scholarly journals Nail Biopsy in the Diagnosis of Systemic Amyloidosis

2018 ◽  
Vol 2 (4) ◽  
pp. 252-255
Author(s):  
Cristopher C Briscoe ◽  
Zachary P Nahmias ◽  
Ilana S Rosman ◽  
Milan J Anadkat
Keyword(s):  
Monoclonal Gammopathy ◽  
Conventional Treatment ◽  
Systemic Amyloidosis ◽  
Al Amyloidosis ◽  
Nail Dystrophy ◽  
Hematopoietic Stem ◽  
Nail Changes ◽  
Unknown Significance ◽  
History Of ◽  
Low Threshold

A 60-year-old woman with a 13-year history of monoclonal gammopathy of unknown significance (MGUS) presented with worsening nail dystrophy. Prior workup for systemic amyloidosis had been unrevealing, and no other signs of cutaneous disease were present. Nail biopsy was consistent with amyloid deposition, and the patient subsequently underwent autologous hematopoietic stem cell transplantation for AL amyloidosis. In light of the growing literature regarding nail changes as a presenting sign of systemic amyloidosis and the promising utility of nail biopsy, we suggest a low threshold for biopsy in appropriate patients when nail changes characteristic of amyloidosis are refractory to conventional treatment.

Evaluation of the Cytogenetic Aberration Pattern in AL Amyloidosis Compared to Monoclonal Gammopathies Not Requiring Treatment: Translocation t(11;14) Is More Frequent in AL Amyloidosis.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2500-2500 ◽  
Author(s):  
Tilmann Bochtler ◽  
Stefan O. Schonland ◽  
Anna Jauch ◽  
Christiane Heiss ◽  
Axel Benner ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Control Group ◽  
Al Amyloidosis ◽  
Fish Analysis ◽  
Cell Content ◽  
Cytogenetic Aberration ◽  
Cytogenetic Aberrations ◽  
Plasma Cell Disorders ◽  
Unknown Significance

Abstract Introduction: Cytogenetic aberrations (CA) have emerged as important pathogenetic and prognostic factors in plasma cell disorders. However, in AL amyloidosis (AL) only a few reports with small numbers of patients have been published. Methods: Using interphase FISH analysis in CD138+ cells, we evaluated the role of CA in a series of 85 AL patients as compared to 146 patients with a monoclonal gammopathy without treatment requirement in a prospective manner. Our panel included IgH translocations t(11;14), t(4;14), t(14;16) and translocation of 14q32 with an unidentified partner, gains of 1q21, 11q23 and 19q13 as well as deletions of 8p21, 13q14 and 17p13. Using these probes we could detect at least one of these aberrations in 95% of AL and in 88% of the control group. Age, gender and plasma cell content were statistically equally distributed among both groups. Results: The most frequent aberration in AL was t(11;14), which was detected in 45% of AL patients as compared to 26% of the control group (p=0,056). It was strongly associated with the lack of an intact immunoglobulin (p<0,001), thus accounting for the frequent light chain only subtype in AL. Markedly, t(11;14) was more frequently found in combination with gain 11q23 in AL than in the control group (20% versus 6%, p = 0.005). Other frequent aberrations in AL included deletion 13q14 (32%) and gain 1q21 (21%), which were observed in the control group at comparable frequencies (34% and 20%). The overlapping character of the underlying plasma cell disorder in both disease entities was also emphasized by the similarities of branching patterns of the five major CA in cluster analysis applied in 169 patients (figure 1). The relation of clinical parameters and chromosomal aberrations was also evaluated. The analyzed CA had no impact on the organ involvement pattern in AL patients. Conclusions: We observed a high frequency for t(11;14) in AL. Apart from this finding, the cytogenetic patterns known in monoclonal gammopathy of unknown significance and multiple myeloma were widely shared by AL amyloidosis. Figure Figure

Cardiac Toxicity After High-Dose Melphlan and Autologous Hematopoietic Stem Cell Transplantation for Multiple Myeloma

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2027-2027
Author(s):  
Zartash Gul ◽  
Qaiser Bashir ◽  
Yago Nieto ◽  
Nazeer Sherwani ◽  
Simrit Parmar ◽  
...  
Keyword(s):  
Chest Pain ◽  
Cardiac Disease ◽  
Cardiac Toxicity ◽  
Prior History ◽  
High Dose ◽  
Al Amyloidosis ◽  
Bundle Branch Block ◽  
Hematopoietic Stem ◽  
History Of

Abstract Abstract 2027 Background: High-dose melphalan (HDM), 200 mg/m2, is the standard regimen for autologous hematopoietic stem cell transplant (auto-HCT) for multiple myeloma (MM). Although HDM-induced cardiotoxicity is reported, its incidence is not well known. In this retrospective analysis we evaluated the incidence of cardiotoxicity and its impact on outcome in patients undergoing auto-HCT for MM. Methods: Three-hundred and sixty-nine consecutive patients who received melphalan 200 mg/m2 for auto-HCT ASCT between January 2006 and December 2009 were included in these analyses. Cardiac toxicity was defined as any cardiac adverse event reported from the day of administration of melphalan to day+ 30 after auto-HCT. Results: Patient characteristics are summarized in Table 1. Median age at auto-HCT was 59 years (32–78 years). Median time from diagnosis to auto-HCT was 8.0 months (1.8–266.3). Patients undergoing auto-HCT had a left ventricular ejection fraction (LVEF) of >40%, and no symptomatic cardiac disease. Thirty-five patients had concurrent or AL amyloidosis, and 43 patients had a prior history of cardiac disease. By day +30 after auto-HCT, 34 patients (9.2%) developed one or more adverse cardiac adverse events (AE). Twenty-seven patients experienced a single cardiac AE: atrial fibrillation (a fib): 16, congestive heart failure (CHF): 3, supraventricular tachycardia (SVT): 3, ischemic chest pain: 2, ventricular bigeminy: 1, diastolic dysfunction: 1 and sudden cardiac death in a patient with AL amyloidosis: 1. Seven patients had a combination of 2 or more cardiac adverse events: CHF + a fib: 4, ischemic chest pain + QT prolongation: 1, ischemic chest pain + left bundle branch block (LBBB): 1, ischemic chest pain + premature ventricular contractions (PVCs): 1. Overall, supraventricular arrhythmias were responsible for cardiac AE in 6.2% (23/369) of the patients. Twenty-eight patients (82%) fully recovered from the cardiac AE. Four patients required long term pharmacologic treatment of a fib, and one patient with a fib developed a cerebellar infarct without residual neurologic deficits. Patients with cardiac AE were older (median age: 62 vs. 59, p=0.014) and had a prior history of cardiac disease (17/34 vs. 20/335, p=0.0001) compared to patients without cardiac AE. Gender, concurrent AL amyloidosis, interval between diagnosis and auto-HCT, or transplant before or after the year 2000 were not associated with cardiac AE. Median overall follow up was 28 months (1–369). 100-day non-relapse mortality (NRM) was 3% (1/34) vs. 0% (0/335) in patients with or without cardiac AEs (p=0.09). Kaplan-Meier estimates of 3-year OS were 70% vs. 84% in patients with or without cardiac AE (p=0.084). Conclusions: HDM and auto-HCT was associated with cardiac AEs in 9% of patients. A fib and CHF were the most common cardiac AEs, but >80% fully recovered without long-term sequelae. Patients with cardiac AE were relatively older and had a prior history of cardiac disease. There was no significant difference in NRM or OS in patients with or without melphalan-associated cardiac AEs. <PR, includes Stable Disease, No Response, PD, Minimal Response; >=VGPR: includes Complete Remission (CR);, Very Good Partial Response and Partial Remission (VGPR), PR: Partial remission. RBBB (Right bundle branch block). Disclosures: No relevant conflicts of interest to declare.

Systemic Amyloidosis and Monoclonal Gammopathy in Three Italian Siblings : A Familian Case of AL-Amyloidosis?

1991 ◽  
pp. 227-230
Author(s):  
A. Miliani ◽  
F. Bergesio ◽  
M. Salvadori ◽  
A. M. Ciciani ◽  
G. P. Merlini ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Systemic Amyloidosis ◽  
Al Amyloidosis

Eight novel loci implicate shared genetic etiology in multiple myeloma, AL amyloidosis, and monoclonal gammopathy of unknown significance

Leukemia ◽  
2019 ◽  
Vol 34 (4) ◽  
pp. 1187-1191 ◽  
Author(s):  
Subhayan Chattopadhyay ◽  
Hauke Thomsen ◽  
Niels Weinhold ◽  
Iman Meziane ◽  
Stefanie Huhn ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Al Amyloidosis ◽  
Genetic Etiology ◽  
Unknown Significance ◽  
Shared Genetic

scholarly journals IgG,kappa monoclonal gammopathy of unknown significance with AL amyloidosis simulating giant cell arteritis

2017 ◽  
Vol 55 (3) ◽  
pp. 179-182
Author(s):  
Valer Mihai Pompilian ◽  
Ştefăniţă Tănăseanu ◽  
Camelia Badea ◽  
Sabina Zurac ◽  
Claudiu Socoliuc ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Monoclonal Gammopathy ◽  
Temporal Artery ◽  
Al Amyloidosis ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries ◽  
First Case ◽  
Unknown Significance ◽  
Tunnel Syndrome

Abstract Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA). We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS) associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension. Muscular strength was normal. She had high ESR and CRP; in this clinical context, GCA was suspected. A gamma spike on serum protein electrophoresis raised the suspicion of monoclonal gammopathy (MG). Immunoelectrophoresis revealed monoclonal bands for IgG and kappa chains. Massive deposits of amyloid and no inflammation were found on temporal artery biopsy. Multiple myeloma and lymphoma were ruled out. A diagnosis of AL amyloidosis complicating MGUS was formulated. She did well on therapy with bortezomib, cyclophosphamide and dexamethasone. Cases published in medical literature reveal amyloidosis mimicking GCA in the setting of established MGUS. As far as we know, this is the first case of MGUS with IgG and kappa chains in which a GCA-like picture induced by amyloidosis was present from the very onset.

Detergent properties as the basis of the dimexide therapeutic effect in AA-amyloidosis infectious nature (clinical case analysis)

2020 ◽  
Vol 24 (2) ◽  
pp. 60-71
Author(s):  
V. Rameev ◽  
L. Kozlovskaya ◽  
A. Rameeva ◽  
P. Tao
Keyword(s):  
Therapeutic Effect ◽  
Medical Practice ◽  
Clinical Case ◽  
Clinical Observation ◽  
Case Analysis ◽  
Systemic Amyloidosis ◽  
Aa Amyloidosis ◽  
Functional Amyloid ◽  
History Of

The article discusses the current possibilities of postinfectious AA-amyloidosis treatment with dimexide on the example of clinical observation, discribes in detail the problem of functional amyloid and debates the prospects of the principle of amyloid resorption in the treatment of systemic amyloidosis. The history of the use of dimexide in medical practice is given, thenecessary dataon the pharmacology of dimexide are presented.

scholarly journals Effect of tofogliflozin on arterial stiffness in patients with type 2 diabetes: prespecified sub-analysis of the prospective, randomized, open-label, parallel-group comparative UTOPIA trial

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Naoto Katakami ◽  
◽  
Tomoya Mita ◽  
Hidenori Yoshii ◽  
Toshihiko Shiraiwa ◽  
...  
Keyword(s):  
Risk Factors ◽  
Blood Pressure ◽  
Type 2 Diabetes ◽  
Conventional Treatment ◽  
Mixed Effects ◽  
Mixed Effects Models ◽  
Open Label ◽  
Parallel Group ◽  
History Of

Abstract Background Tofogliflozin, an SGLT2 inhibitor, is associated with favorable metabolic effects, including improved glycemic control and serum lipid profile and decreased body weight, visceral adipose tissue, and blood pressure (BP). This study evaluated the effects of tofogliflozin on the brachial-ankle pulse wave velocity (baPWV) in patients with type 2 diabetes (T2DM) without a history of apparent cardiovascular disease. Methods The using tofogliflozin for possible better intervention against atherosclerosis for type 2 diabetes patients (UTOPIA) trial is a prospective, randomized, open-label, multicenter, parallel-group, comparative study. As one of the prespecified secondary outcomes, changes in baPWV over 104 weeks were evaluated in 154 individuals (80 in the tofogliflozin group and 74 in the conventional treatment group) who completed baPWV measurement at baseline. Results In a mixed-effects model, the progression in the right, left, and mean baPWV over 104 weeks was significantly attenuated with tofogliflozin compared to that with conventional treatment (– 109.3 [– 184.3, – 34.3] (mean change [95% CI] cm/s, p = 0.005; – 98.3 [– 172.6, – 24.1] cm/s, p = 0.010; – 104.7 [– 177.0, – 32.4] cm/s, p = 0.005, respectively). Similar findings were obtained even after adjusting the mixed-effects models for traditional cardiovascular risk factors, including body mass index (BMI), glycated hemoglobin (HbA1c), total cholesterol, high-density lipoprotein (HDL)-cholesterol, triglyceride, systolic blood pressure (SBP), hypertension, smoking, and/or administration of drugs, including hypoglycemic agents, antihypertensive agents, statins, and anti-platelets, at baseline. The findings of the analysis of covariance (ANCOVA) models, which included the treatment group, baseline baPWV, and traditional cardiovascular risk factors, resembled those generated by the mixed-effects models. Conclusions Tofogliflozin significantly inhibited the increased baPWV in patients with T2DM without a history of apparent cardiovascular disease, suggesting that tofogliflozin suppressed the progression of arterial stiffness. Trial Registration UMIN000017607. Registered 18 May 2015. (https://www.umin.ac.jp/icdr/index.html)

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