What should I see if I have amyloidosis? In fact, there is no special department for the treatment of amyloidosis. For example, if the main problem is kidney, such as proteinuria and renal insufficiency, then you should register with the nephrology department, if the main problem is heart, then you should register with the cardiovascular department, and if the problem is gastrointestinal, then you can register with the gastroenterology department. If the clinician does not have a good understanding of primary amyloidosis, there may be some delay in the diagnosis of the patient. Therefore, our hospital has set up a working group for the diagnosis and treatment of amyloidosis, including doctors from various departments such as hematology, cardiovascular medicine, nephrology and medical imaging, and we have also developed a procedure for examination and treatment, which is believed to play a good role in the timely diagnosis and treatment of amyloidosis patients. Qiu Zhixiang, Department of Hematology, Peking University First Hospital, Beijing, China The examination process for patients with suspected amyloidosis The presence of these clinical manifestations should be suspected to amyloidosis The presence of unexplained tongue enlargement, snoring, and hypotension or hypotensive drug reduction in patients with pre-existing hypertension, or a decrease in systolic blood pressure by ≥20 mmHg. The presence of unexplained proteinuria, renal insufficiency, and not small or even enlarged kidneys; especially in patients aged 40 above, manifesting as nephrotic syndrome with insignificant hematuria. The presence of unexplained cardiac insufficiency, arrhythmia, cardiac hypertrophy, especially septal thickening. Unexplained liver function abnormalities, elevated bilirubin and ALP, GGT, hepatomegaly and changes in stool pattern, constipation and/or diarrhea. Questioning and examination think of amyloidosis Questioning and examination focus on systemic review – find multisystem damage Examination focus on the following: Tongue hypertrophy Skin lesions: petechiae, purpura, waxy or translucent changes Organ enlargement: heart, liver, spleen Blood pressure: postural hypotension Adjunctive examination think of amyloidosis Pathology: positive Congo red stain (abdominal wall subcutaneous fat, tongue. Rectum, kidney, myocardium) Etiology: routine blood, blood and urine immunofixation electrophoresis; blood free light chains; bone aspiration, cranial and pelvic plain films if necessary. Evaluation of involved organs: kidney: urine routine, urine sediment microscopy (urine erythrocyte phase contrast microscopy and leukocyte classification), 24-hour urine protein quantification, complete biochemistry, ultrasound of both kidneys cardiovascular system: ECG, holter, UCG, cardiac enhanced MRI, BNP, digestive system: abdominal ultrasound, gastroscopy, enteroscopy if necessary whole-body amyloid load: nuclear medicine SAP scan (currently not possible Special examinations and treatment: see our working group Renal puncture: see Dr. Qu Zhen – green channel Pathological diagnosis (gold standard): Fluorescence: add staining for light chains Light microscopy: add staining for Congo red, add staining for immunohistochemistry when specific proteins are suspected to form amyloid deposits (e.g., amyloid A, apolipoprotein A, transthyretin, etc.) Electron microscopy: routine electron microscopy can detect early amyloidosis, but cannot distinguish pathogenic protein components. Immunoelectron microscopy if necessary Other: amyloidogenic proteins that are difficult to identify routinely can be analyzed by mass spectrometry. For suspected hereditary, genetic analysis can be performed Bone puncture: see Dr. Chih-Chiang Qiu – special preparation Special tests related to prognosis and treatment of primary amyloidosis: Bone marrow cell MUC1 gene testing and bone marrow biopsy for MUC1 expression (indicate on the bone marrow biopsy application form to do MUC1 testing, and leave a purple tube specimen for 4° refrigerator during bone puncture Bone marrow cell CALR gene test and CRT flow cytometry (leave a green tube specimen in 4° refrigerator for bone puncture. AHSCT) Exclusion criteria: ) cTnT ≥ 0.06ug/L and NT-proBNP ≥ ) septal thickening ≥ 15mm or LVEF < ) blood creatinine > ) alkaline phosphatase more than 3 times normal or direct bilirubin > ) age ≥ 70 years