Author: Shao Ming Xiao Yuzhen Zhang Cuiting Shanxi Yongji Hepatobiliary Stomach Specialist Hospital
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Summary of Medical History
< p>Patient, Wu, male, 64 years old, farmer, from Shanxi.
Complaint: 2 years after the discovery of liver cirrhosis, abdominal distension, liver discomfort 15 sky.
History of present illness: Fatigue, abdominal distension, liver area after exertion 2 years ago Unwell, he was hospitalized in a hospital, and he checked abdominal ultrasound: liver cirrhosis and splenomegaly with ascites. Hepatitis B series: HBsAg(+), anti-HBs(-), HBeAg(-), anti-HBe(+), anti-HBc(+), anti-HBc-IgM(-); HBV-DNA: 5.0×104 IU/ml; hepatoprotective, antiviral, correction of hypoalbuminemia, diuresis and other supportive and symptomatic treatment, diagnosis during hospitalization: decompensated liver cirrhosis Period Viral hepatitis (type B) is chronic; after discharge from hospital, he has been taking entecavir tablets for treatment till now. Abdominal distention and liver discomfort after exertion 15 days ago, no nausea, vomiting, abdominal pain, diarrhea and other symptoms, abnormal blood routine examination in the external hospital (PLT: 1129.00×109/ L), felt that the above symptoms were aggravated, in order to seek further diagnosis and treatment, he came to our hospital, and the outpatient was admitted to the hospital with “chronic viral hepatitis (type B) in decompensated liver cirrhosis”. The patient was sane and in poor spirits due to the onset, his diet was 1/3 less than normal, the nighttime sleep was poor, and the urination and bowel movements were basically normal. There was no significant weight loss since the onset, and he was admitted to the hospital on 2018-8-23.
Past history: chronic hepatitis B for 10 years, no regular check-up and standardization treat. Denied the history of hypertension, diabetes, heart disease; denied the history of typhoid fever, tuberculosis and other infectious diseases. There was no history of surgery or trauma. Denied the history of poisoning, denied the history of drug allergy.
Personal History: Non-smoking and alcohol addiction. No travel history.
Family History: His father has a history of hepatitis, and he has not been regularly reviewed.
Epidemiological History: Denial of blood transfusion, blood donation, vaccination history Unknown. No history of living in the foci.
Physical examination:T: 36.6°C< /span>, P: 82 times/min, R: 19 times/min, BP: 120/64mmHg, body weight: 63 kg. Consciousness, moderate nutrition, liver disease face, spider nevus (+), liver palm (+). The skin and sclera were mildly yellowed, and there was no obvious enlargement of the bilateral thyroid. Breath sounds were clear in both lungs, and no dry or wet rales were heard. Heart auscultation, percussion (-). The liver was not palpable under the ribs, but the spleen was palpable at 4 cm below the ribs, hard in texture, smooth in surface, tender (-), and percussion pain in the liver area (+). Abdominal tenderness, rebound tenderness and abdominal muscle tension were absent. Fluid tremor (-). Abdominal percussion mobile dullness (+), bilateral lower extremity edema (+), the rest was negative.
Preliminary Diagnosis
1. Liver cirrhosis
2. Viral hepatitis (type B) chronic
3. Thrombocytosis?
The underlying disease of this patient is liver cirrhosis caused by chronic viral hepatitis B, combined with physical examination, spleen enlargement, liver cirrhosis and spleen enlargement. Some patients may be accompanied by hypersplenism, and hypersplenism will lead to the decrease of white blood cells, red blood cells, and platelets in the blood routine, one or more of which are reduced. This patient has more than 1,000 platelets. Is it a test error or thrombocytosis? Can’t be explained by the monism of liver cirrhosis and splenomegaly and hypersplenism, or is there another reason? Look for clues.
Auxiliary tests
Liver function< /span>: TBIL: 57.4 µmol/L, DBIL: 23.3 µmol/L, ALT: 151 U/L, AST: 159 U/L, ALP: 135 U/L, GGT: 60 U /L, ChE: 4195 U/L, TP: 63 g/L, Alb: 33.9 g/L, PA: 145 mg/L, TBA: 76.1 µmol/L.
HBVM: HBsAg(+), anti-HBs(-), HBeAg( -), anti-HBe(+), anti-HBc(+), anti-HBc-IgM(-).
A, C, D, E, Ghepatitis markers strong> were all negative, anti-HIV and RPR were negative.
HBV-DNA quantification<1.0×102 IU/ml.
Glu: 4.96mmol/L; renal function, electrolytes, blood lipids: normal . CRP: 4.3 mg/L.
Blood routine: WBC: 15.32×109/L, RBC: 4.28×1012/L, HGB: 137.00 g/L, PLT: 1187.00×109/L. ESR: 10 mm/h.
Coagulation Complete Set: PT: 15.30 Sec.
thyroid function: normal.
Autoantibody results: antinuclear antibody: weak positive, nucleolar type; Anti-smooth muscle antibody, anti-mitochondrial antibody, anti-liver-kidney microsomal antibody, anti-liver antigen, anti-hepatic solute antibody type I, anti-Ro-52, anti-Sp100, anti-3E (BPO), anti-PML and anti-gp210 were all negative; IgG , IgA, IgM, IgG 4: normal.
AFP: 4.12 IU/ml, CEA, CA-125, CA-199: normal.
Ten Urine Items: Vitamin C: +2, the rest is normal.
Stool routine: OK. Fecal OB (-).
abdominal ultrasound: 1. Liver cirrhosis with splenomegaly A small amount of ascites 2. Secondary gallbladder changes.
ECG: Normal. Chest X-ray: No abnormality was found in both lungs, heart and diaphragm.
Gastroscopy: Esophageal varices (moderate) Chronic non-atrophic gastritis with erosion.
Further analysis
Analysis of the condition, clinically for For difficult diseases, common diseases should be considered first, then rare diseases, and finally rare diseases. There are also some difficult cases until the patient’s death, and the cause is not clear. Such as: hemochromatosis, hepatolenticular degeneration, cytomegalovirus infection, EB virus infection, etc. need to be further excluded, autoimmune liver disease has basically been excluded, and further improve the examination. Combined with the blood routine results, the patient’s white blood cell and platelet values increased significantly, which cannot be explained by monism, so it is necessary to continue to find the reasons for these two increases. Bone marrow biopsy and genetic testing are needed to further clarify the cause, and then treat the cause.
Check further
< span>serum CMV, EBV-DNA: negative.
serum copper, serum iron, ceruloplasmindetection span>The results are normal.
Bone marrow biopsyThe results are shown: (400 times magnification Picture 1: HGE staining , Image 2: Gomori Staining):
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Figure 2
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Bone marrow hyperplasia was more active, and interstitial neutrophils, erythroid and megakaryocyte systems were proliferated. The granulocyte system is mainly composed of cells in the middle and lower stages, with a slight increase in eosinophils; the precursor cells are not significantly increased, and the distribution is not abnormal; the erythrocyte is mainly composed of middle and late erythrocytes; Too many leaves), and clustering can be seen.
Matrix edema, mild hyperplasia of fibrous tissue, and focal distribution.
Gomori staining: reticular fibers+
bone marrow Fibrosis grade: MF-1.
Diagnosis: bone marrow hyperplasia.
blood tumor-related gene testThe results show: 1. In this test, BCR/ ABL-190.-210.-230 fusion gene was negative. 2. Detect JAK2 (EXON14) V617F gene mutation.
Final Diagnosis
1. Liver cirrhosis
2. Hypoproteinemia
3. Chronic viral hepatitis (type B)
4. Portal hypertensive gastropathy
5. Pre-myelofibrosis
Treatment
Hepatoprotection, jaundice, antiviral, correction of hypoalbuminemia, diuresis, anti-infection , anti-platelet aggregation (aspirin), acid suppression, inhibition of extramedullary hematopoiesis (hydroxyurea) and other supportive symptomatic treatment, review the indicators improved, the patient was discharged.
Discussion
Myelofibrosis is a myeloproliferative disease in which the hematopoietic tissue of the bone marrow is affected by a certain Stimulated by these factors, hematopoietic stem cells develop lesions, fibrous connective tissue continues to proliferate, and hematopoietic parenchyma cells decrease, thereby affecting the bone marrow hematopoietic tissue, leading to hematopoietic dysfunction or even failure. It is a type of myeloproliferative tumor with a relatively low incidence. Myelofibrosis can be divided into two categories from the etiology, namely primary myelofibrosis and secondary myelofibrosis. In the early stage, there are no obvious symptoms or only symptoms of hypermetabolism such as weight loss, weakness, and excessive sweating. As the disease progresses, the patient may present with abdominal distension, hepatosplenomegaly and other oppressive symptoms, accompanied by anemia, fever, bleeding and other symptoms. In severe cases, bone pain, liver cirrhosis, and hyperuricemia may occur.
So, what is the difference between primary and secondary?
Primary myelofibrosis:mostly Onset after middle age, the onset is mostly insidious, and the progress is slow.
Secondary myelofibrosis:Secondary myelofibrosis Myelofibrosis occurs in tumors, infections and other diseases. The clinical symptoms and signs are the comprehensive manifestations of the primary cause and myelofibrosis, and corresponding treatment should be given according to the pathogenic cause and the symptoms it produces.
Follow-up
Regularly review related items after discharge , The anti-virus and extramedullary hematopoietic inhibition program will continue. In February 2021, liver cancer will be detected and surgical resection of liver cancer will be performed. Review on January 14, 2022, blood routine WBC: 5.06×109/L, RBC: 4.20×1012 /L, HGB: 135.00 g/L, PLT: 452.00×109/L; liver function, AFP were normal, no new tumor lesions on abdominal color Doppler ultrasound, The condition is stable, and the patient has no discomfort; the treatment plan continues, and follow-up is underway.
Experience of diagnosis and treatment
The diagnosis and treatment process of this patient Tip: In clinical any disease, the results of routine blood tests should be paid attention to. If you cannot use monism to explain, you should expand your thinking. If necessary, please consult a hematology department. Bone marrow biopsy and genetic testing have important clinical significance; avoid missed diagnosis and delay Treatment; treatment for the cause can only be targeted.