Mayo Clinic proceedings
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Mayo Clinic proceedings · May 2006
ReviewMonoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, AL amyloidosis, and related plasma cell disorders: diagnosis and treatment.
The spectrum of plasma cell disorders is broad. Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma are asymptomatic disorders characterized by monoclonal plasma cell proliferation in the bone marrow in the absence of end-organ damage. ⋯ Finally, AL (immunoglobulin light chain) amyloidosis and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome are disorders characterized by low tumor burden but profound multisystemic disease. Updated diagnostic criteria for these disorders, risk stratification models to determine prognosis, and the current management of these diverse entitles are discussed in this review.
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Mayo Clinic proceedings · May 2006
ReviewA practical approach to genetic testing for von Willebrand disease.
von Willebrand disease (vWD) is the most commonly diagnosed congenital bleeding disorder. The laboratory diagnosis of type 2 variants and type 3 vWD is reasonably well defined, and characterization of the von Willebrand factor (vWF) gene has facilitated definition of their molecular basis. ⋯ Genetic testing is costly, and thus judicious use will be increasingly important for appropriate genetic-counseling of patients with vWD and their family members. This article provides a practical approach to utilization of genetic testing in vWD.