Systemic Immunoglobulin Light-chain Amyloidosis:A Case Report And Review Of The Literature

Objects: To investigate the diagnosis and treatment of systemic immunoglobulin light-chain amyloidosis.Methods: To analyze one case of recently diagnosed AL amyloidosis and to review relevant literature.Data:Though the history and a complete physical examination,retrospective analysis o f his clinical information,we find the plasma globulin was repeatedly elevated and his ski n is dry within last 4 years,and the right carotid artery systolic bruits.Regular examination,chest X-ray,abdominal ultrasonography,abdomen enhanced CT scan and MRI,urine protein quantitation,serum protein electorphoresis,myocardial enzyme,serum and urine immunoglobulin light chains,bone marrow puncture for hematology and genetic analysis were carried out.Clinical diagnosis was based on combination of the patient clinical history,physical examination and laboratory results.Finally,the abdomen subcutaneous fat biopsies and pathologyical study was employed to comfirm the diagnosis.Results: Integrating the patient clinical history,physical examination,laboratory and imaging results,the final diagnosis were as following: 1.systemic immunoglobulin light-chain(AL)amyloidosis(?);2.type 2 diabetes;3.hypertension;4.chronic atrophic gastritis;5.old pulmonary tuberculosis;6.cholecystitis and cholecystic polypus;7.hepatic cysts.Conclusion: AL amyloidosis belongs to primary amyloidosis.The culprit substance is the immunoglobulin light chains(? or ?)which formed amyloid fibril with ?-folding.The unsolvable amyloid deposited in body tissue,leading to organ dysfunction.Because a variety of organs involved in the process,the clinical manifestations were diversified.We should do comprehensive examinations to clarify the diagnosis of AL when the manifestation were atypical.Early diagnosis is closely related to the prognosis of disease.Diagnosis of AL mostly rely on the biopsy,if pathological study confirm the diagnosis,assessment of organ damage and prognosis is extremely important in order to choosing the optimal treatment opportunity and program.At present the predominant treatment is chemotherapy,autologous stem cell transplantation and supportive treatment.