Different types of amyloid concomitantly present in the same patients
Francesca Martini1, Gabriele Buda1, Vincenzo De Tata1
1Department of Clinical and Experimental Medicine, U.O. Hematology, University of Pisa, Italy.
Abstract:
Different types of amyloid concomitantly present in the same patient is believed to be improbable. We reported four cases of patients with plasma cell disorders who were found to have biopsy proven concomitant different types of amyloid fibrils deposition. We characterized amyloid fibrils using immunogold electron microscopy. There is lack of experience in the treatment of these frail and elderly patients, who are on the threshold between necessity of chemotherapy for AL amyloidosis and necessity to avoid harmful treatment related toxicity. All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible Requirements for safe SCT include systolic blood pressure >90 mm Hg, troponin T <0.06 ng/mL, age <70 years, and serum creatinine ≤1.7 mg/dL Nontransplant candidates can be offered melphalandexamethasone or cyclophosphamide-bortezomibdexamethasone.
Insights
Simultaneous deposition of different amyloid types is rare but possible in plasma cell disorders. This study presents four cases, highlighting treatment challenges for elderly patients with systemic amyloidosis.
Area of Science:
- Hematology
- Pathology
- Biochemistry
Background:
- Concomitant deposition of different amyloid fibril types in a single patient is considered improbable.
- Amyloidosis, a group of diseases characterized by protein misfolding and deposition, presents diagnostic and therapeutic challenges.
Purpose of the Study:
- To report cases of biopsy-proven concomitant different types of amyloid fibrils in patients with plasma cell disorders.
- To discuss the therapeutic implications and challenges in managing frail, elderly patients with systemic amyloidosis.
Main Methods:
- Immunogold electron microscopy was used to characterize amyloid fibrils.
- Case series reporting clinical findings and treatment approaches.
Main Results:
- Four patients with plasma cell disorders were diagnosed with biopsy-proven concomitant different types of amyloid fibrils.
- Treatment decisions for elderly, frail patients involve balancing chemotherapy necessity for AL amyloidosis against treatment toxicity.
Conclusions:
- The co-occurrence of different amyloid types, though rare, should be considered in plasma cell disorder patients.
- Systemic amyloid syndromes necessitate therapy to prevent organ damage, with stem cell transplant as a preferred option for eligible candidates.
More Related Videos
Related Concept Videos
03:03Amyloid Fibrils
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
03:03Amyloid Fibrils
01:26Alzheimer's Disease: Overview
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ...