Amyloidosis and Myelodysplastic Syndrome
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of Urology, Jichi Medical University Hospital in January 1999.She had no significant past medical history.There were no hematological disorders including amyloidosis in her family.Physical examination did not show any abnormalities including skin and heart.Keywords:
AL amyloidosis
AL amyloidosis is one type of systemic amyloidosis. AL represents Amyloid of Light chains (of Ig's) type. Diagnosis of amyloid must be carried out by patho-histological Congo-red stains of the biopsied materials under suspicion of amyloid deposits from patients with various clinical manifestations, including macroglossia, cardiac abnormalities, ECG abnormalities, GI tract problems or carpal-tunnel syndrome. Al amyloidosis may be also termed as plasma cell dyscrasia (PCD) associated amyloidosis, or monoclonal gammopathy associated amyloidosis. As to classification, AL amyloidosis consists of primary (or idiopathic) amyloidosis, and myeloma-associated (and then macroglobulinemia-associated) amyloidosis. As similarly to myeloma, patients with AL amyloidosis have monoclonal Ig's in the serum and-or in the urine. Furthermore, there have been known to be a higher incidence of Bence Jones proteins as well as more lambda than kappa among patients with AL amyloidosis compared with distributions of monoclonal Ig's among myeloma patients. It is also important for clinicians to recognize a data of a median survival of 12 months (only 4 months if cardiac amyloid occurred) in all patients with AL amyloid VS, a data of a median survival of about 36 months in myeloma patients. An attempt to clarify a way to an early diagnosis of AL amyloidosis should be investigated.
Macroglossia
AL amyloidosis
Plasma cell dyscrasia
Amyloid (mycology)
Dyscrasia
Bence Jones protein
Cardiac Amyloidosis
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Amyloidosis is the term for specific pathological peptide deposits in various tissues. Amyloid substances may be the manifestation of the following nosological units: AL-amyloidosis, AA-amyloidosis, ATTR-amyloidosis, beta2-microglobulin amyloidosis and possibly othr familiar forms of amyloidosis. The most frequent symptoms of amyloidosis are: 1. proteinuria with nephrotic syndrome, 2. cardiac failure with restrictive cardiomyopathy, 3. unexplicable hepatomegaly, 4. idiopathic peripheral polyneuropathy, 5. haemorrhagic manifestations and symptoms of increased fragility of the capillaries while the number of thrombocytes is normal, 6. periorbital haematomas and 7. macroglossia. Diagnostic and therapeutic procedures differ according to the type of amyloidosis and are analyzed in detail in the text.
Macroglossia
AL amyloidosis
Restrictive cardiomyopathy
Amyloid (mycology)
Beta-2 microglobulin
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Background. AL amyloidosis is an acquired systemic disease in which a pathologic amorphous substance produced as a result of abnormal protein metabolism is deposited in the extracellular space of various tissues.Objectives. The aim of the study was to investigate the relationship between the kappa and lambda serum free light chains (sFLCs) and the development of AL amyloidosis in patients suffering from multiple myeloma (MM).Material and Methods. The investigations included 70 MM patients, 40 females and 30 males, aged 28–83 years. In 37 persons, MM was had been diagnosed recently; 33 patients had been undergoing treatment. Amyloidosis was diagnosed in 18 patients (25.7%), including nine females, nine males; six had newly diagnosed disease. Fifteen patients developed kidney failure. The control group consisted of 10 healthy donors. The concentration of sFLC ls were determined using the immunonephelometric method and expressed in mg/L.Results. In 18 MM patients with amyloidosis the concentration of κ sFLCs ranged from 0.3 to 4780 (x = 854.5, SD = 1289), and was significantly higher (p = 0.039) than in the group without amyloidosis, where the range was from 0.3 to 426.0 (x = 68.9, SD = 98.1). The highest concentration of κ sFLCs was observed in the group of five patients with amyloidosis and renal failure. The concentration of λ sFLCs in patients with amyloidosis ranged from 0.5 to 41600 (x = 3035.7, SD = 9735) and was higher than in MM patients without amyloidosis, where it ranged from 0.5 to 834.0 (x = 79.3, SD = 193). In amyloidosis patients, the concentration of λ sFLCs was significantly higher (p = 0.05) in cases of renal failure as compared with the patients with normal renal function.Conclusion. The concentration of sFLCs is a strong indicator of amyloidosis development in MM patients.
AL amyloidosis
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AL amyloidosis
Primary systemic amyloidosis
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Light chain (AL) amyloidosis is a disease in which malignant plasma cell clones affect multiple organs including the heart and kidney. The mechanism for organ function deterioration in AL amyloidosis differs from multiple myeloma. Thus, not all agents used to treat multiple myeloma shows similar efficacy in AL amyloidosis. In AL amyloidosis, both hematologic and organ responses after treatment are important to improve the clinical outcome. Especially, improving heart function is one of the key aspects in the treatment of AL amyloidosis. With recent advances in the understanding of the pathophysiologic mechanism of AL amyloidosis, novel treatment methods are under active trial. In this article, I have reviewed the advances in pathophysiology, diagnosis, risk stratification, and treatment of AL amyloidosis.
AL amyloidosis
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AL amyloidosis
Primary systemic amyloidosis
Amyloid (mycology)
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Amyloidosis is a rare group of disorders characterized by the extracellular deposition of misfolded protein aggregates that interfere with the function of the tissue affected. In some patients, the presenting symptom of monoclonal gammopathies, such as multiple myeloma, can be a gastrointestinal bleed with a further report of amyloidosis in gastrointestinal samples. In all the cases the pathology report is read as AL (light chain) amyloidosis. We present a case of a 57-year-old male patient with no medical history who debuted with gastrointestinal bleeding. A colonoscopy revealed a colonic ulcer with a pathologic diagnosis of amyloid A (AA) amyloidosis. Further investigation led to the finding of multiple myeloma (MM) with no evidence of systemic amyloidosis. Although there is little evidence in the literature of the association or even causative relationship between multiple myeloma and AA amyloidosis, our case highlights the importance of searching for an underlying monoclonal gammopathy like MM in a patient with a confirmed diagnosis of AA amyloidosis.
AL amyloidosis
Amyloid (mycology)
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AL amyloidosis
AA amyloidosis
Amyloid (mycology)
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AL amyloidosis
Cardiac Amyloidosis
Amyloid (mycology)
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AL amyloidosis
Macroglobulinemia
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