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.
Highlights
Different types of amyloid analyzed and interpreted the data; FM: drafted concomitantly present in the same patient is the manuscript; and all authors: critically read believed to be improbable
We describe four patients, affected by monoclonal gammopathy, who underwent fat pad biopsy, because of clinically suspected amyloidosis
Considering the worsening cardiac function, the increasing in lambda free light chain (FLC) value, and the previous good response obtained with MelDex, the patient was treated with Mel-Dex again (3 cycles) obtaining a mild clinical picture improvement
Summary
We describe four patients, affected by monoclonal gammopathy, who underwent fat pad biopsy, because of clinically suspected amyloidosis. The diagnostic approach is based on two fundamental steps: i) The identification of amyloid in bioptic samples ( testing for amyloid deposits by Congo red staining under polarized light primary antibodies (Agilent Technologies Italia S.p.A., Cernusco sul Naviglio, Milano, Italy), together with protein A conjugated gold particles sized 15 nm (Agar Scientific, Stansted, UK). Twenty-four-hour urine protein electrophoresis (UPEP) demonstrated positive k light chain proteinuria, Bence. We assessed amyloidosis as a concomitant CKD’s cause: Congo red staining test on fat pad biopsy was positive. Immunogold on fat pad sample revealed transthyretin (ATTR) and serum amyloid A (SAA) amyloidosis. We screened for genetic mutations: the patient was affected by wild type form of the disease (wtATTR) (Figure 1). This is an IMWG criteria for treatment (FLC ratio >100 with concomitant FLC k >100 mg/L), but in this case, treatment was delayed and FLC ratio monitored
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