical Presentation.

J Ayub Med Coll Abbottabad 2022 Jan-Mar;34(1):197-199

Department of Oral Medicine and Radiology, Government Dental College Kottayam, Kerala, India.

Background: Amyloidosis is characterised by the deposition of fibrillar insoluble proteinaceous material called amyloid in the extracellular spaces. It may present as localized form which is rare and systemic form. Systemic amyloidosis involves many organs like kidney, heart and liver. Read More

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Evaluation of multinodular goiter and primary hyperparathyroidism leads to a diagnosis of AL amyloidosis.

Thyroid Res 2022 Apr 20;15(1). Epub 2022 Apr 20.

Division of Endocrinology, Diabetes and Metabolism, University of Florida, Gainesville, FL, USA.

Background: Amyloid goiter, defined as excess amyloid within the thyroid gland in such quantities that it produces a clinically apparent goiter, is a very rare manifestation of systemic amyloidosis with cases commonly seen in the setting of Amyloid A (AA) amyloidosis. Amyloid goiter as the primary clinical manifestation secondary to Amyloid light chain (AL) amyloidosis is very rare. We present a case of AL amyloidosis with initial manifestation as goiter with amyloid deposition in the thyroid and the parathyroid gland. Read More

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Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment.

Authors:
Morie A Gertz

Am J Hematol 2022 06 25;97(6):818-829. Epub 2022 Apr 25.

Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.

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 heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and "atypical smoldering multiple myeloma or monoclonal gammopathy of undetermined significance (MGUS)."

Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for the diagnosis of AL amyloidosis. Read More

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A stepwise data interpretation process for renal amyloidosis typing by LMD-MS.

BMC Nephrol 2022 04 13;23(1):144. Epub 2022 Apr 13.

Guangzhou KingMed Center for Clinical Laboratory Co.,Ltd, Guangzhou, 510005, China.

Backgrounds: Systemic amyloidosis is classified according to the deposited amyloid fibril protein (AFP), which determines its best therapeutic scheme. The most common type of AFP found are immunoglobulin light chains. The laser microdissection combined with mass spectrometry (LMD-MS) technique is a promising approach for precise typing of amyloidosis, however, the major difficulty in interpreting the MS data is how to accurately identify the precipitated AFP from background. Read More

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Daratumumab: A Review in Newly Diagnosed Systemic Light Chain Amyloidosis.

Authors:
Hannah A Blair

Drugs 2022 Apr;82(6):683-690

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Subcutaneous daratumumab (DARZALEX) co-formulated with recombinant human hyaluronidase (DARZALEX FASPRO) is approved in several countries, including the USA and those of the EU, for use in combination with bortezomib, cyclophosphamide and dexamethasone for the treatment of adult patients with newly diagnosed light chain (AL) amyloidosis. Daratumumab is a CD38-targeting, human IgG1κ monoclonal antibody. In the pivotal phase III ANDROMEDA trial in adults with newly diagnosed systemic AL amyloidosis, the addition of daratumumab to bortezomib, cyclophosphamide and dexamethasone significantly increased the proportion of patients achieving a haematological complete response relative to bortezomib, cyclophosphamide and dexamethasone alone (primary endpoint). Read More

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Clinical Clues and Diagnostic Workup of Cardiac Amyloidosis.

Methodist Debakey Cardiovasc J 2022 14;18(2):36-46. Epub 2022 Mar 14.

Knight Cardiovascular Institute, Oregon Health & Science University School of Medicine, Portland, Oregon, US.

Cardiac amyloidosis is increasingly recognized as an underlying cause of left ventricular wall thickening, heart failure, and arrhythmia with variable clinical presentation. Due to the subtle cardiac findings in early transthyretin cardiac amyloidosis and the availability of therapies that can modify but not reverse the disease progression, early recognition is vital. In light chain amyloidosis, timely diagnosis and treatment can significantly improve survival. Read More

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Autophagy Alteration in ApoA-I Related Systemic Amyloidosis.

Int J Mol Sci 2022 Mar 23;23(7). Epub 2022 Mar 23.

Department of Chemical Sciences, University of Napoli Federico II, Complesso Universitario Monte Sant'Angelo, 80126 Napoli, Italy.

Amyloidoses are characterized by the accumulation and aggregation of misfolded proteins into fibrils in different organs, leading to cell death and consequent organ dysfunction. The specific substitution of Leu 75 for Pro in Apolipoprotein A-I protein sequence (ApoA-I; L75P-ApoA-I) results in late onset amyloidosis, where deposition of extracellular protein aggregates damages the normal functions of the liver. In this work, we describe that the autophagic process is inhibited in the presence of the L75P-ApoA-I amyloidogenic variant in stably transfected human hepatocyte carcinoma cells. Read More

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Eyelid Amyloid Deposition in Systemic Light Chain Amyloidosis.

Actas Dermosifiliogr 2022 03 25;113(3):T305. Epub 2021 Sep 25.

Departamento de Hematología, Centro Hospitalario Universitario Lisboa Norte, Lisboa, Portugal.

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Multimodal Imaging and Biomarkers in Cardiac Amyloidosis.

Diagnostics (Basel) 2022 Mar 3;12(3). Epub 2022 Mar 3.

Division of Cardiology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea.

Amyloidosis is a progressive infiltrative disease instigated by the extracellular deposition of amyloid fibrils in various organs such as the heart, kidney, and peripheral nerves. Cardiac amyloid deposits cause restrictive cardiomyopathy, leading to a poor prognosis in systemic amyloidosis. The most common etiologies of cardiac amyloidosis (CA) are immunoglobulin light chain deposits (AL-CA) and misfolded transthyretin deposits (ATTR-CA). Read More

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Circulating biomarkers in diagnosis and management of cardiac amyloidosis: a review for internist.

Intern Emerg Med 2022 Jun 24;17(4):957-969. Epub 2022 Mar 24.

Regional Referral Center for Systemic Amyloidosis, Careggi University Hospital, Largo Brambilla 3, 50134, Florence, Italy.

Cardiac amyloidosis (CA) is due to extracellular myocardial deposition of misfolded proteins resulting in severe cardiac dysfunction and death. The precursors of amyloid fibrils, able of determining a relevant cardiac infiltration, are immunoglobulin-free light chains (AL amyloidosis) and transthyretin (TTR) (both wild and mutated types). The diagnosis of amyloidosis represents a challenge for the clinician given its rarity and its protean clinical presentation, thus an early diagnosis remains a cornerstone for the prognosis of these patients, also in light of the growing available treatments. Read More

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Joint Pain and Arthritis as First Clinical Manifestation of Systemic Amyloidosis and Multiple Myeloma: Case Report and Brief Literature Review.

Hematol Rep 2022 Mar 2;14(1):19-23. Epub 2022 Mar 2.

Hematology Division, Pisa University Hospital, 56126 Pisa, Italy.

Amyloidosis is a rare disease that is often seen in conjunction with multiple myeloma (MM). Its damage varies depending on the anatomical site affected; however, it is believed that many cases of amyloidosis are misrecognized due to the fact that its signs and symptoms are nonspecific. Joint amyloidosis, in particular, may be confused with degenerative or autoimmune diseases. Read More

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Multiple Indolent Asymptomatic Yellow-Orange Patches and Plaques.

Cureus 2022 Feb 3;14(2):e21870. Epub 2022 Feb 3.

Dermatopathology, Advanced Dermatology and Cosmetic Surgery, Delray Beach, USA.

An 83-year-old Caucasian male presented with a history of asymptomatic yellow-orange macules and plaques concentrated on his trunk and proximal extremities that have been slowly progressing for the past three years. A punch biopsy revealed the presence of eosinophilic amorphous and fissured material within the superficial and interstitial dermis consistent with nodular amyloidosis. With the lack of concurrent systemic symptoms and negative systemic laboratory workup, the patient was diagnosed with disseminated primary localized cutaneous nodular amyloidosis (PLCNA). Read More

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February 2022

Peritumoral Immunoglobulin M Lambda Light Chain Amyloidosis in a Patient With Advanced Follicular Lymphoma.

Cureus 2022 Jan 30;14(1):e21738. Epub 2022 Jan 30.

Hematology/Oncology, Saint Michael's Medical Center, Newark, USA.

Peritumoral light chain (AL) amyloidosis secondary to lymphoid malignancies is a rare but well-described entity. Peritumoral deposition of amyloid without systemic amyloidosis has been described in mucosa-associated lymphoid tissue (MALT) lymphomas; however, there are no reported cases of follicular lymphoma with localized peritumoral AL amyloidosis without systemic involvement of amyloidosis. We present a rare case of a patient with advanced follicular lymphoma with peritumoral lymph node IgM lambda light chain amyloidosis without an underlying monoclonal gammopathy or plasma cell dyscrasia. Read More

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January 2022

Analysis of the complete lambda light chain germline usage in patients with AL amyloidosis and dominant heart or kidney involvement.

PLoS One 2022 25;17(2):e0264407. Epub 2022 Feb 25.

Medical Department V, Section of Multiple Myeloma, Heidelberg University Hospital, Heidelberg, Germany.

Light chain amyloidosis is one of the most common forms of systemic amyloidosis. The disease is caused by the misfolding and aggregation of immunoglobulin light chains to insoluble fibrils. These fibrils can deposit in different tissues and organs such as heart and kidney and cause organ impairments that define the clinical presentation. Read More

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Physiology, Diagnosis and Treatment of Cardiac Light Chain Amyloidosis.

J Clin Med 2022 Feb 9;11(4). Epub 2022 Feb 9.

Department of Experimental Immunology, Medical University of Lublin, 20-093 Lublin, Poland.

AL (light-chain) amyloidosis is a systemic disease in which amyloid fibers are formed from kappa or lambda immunoglobulin light chains, or fragments thereof, produced by a neoplastic clone of plasmocytes. The produced protein is deposited in tissues and organs in the form of extracellular deposits, which leads to impairment of their functions and, consequently, to death. Despite the development of research on pathogenesis and therapy, the mortality rate of patients with late diagnosed amyloidosis is 30%. Read More

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February 2022

Mucocutaneous manifestations of systemic amyloidosis in a Black patient.

Authors:
V Ramachandran

QJM 2022 04;115(4):237-238

Department of Internal Medicine, Texas Health Presbyterian Hospital, Dallas, TX, USADepartment of Dermatology, New York University, New York City, NY, USA.

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Renal Amyloidosis: A Clinicopathological Study From a Tertiary Care Hospital in Pakistan.

Cureus 2022 Jan 11;14(1):e21122. Epub 2022 Jan 11.

Nephrology, Shifa International Hospital, Islamabad, PAK.

Introduction Systemic amyloidosis can affect any organ in the body, but the kidney is the most commonly involved site. It is characterized by the extracellular deposition of insoluble fibrillar proteins. Amyloid deposits can be identified histologically by Congo red stain, which gives apple-green birefringence under polarized light. Read More

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January 2022