AL amyloidosis: advances in diagnosis and management.

AL amyloidosis: advances in diagnosis and management. Blood Res. 2020 Jul 31;55(S1):S54-S57 Authors: Koh Y Abstract Light chain (AL) amyloidosis is a disease in which malignant plasma cell clones affect multiple organs including the heart and kidney. The mechanism for organ function deterioration in AL amyloidosis differs from multiple myeloma. Thus, not all agents used to treat multiple myeloma shows similar efficacy in AL amyloidosis. In AL amyloidosis, both hematologic and organ responses after treatment are important to improve the clinical outcome. Especially, improving heart function is one of the key aspects in the treatment of AL amyloidosis. With recent advances in the understanding of the pathophysiologic mechanism of AL amyloidosis, novel treatment methods are under active trial. In this article, I have reviewed the advances in pathophysiology, diagnosis, risk stratification, and treatment of AL amyloidosis. PMID: 32719177 [PubMed]
Source: Blood Research - Category: Hematology Tags: Blood Res Source Type: research

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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
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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 ...
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Source: American Journal of Clinical Pathology - Category: Pathology Authors: Tags: Am J Clin Pathol Source Type: research
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Source: Leukemia Research Reports - Category: Hematology Source Type: research
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