[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.