After the first description of amyloid infiltration in 1842 by Rokitansky, the chemical composition and pathogenesis of amyloid deposits became a matter of a heated dispute. Extensive evidence of a significant association between “primary” amyloidosis and myeloma-type proteins, particularly Bence Jones proteins, has been provided by the observations of my mentor, Elliott Osserman, in a seminal paper in 1964.1 The strongest evidence for the immunoglobulin origin of amyloid fibrils has come with the determination of the am ino acid sequence of 2 amyloid fibril proteins by George Glenner in 1971.
Source: Hematology/Oncology Clinics of North America - Category: Cancer & Oncology Authors: Source Type: research

Related Links:

Colon perforations are rare in MM and here we report characteristics and clinical outcomes of 30 patients that were treated at Mayo Clinic. We believe that steroids are the precipitating factor. AbstractGastrointestinal complications of multiple myeloma (MM) treatment are common and include nausea, constipation, and diarrhea. However, acute gastrointestinal events like perforations are rare. We aimed to describe the characteristics and outcomes of patients with MM that had colonic perforations during their treatment. This is a retrospective study that included patients from all three Mayo Clinic sites who had MM and develo...
Source: Cancer Medicine - Category: Cancer & Oncology Authors: Tags: ORIGINAL RESEARCH Source Type: research
This article discusses how to approach patients with monoclonal gammopathy and peripheral neuropathy, highlighting clinical and laboratory clues that may aid in establishing a diagnosis in a timely manner. RECENT FINDINGS From a hematologic standpoint, a monoclonal gammopathy may be of undetermined significance or can be associated with an underlying myeloma, lymphoplasmacytic lymphoma, or amyloidosis. Each of these conditions can cause peripheral neuropathy, with varying clinical and electrodiagnostic profiles. Treatment usually consists of treating the underlying hematologic disorder. IgM-associated peripheral neuropa...
Source: CONTINUUM: Lifelong Learning in Neurology - Category: Neurology Tags: REVIEW ARTICLES Source Type: research
The spectrum of immunoglobulin paraprotein-associated diseases requiring therapy extends beyond multiple myeloma and AL amyloidosis. Awareness of these is essential in ensuring timely accurate diagnosis and appropriate treatment. As most paraprotein-associated diseases are fairly uncommon, therapeutic decisions must often be made in the absence of data from randomized controlled trials. Treatment is generally directed at the underlying clonal cell population. This review focuses on the spectrum of the less common paraprotein-associated disorders. In most instances, the monoclonal immunoglobulin plays a direct role in the p...
Source: Hematology/Oncology Clinics of North America - Category: Cancer & Oncology Authors: Source Type: research
We present a rare case of cholestatic LF secondary to primary AL (kappa-light chain) amyloidosis as a presentation associated to multiple myeloma. PMID: 32954783 [PubMed - as supplied by publisher]
Source: Revista Espanola de Enfermedades Digestivas - Category: Gastroenterology Tags: Rev Esp Enferm Dig Source Type: research
Many challenges remain in diagnosing monoclonal immunoglobulin-associated renal disease, despite widespread application of immunofluorescence (IF) and immunohistochemistry. Here, we report a newly diagnosed case of multiple myeloma with clinical suspicion of renal amyloidosis, which had negative IF staining for kappa and lambda light chains in the glomeruli. Although laser microdissection and mass spectrometry-based proteomic analysis have emerged as important tools for amyloid typing in the literature, such facilities are still not widely available in Asia. We propose that a clinicopathological algorithm for the evaluatio...
Source: Case Reports in Nephrology and Dialysis - Category: Urology & Nephrology Source Type: research
An 85-year-old Caucasian female presented with a 3-year history of tongue enlargement associated with serious difficulties with swallowing and speech. Her medical history of relevance was hypertension and anxiety. Physical examination revealed multiple asymptomatic multisized nodules of fibrous consistency spread throughout the skin and oral mucosa. A truly remarkable sign observed was the prompt development of ecchymosis during manipulation of oral tissues. Histopathologic examination of her tongue biopsy revealed the presence of homogeneous, eosinophilic material within connective tissue and around capillaries.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Tags: 20190114 Source Type: research
Multiple myeloma (MM) is an uncommon plasma cell malignancy with a slight male predilection. Up to 15% of patients with MM present an abnormal deposition of amyloid tissue. In the oral cavity, this deposition develops as macroglossia. A 69-year-old woman presented with a 9-month history of painless tongue enlargement. Microscopic analysis showed amorphous and eosinophilic deposits in the dermis compatible with amyloid. Congo red stain was positive. Myelogram verified 29% of plasma cells, and monoclonal isolated lambda protein was found in the urine and serum.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Tags: 20190522 Source Type: research
Conclusion: Renal biopsy is safe in the elderly and provides a wealth of information with regards to the diagnosis and prognosis of renal disorder.
Source: Indian Journal of Nephrology - Category: Urology & Nephrology Authors: Source Type: research
Abstract Light chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. In Europe, approximately 5000 new diagnosis per year are reported. Deposition of amyloid fibrils derived from antibody light chains are key pathogenic agents in AL amyloidosis. They can be deposited in multiple organs but cardiac involvement carries a major risk of mortality. The prognosis is poor in cases associated with multiple myeloma. The average survival is around 1 year. Up to half of all patients with cardiac amyloidosis die suddenly; 75% ofthose deaths are due to heart fa...
Source: International Journal of Clinical and Experimental Pathology - Category: Pathology Authors: Tags: Int J Clin Exp Pathol Source Type: research
We report a case of a patient with amyloidosis who developed bilateral, acute secondary angle closure during an infusion of daratumumab. Ultrasound biomicroscopy obtained 3 days after the onset of her symptoms demonstrated the cause to be bilateral choroidal effusions. Taken together with several previous case reports, the evidence suggests that, like topiramate, daratumumab is associated with the idiosyncratic reaction of choroidal effusions, resulting in a spectrum of clinical outcomes from myopic shift to acute angle closure. The treating oncologist and eye care provider should be aware of these adverse outcomes in any ...
Source: Journal of Glaucoma - Category: Opthalmology Tags: Online Articles: Case Report/Small Case Series Source Type: research
More News: Amyloidosis | Cancer & Oncology | Chemistry | Hematology | Men | Myeloma