Fig. 3 Pathological change of the spleen
Summary
After admission to our hospital, the patient’s manifestations in the central nervous system were as follows: He was slow in response, occasionally raving, but with asterixis and ankle clonus negative, plasma ammonia normal, for which hepatic encephalopathy was excluded, and nerve damage was highly suspected. Peripheral nervous system: Extreme fatigue of lower extremities, for which the patient felt that the lower limbs “had no nowhere to place”, and his sleep was seriously affected, but the head MRI and lower extremities EMG showed no obvious abnormalities. C3-7 and L5-S1 intervertebral disc degeneration and herniation, without obvious abnormalities in thoracic vertebra and thoracic pulp, for which after consulting the Psychosocial Department, Neurology Department and Orthopedics Department were consulted respectively, the patient was diagnosed as ”restless leg syndrome”, with involvement of peripheral nerve very likely. Cardiovascular system: The patient developed left chest pain during hospitalization. Re-examination of ECG showed sinus rhythm, and generally, the ECG results were normal. There was no obvious abnormality in myocardial enzyme profile. Cardiac ultrasonography showed that the left ventricular soothing function was reduced. The chest pain could not exclude the possibility of myocardial amyloidosis. Blood system: Peripheral blood: progressive decrease of red blood cells and platelets; high KAP light chain and LAM light chain. Bone marrow: signs of hyperplasia, megaloblastoid changes of granulocytes and erythrocytes, increased proportion of reticular cells, increased peripheral leukocytes, visible abnormal lymphocytes, both cell lines reduced after splenectomy, which might be caused by liver damage, but in combination with bone marrow examination and related immune examination, myeloma could not be excluded. Kidney: Blood β2 microglobulin increased, with urine β2 microglobulin and 24h urine protein negative. eGFR: 140.31 ml/min / 1.73 m2 (MDRD method). Enhanced CT showed no obvious abnormality. Angiography showed no obvious abnormality in renal morphology, indicating that the kidney was not involved. Liver: Progressive decline of liver function. CT showed hepatomegaly, diffuse low-density area, and no obvious enhancement. The CT value was lower than that of spleen, and similar to that of diffuse invasive hepatic fat infiltration. The spleen ruptured spontaneously on 2020-1-24. Re-examination of spleen and liver biopsy specimens showed Congo red (+), with amyloidosis of both liver and spleen. In combination with laboratory examination of free light chain: KAP light chain 77.7g/L; LAM light chain: 80.30g/L, the patient was diagnosed as light chain amyloidosis at last.
Problems in the systemic review on changes of the patient’s conditions in the past year: First, during splenomegaly, ascites, and spider nevus as typical manifestations of decompensation in cirrhosis, there appeared giant liver as clinical manifestation, which was significantly inconsistent with liver cirrhosis. Second, after splenectomy, routine blood test showed that HGB and PLT were still decreasing. Third, there was no exact cause or obvious trigger for rapid decline of liver function. Fourth, the true cause for spontaneous splenic rupture. Furthermore, the incidence rate of amyloidosis was low, relevant experience was short, and it could not be identified by imaging alone. Therefore, as a rare disease, amyloidosis should be considered after excluding viral hepatitis, alcoholic liver disease, autoimmune liver disease, metabolic liver disease, biliary tract disease and liver damage caused by parasites.