Clinical Analysis Of85Cases Of Adult Patients With Primary Amyloidosis

Objective To summarize the clinical features of patients with amyloidosis in our hospital to raise awareness of amyloidosis.Methods A retrospective analysis of clinical features, laboratory tests and treatments of85cases with amyloidosis diagnosed by tissue biopsy from February2003to February2013in our hospital.Results1.General information85patients were diagnosed as amyloidosis by tissue biopsy,54males and31females.2. Systemic amyloidosis There were38patients with systemic amyloidosis,22males and16females,16cases identified by immunohistochemical examination for the type of primary systemic light chain amyloidosis,22patients did not undergo immunohistochemical examination or examination was negative, unable to determine the type. Patients with clinical manifestations of lower extremity edema, weight loss, exertional dyspnea, hepatomegaly, hypotension, bloating, fatigue, foamy urine, numbness. Kidney, heart and liver involvements were common.3. Localized amyloidosis There were47patients with localized amyloidosis, 32males and15females. They were not determined the type without immunohistochemical examination. The clinical manifestations were dysfunctions of involved organ. Involvements in the throat and skin were the most common.4.Treatment There are four cases of systemic amyloidosis patients eventually died due to the rapid progress of the disease during hospitalization, the cause of death was heart failure.12cases received chemotherapy with melphalan and prednisone(MP program) or chemotherapy regimen combined with bortezomib. Cutaneous amyloidosis patients after anti-allergy, topical corticosteroid therapy, the effect of varying condition were repeatedly delayed healing. The remaining localized amyloidosis patients get cured by surgical resection of the lesion.Conclusion1. Amyloidosis is a disease that amyloid deposits in the extracellular of different organs, resulting in a corresponding organ or tissue dysfunction. Amyloidosis can affect multiple organ systems, divided into systematic amyloidosis and localized amyloidosis.2. Systemic amyloidosis is a systemic disease, the disease can affect multiple organs, causing the affected organ dysfunction, the most commonly affected organ is the kidney, heart, liver. It has a variety of clinical manifestations, causing high misdiagnosis rate, and the prognosis is poor.3. Localized amyloidosis cause mainly involved organ dysfunction, no other organ involvement. Throat, skin are the most common involved organs. Clinical manifestations are simple, so it is easily diagnosed, and the treatment is effect.4. Noninvasive tests of amyloidosis are not specific, diagnosis requires biopsy.