[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.
Isobe
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Faculty of Health Science, Kobe University School of Medicine.
D000686:Amyloidosis / Q000401:mortality; D005260:Female; D006801:Humans; D008875:Middle Aged; D011379:Prognosis