[General information]
Medical history
The patient, Last name: Shi, male, 50 years old, living in Tongchuan City, China, has been engaged in automobile repair for 30 years. He was admitted to our department on June 13, 2020 for chief complaint of ”1 year of abdominal distension, accompanied with yellowing of eyes, which was aggravated with edema of lower limbs 20 days ago”. One year ago, the patient developed abdominal distension, yellowing of eyes, fatigue, nausea without vomiting, abdominal pain and diarrhea, and body weight loss of about 15 kg. He was treated in a local hospital, Xijing Hospital, Fengxian Hospital and our hospital successively, and checked for liver function. The results were as follows: albumin 27.2g/L, TBil 44.9umol/L, ALT 14IU/L; AST:45IU/L. Gastroscopy found reflux esophagitis (Grade A), superficial gastritis with erosion. Ultrasonography showed no abnormality in thyroid function. Enhanced CT of upper abdomen found large liver with decreased density, liver cyst and several small retroperitoneal lymph nodes. Transient elastography of the liver showed a median liver hardness of 25.9 kPa and a median fat attenuation of 21. dm /m, for which the patient was diagnosed as ”fatty liver” and given oral Methimazole, Ursofalk, Wuling Capsules, Diammonium Glycyrrhizinate Enteric-coated Capsules, and Glutathione Tablets, as well as a traditional Chinese medicine for twice (not specified), after which abdominal distension and yellowing of eyes were alleviated. Twenty days ago, his abdominal distension was aggravated for unknown reasons, and there was edema of lower limbs after activity, which could be relieved after rest. He felt that the lower limbs “had no nowhere to place”, with poor sleep at night, and then, he was treated at the Second Affiliated Hospital of Xi ’an Jiaotong University. Relevant examination results were as follows: Lier function: albumin 33.5g/L, TBil: 91.20umol/L, ALT: 27IU/L; AST: 85IU/L, Hepatic angiography findings: The posterior hepatic segment of inferior vena cava was thin, and the intrahepatic vein was unclear, with enlarged liver and ascites. Chest CT findings: chronic inflammation in the upper lobe of right lung, and in the left lung; right pleural effusion. Bone biopsy: myelodysplastic syndrome with abnormal lymphocytes, for which the patient received supportive therapies, such as liver protection and yellowing alleviation, ammonia reduction, albumin infusion, plasma and diuresis. After this, the abdominal distension and edema of lower limbs were alleviated, but the sleep was still poor at night. Then he came to our hospital for further diagnosis and treatment. Since the onset of the disease, his mental conditions, as well as his appetite, were poor, his stool was dry and the urine was yellow and heavy. Also, his sleep was poor at night. Past history: The patient received cholecystectomy more than 1 year ago, and splenectomy for spontaneous rupture of the spleen about 5 months ago, with blood transfusion history. He denied the histories of hepatitis B, hepatitis C, tuberculosis, malaria, hypertension, heart disease, diabetes, cerebrovascular disease, and mental disease. He had 30 years of smoking history, average 2 cigarettes/day, but has given up smoking. Also, he had 25 years of drinking history, 2-3 times a week, about 150g/ time, but has given up drinking. Family history: His father died for renal failure, with no special epidemiological history.
Physical examination
Vital signs after admission: T: 37.2℃, P: 101 times/min, R: 20 times/min, BP :124/78mmHg, height 170cm, weight 62kg. With appearance of liver disease, autonomous position, conscious, slow in response, good in mental conditions, without yellow stain or rash on skin of the whole body, with ecchymosis at the puncture point on the forearm, 1 spider mole on the chest, and liver palms. The sclera was slightly yellow and the eyelids was pale, with enlarged lymph nodes palpable in the neck; Oral cavity: There were ulcers at the buccal mucosa and lower lip, and pigmentation on the right tongue bod. There was no thyroid enlargement, and no vascular murmur was heard. Chest: The chest was symmetrical without deformity, without obvious abnormality of respiratory movement, and the vocal fremitus was not enhanced or weakened. Double lung percussion, as well as double lung auscultation for breath, sounds clear. Dry and wet rales and pleural frictions were not heard. Heart: There was no eminence in the precardiac area, and there was cardiac apex fluctuation 0.5cm in the midline of the left fifth intercostal clavicle. No tremor were touched and pericardial friction sound. Percussion showed not cardiac enlargement. The heart rate was 101 times/min, with regular hear rhythm. No murmur was heard in each valve area. Abdomen: Abdominal distension, without abdominal varicose veins, with about 15cm surgical scar under the left rib. There were tenderness below the liver margin and under the xiphoid process, without rebound pain, and the liver is 8cm below the ribs, spleen was not touched, with abdominal dull percussion note, and positive shifting dullness. The auscultation showed weakened borborygmus. Nervous system: asterixis, ankle clonus (-). Initial diagnosis: chronic liver failure, damage for alcoholic liver? Hepatic encephalopathy? spontaneous bacterial peritonitis after splenectomy and cholecystectomy.
Auxiliary examination
The laboratory examination results after admission in our hospital were as follows: routine blood test: HGB92g/L, WBC 16.17×109/L, N% 74.4%, L%17.2%, PLT83×109/L. Hepatic function: ALB37.3g/L, AST60U/L, ALT30U/L, CHOL1.24umol/L, TBIL142.2umol/L. Blood coagulation: PT:28.60S, APTT62.40S, INR2.66, PTA:27% , FIB1.45g/L. Myocardial enzyme spectrum: ADH390U/L, HBDH378U/L, CK291U/L, CK-MB 27U/L, IMA 61.8U/ml. ECG: sinus rhythm, low voltage, ST-T segment changes; Renal function: cysC2.000mg/L Cr68umol/L, BUN 11.76mmol/L; ESR: 54mm/h; PCT: 0.818 ng/ml. No obvious abnormalities were observed in 10 indicators for tumor, 8 indicators for infection, routine feces and urine tests, plasma ammonia, G/GM, auto-immune liver antibody, ceruloplasmin, and ANCA. Hepatitis virus series of HBsAg, HBeAg, Anti-HBe, Anti-HBc (-), Anti-HBs(+); Hepatitis A, C and E antibodies: negative. EB-IgM (-), EB-IgG(+); EBV, CMV quantitative tests: negative. Among the immune indexes, there were eight for immunity and 3 for rheumatism: ASO 327IU/ mL, KAP light chain 7.58g/L, LAM light chain 4.32g/L, IgE1360IU/ml,IgG23.10g/L, IgA11.60g/L, C3:0.47g/L. Immunofixation electrophoresis: IgG, IgA, IgM, K, L: negative; Free light chain: KAP light chain 77.7g/L; LAM light chain: 80.30g/L; Lymphocyte subsets: total T cell percentage: 53.67%; NK cell percentage 38.51%; Helper T cell percentage: 21.26%; B cell percentage: 7.26%; Blood β 2 - ug 3388.7 MG/L; Urine β 2 - ug 50.0 MG/L; Serum protein electrophoresis: ALB: 51.50, α1-globulin 3.50, α2-globulin 3.50; β1-globulin 5.00, β2-globulin 12.10, γ-globulin 24.40; M protein 0.00; IgG typing: IgG18.90g/L; IgG1:16.90 g/L; IgG4:1.17 g/L; Imaging findings: MR plain scan of the head: no obvious abnormalities in brain parenchyma. Vertebral body MR: C3-7 and L5-S1 intervertebral disc degeneration and herniation, without obvious abnormalities in thoracic vertebra and thoracic pulp; Cardiac Ultrasonography: Left ventricular soothing function decreased; Enhanced CT for the upper abdomen: enlarged liver, sign of heterogeneous fatty liver, and slight hypodense lesion in the right anterior lobe of liver. The gallbladder and spleen were not clearly displayed. No obvious abnormal enhancement was observed in the pancreas, bilateral kidneys and bilateral adrenal glands (for changes in the liver and spleen, see Figure 1). Angiography for abdominal great vessels: No obvious abnormalities were found in the proper hepatic artery, and the left and right hepatic arteries, as well as in the superior mesenteric vein, residual splenic vein, main portal vein, and the left and right branches of portal vein. Localized stenosis was observed in the intrahepatic segment of inferior vena cava. Re-examination results of the spleen and liver biopsy specimens showed that the white pulp structure of spleen amyloidosis disappeared, with significant changes for congestion, local massive bleeding, and red stained vascular wall without structural deposits. Cargo red staining indicated amyloidosis. Immunohistochemical results of liver: CD34 staining showed local hepatic sinus endothelium vascularization, CK7 staining showed notable hyperplasia of bile canaliculus, HBsAg (-), special staining results: Masson, PSA, PAS-D, iron staining showed no abnormal local destruction of mesh fibers, Congo red (+). (See Fig. 2 and Fig. 3)
Fig. 1 Changes of liver and spleen
Note: The time changes of top left, top right, bottom left and bottom right are 2019-07-24, 2020-01-07, 2020-04-09 and 2020-06-12 respectively.