scholarly journals CLINICAL AND MORPHOLOGICAL CHARACTERISTIC CASE OF SYSTEM AL-AMYLOIDOSIS

2020 ◽  
pp. 53-53
Author(s):  
O.V. Vorobeva ◽  
A.V. Lastochkin ◽  
N.E. Gimaldinova ◽  
E.M. Luzikova ◽  
I.P. Malov
Keyword(s):  
Morphological Characteristic ◽  
Al Amyloidosis ◽  
Characteristic Case

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.

Cutaneous amyloidosis as major manifestation of systemic AL-amyloidosis

2018 ◽  
Author(s):  
Irene Gullo ◽  
Joao Magalhaes
Keyword(s):  
Al Amyloidosis

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.

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
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