Citations

Data on 11 patients with orthostatic hypotension and primary systemic amyloidosis have been presented. Dizziness and light-headedness or syncope significantly hampered the involved patients. Three were incapacitated by orthostatic hypotension. Diarrhea, nausea, vomiting, and loss of weight were common, and adrenal insufficiency was seriously considered in the differential diagnosis of six of the 11 patients. Abnormal sweating was found in all six in whom this test was done. The drug 9-α-fluorohydrocortisone showed promise in symptomatic therapy of the condition. The possibility of primary systemic amyloidosis should be considered in the presence of orthostatic hypotension. Rectal biopsy and bone-marrow aspiration are recommended as initial biopsy procedures unless another organ suitable for biopsy is obviously involved.

AbstractThe POEMS syndrome (polyradiculoneuropathy, organomegaly, multiple endocrinopathies, monoclonal protein, skin changes) is a rare disease associated with a plasma cell dyscrasia. Patients with disseminated POEMS can be treated with high-dose therapy and autologous stem cell transplantation (ASCT). While clinical improvement is nearly universal in these patients, the long-term outcomes after transplantation are unclear. We therefore assessed the long-term clinical outcomes of 59 POEMS patients treated with ASCT at our institution. With a median follow-up of 45 months, 14 patients have progressed with a progression-free survival of 98% and 75% at 1 and 5 years, respectively. Factors associated with progression have included an IgG-λ monoclonal component (hazard ratio [HR] 7.5; 95% confidence interval [CI], 2.3-28.3; P = .0008), fluorodeoxyglucose-avid lesions on baseline positron emission tomography (HR 6.4; 95% CI, 1.2-120; P = .03), lack of complete hematologic response (HR 5.4; 95% CI, 1.8-16.7; P = .003), and patients aged 50 years or younger at transplantation (HR 4.4; 95% CI, 1.3-20; P = .01). The most common progression events have been radiologic followed by rising VEGF. Symptomatic progression has been rare. Most patients could be salvaged with immunomodulatory drugs or radiation. The 5-year survival is 94%. Herein, we describe a system of monitoring response and progression among patients with POEMS after ASCT.