OVERVIEW
Hepatic purpura disease is a relatively rare benign lesion of the liver that presents as multiple blood-filled cystic cavities of varying sizes in the liver. It can be seen at any age, but is more common in adults. It has been reported more frequently in recent years and may be associated with chemical cytotoxic drugs, gamma radiation, bacterial or viral infections, and immunodeficiency, and is characterized by a random distribution of blood-filled cavities in the liver.
Etiology
The etiology is unclear and may be related to a number of diseases such as severe tuberculosis, malignancy, acquired immunodeficiency syndrome, administration of certain drugs (e.g., hormones, azathioprine, tamoxifen), long-term hemodialysis, and application of immunosuppressive drugs after organ transplantation. It has been reported that a type of mycotic purpura of the liver or spleen may occur in human immunodeficiency virus-positive people, which is associated with infection with germs of the genus r. henselae, and belongs to the parasitic diseases of animal origin, which is mainly seen in people who have close contact with cats or are infected with germs of the genus r. henselae and related pathogens.
Symptoms
Most patients are asymptomatic; a few may present with hepatomegaly and mild transaminase elevation, occasionally with splenic purpura, and rarely with hepatic failure due to massive hepatocellular injury, severe complications, and widespread diffuse hepatic purpura. Patients with mycotic purpura may present with fever, weight loss, anorexia, diarrhea, abdominal pain and distension, and hepatosplenomegaly.
Examination
1. Laboratory examination
If accompanied by secondary immunodeficiency disease, there may be decreased immunoglobulin IgA, IgG, IgE and IgM, decreased complement and lymphocytes. Anemia, hematopoiesis, moderate elevation of serum aminotransferase, alkaline phosphatase and γ-glutaminotransferase are common, and bilirubin is increased in most patients.
2. Other auxiliary examinations
(1) Imaging examination: B-mode ultrasound can detect inhomogeneous hypoechoic areas, but there are no characteristic changes; CT can show limited or diffuse hypointense or hyperintense foci in the liver, which are also not characteristic. Hepatic venography is a powerful basis for the diagnosis of hepatic purpura disease if the contrast agent is directed into the cystic cavity. Selective hepatic arteriography, from the late arterial phase to the venous phase, can be seen in the aggregation of contrast medium, but the characteristics are not strong, and it is not easy to distinguish from hepatocellular adenoma and regenerative nodules.
(2) Histological examination Laparoscopic liver is mostly enlarged with purple-blue or purple-black plaques on the surface. Needle biopsy under direct vision is a simple, safe and less traumatic examination method, as it is more targeted and can take corresponding hemostatic measures to stop bleeding from the puncture needle tract. When dissecting the abdomen, the liver tissue can be wedge cut and sent for pathological examination. For those with limited lesions and ruptured bleeding, corresponding hemostasis or partial hepatic resection will be used, and then sent for pathological examination.
Diagnosis
Symptoms are insidious and difficult to diagnose, mostly found by chance. Hepatic arteriography is helpful in severe cases when the lesion is widely distributed; CT, ultrasound and MRI are also helpful when the lesion is diffusely distributed; histologic examination is the method to confirm the diagnosis, but hepatic puncture biopsy may lead to bleeding and should be avoided.
Complications
Abdominal hemorrhage due to spontaneous rupture of the cystic cavity is its major complication.
Treatment
There is no specific treatment for this disease, but once the diagnosis is established, drugs related to this disease, such as hormones and immunosuppressive drugs, should be discontinued immediately. The treatment of the original disease is especially important, such as various serious infections, malignant tumors, diabetes, tuberculosis, blood diseases and so on. If the lesion is not big, it can be left untreated and closely observed; if the lesion increases, it can be treated with microwave curing and radiofrequency ablation; if the lesion is huge and there is a risk of hemorrhage, it can be treated with hepatic artery cannula embolization or surgical resection.
For those with bleeding and spontaneous rupture of the liver, comprehensive measures should be used to stop bleeding, and partial hepatic resection can be considered if the lesion is limited; liver transplantation can also be carefully considered if the liver lesion is serious.
For mycotic purpura, correct antibiotic application, such as erythromycin and doxycycline (doxycycline) can make purpura subside. For severely ill patients or those who cannot take the medication orally, the medication can be given intravenously instead. Note: In the early stage of drug administration, exacerbation and fever may occur, which can be prevented by applying antipyretic drugs in advance.
Prevention
The natural prognosis of most cases is still good, and the prognosis is poor in a few cases with severe hepatic impairment.