News in the treatment of AL amyloidosis

Hématologie ◽  
2012 ◽  
Vol 18 (2) ◽  
pp. 116-123
Author(s):  
Arnaud Jaccard ◽  
Dania Mohty ◽  
Estelle Desport ◽  
Frank Bridoux
Keyword(s):  
Al Amyloidosis

Author response for "Daratumumab for Relapsed or Refractory AL Amyloidosis with high Plasma Cell Burden"

2019 ◽  
Author(s):  
Rahel Schwotzer ◽  
Markus Gabriel Manz ◽  
Stefanie Pederiva ◽  
Christine Waibel ◽  
Clemens Caspar ◽  
...  
Keyword(s):  
Plasma Cell ◽  
High Plasma ◽  
Author Response ◽  
Al Amyloidosis

DETERMINING OF MONOCLONAL GAMMOPATHY IN NEPHROLOGICAL PATIENTS

2019 ◽  
Vol 23 (2) ◽  
pp. 82-90
Author(s):  
L. B. Lysenko ◽  
N. V. Chebotareva ◽  
N. N. Mrykhin ◽  
V. V. Rameev ◽  
T. V. Androsova ◽  
...  
Keyword(s):  
Renal Diseases ◽  
Chronic Glomerulonephritis ◽  
Al Amyloidosis ◽  
Hematological Neoplasms ◽  
Cast Nephropathy ◽  
Number Of Patients ◽  
Light Chain Disease ◽  
Kidney Involvement ◽  
Cryoglobulinemic Glomerulonephritis ◽  
Electrophoresis Of Proteins

BACKGROUND. Мonoclonal gammopathy (MG) is not only the state preceding of hematological neoplasms, but also associated with non- hematological diseases, in particular damage of kidneys. Earlier diagnosis of MG represents an important area in treating patients with renal diseases associated with MG. THE AIM: To determine the frequency of MG among therapeutic and nephrological patients for optimization of methods of their diagnosis and treatment. PATIENTS AND METHODS: In common, 11392 patients were analyzed within 4 years (2013-2016). The standard clinical examination was conducted. Method of an electrophoresis of proteins of serum of blood and the 24-hour urine, method of immunofixation of proteins of serum and urine, and method of free light chains definition in serum (Freelite) were used for MG identification. RESULTS: MG is diagnosed in 174 of 11392 patients: 49 % of men and 51 % of women aged from 18 up to 85 years. MG was found 2.1 times more often in nephrological patient than in patients of therapeutic departments. Among patients of this group, AL-amyloidosis with kidney involvement was diagnosed in 41 %, cryoglobulinemic glomerulonephritis – in 18 %, chronic glomerulonephritis – in 35 %, also there was small number of patients with light chain disease and cast-nephropathy. 86 % of nephrological patients had less than 5 g/l of monoclonal protein that corresponds oligo secretory MG, and at 46 % from them – less than 1 g/l, other 10 % had MG of 5-10 g/l, and only in 4.42 % of patients MG more 10g/l was defined. CONCLUSION: We conclude that MG, especially oligo secretory form, play a significant role in pathogenesis of renal damage. It is important to apply sensitive methods – immunofixation of proteins and method «Freelite» for nephrological patients.

Bone disease as the first manifestation of systemic AL-amyloidosis

2020 ◽  
Vol 92 (7) ◽  
pp. 85-89
Author(s):  
L. P. Mendeleeva ◽  
I. G. Rekhtina ◽  
A. M. Kovrigina ◽  
I. E. Kostina ◽  
V. A. Khyshova ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Bone Disease ◽  
Plasma Cells ◽  
Interventricular Septum ◽  
Bone Destruction ◽  
Amyloid Deposition ◽  
Bone Lesions ◽  
Al Amyloidosis ◽  
Linear Type

Our case demonstrates severe bone disease in primary AL-amyloidosis without concomitant multiple myeloma. A 30-year-old man had spontaneous vertebral fracture Th8. A computed tomography scan suggested multiple foci of lesions in all the bones. In bone marrow and resected rib werent detected any tumor cells. After 15 years from the beginning of the disease, nephrotic syndrome developed. Based on the kidney biopsy, AL-amyloidosis was confirmed. Amyloid was also detected in the bowel and bone marrow. On the indirect signs (thickening of the interventricular septum 16 mm and increased NT-proBNP 2200 pg/ml), a cardial involvement was confirmed. In the bone marrow (from three sites) was found 2.85% clonal plasma cells with immunophenotype СD138+, СD38dim, СD19-, СD117+, СD81-, СD27-, СD56-. FISH method revealed polysomy 5,9,15 in 3% of the nuclei. Serum free light chain Kappa 575 mg/l (/44.9) was detected. Multiple foci of destruction with increased metabolic activity (SUVmax 3.6) were visualized on PET-CT, and an surgical intervention biopsy was performed from two foci. The number of plasma cells from the destruction foci was 2.5%, and massive amyloid deposition was detected. On CT scan foci of lesions differed from bone lesions at multiple myeloma. Bone fragments of point and linear type (button sequestration) were visualized in most of the destruction foci. The content of the lesion was low density. There was no extraossal spread from large zones of destruction. There was also spontaneous scarring of the some lesions (without therapy). Thus, the diagnosis of multiple myeloma was excluded on the basis based on x-ray signs, of the duration of osteodestructive syndrome (15 years), the absence of plasma infiltration in the bone marrow, including from foci of bone destruction by open biopsy. This observation proves the possibility of damage to the skeleton due to amyloid deposition and justifies the need to include AL-amyloidosis in the spectrum of differential diagnosis of diseases that occur with osteodestructive syndrome.

Localized intestinal AL amyloidosis detected as bright green using autofluorescence endoscopy

2021 ◽  
Author(s):  
Masatake Kuroha ◽  
Tsuneaki Yoshinaga ◽  
Masahide Yazaki ◽  
Fumiyoshi Fujishima ◽  
Tomoyuki Handa ◽  
...  
Keyword(s):  
Al Amyloidosis ◽  
Bright Green ◽  
Autofluorescence Endoscopy

scholarly journals Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

2021 ◽  
Vol 11 (8) ◽  
Author(s):  
Andrew Staron ◽  
Luke Zheng ◽  
Gheorghe Doros ◽  
Lawreen H. Connors ◽  
Lisa M. Mendelson ◽  
...  
Keyword(s):  
Standards Of Care ◽  
Al Amyloidosis ◽  
Unexpected Death ◽  
Related Factors ◽  
Term Survival ◽  
Natural History Study ◽  
Boston University ◽  
Disease Outcomes ◽  
Patient Subgroups ◽  
Over Time

AbstractThe recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival trends and primary causes of death among 2337 individuals with AL amyloidosis referred to the Boston University Amyloidosis Center. Outcomes were analyzed according to date of diagnosis: 1980-1989 (era 1), 1990-1999 (era 2), 2000-2009 (era 3), and 2010-2019 (era 4). Overall survival increased steadily with median values of 1.4, 2.6, 3.3, and 4.6 years for eras 1–4, respectively (P < 0.001). Six-month mortality decreased over time from 23% to 13%. Wide gaps in survival persisted amid patient subgroups; those with age at diagnosis ≥70 years had marginal improvements over time. Most deaths were attributable to disease-related factors, with cardiac failure (32%) and sudden unexpected death (23%) being the leading causes. AL amyloidosis-unrelated mortality increased across eras (from 3% to 16% of deaths) and with longer-term survival (29% of deaths occurring >10 years after diagnosis). Under changing standards of care, survival improved and early mortality declined over the last 40 years. These findings support a more optimistic outlook for patients with AL amyloidosis.

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