Banti syndrome is a disorder of the spleen, the large, gland-like organ in the upper left side of the abdomen that produces red blood cells before birth and, in newborns, removes and destroys aged red blood cells, and plays a role in fighting infection. In the case of Banti syndrome, the spleen rapidly but prematurely destroys blood cells. Syndrome characterized by increased splenic and portal pressures resulting in portal hypertension of unknown etiology. Possible causes of portal hypertension include toxins, infections (bacterial/malaria), and immunologic or genetic factors. The portal hypertension is accompanied by splenomegaly and esophageal varices. Liver failure is an uncommon finding. Anemia and thrombocytopenia accompany the hypersplenism.
This syndrome is characterized by abnormal enlargement of the spleen (splenomegaly) due to obstruction of blood flow in some veins and abnormally increased blood pressure (hypertension) within the veins of the liver (e.g., hepatic or portal veins), or the spleen (splenic veins). The disorder may be due to any number of different factors obstructing the portal, hepatic, or splenic veins including abnormalities present at birth (congenital) of such veins, blood clots, or various underlying disorders causing inflammation and obstruction of veins (vascular obstruction) of the liver.
Symptoms
In the early stages, symptoms of Banti syndrome include weakness, fatigue, anemia, and abnormal enlargement of the spleen. As the disorder progresses, the anemia becomes more severe. The anemia may be aggravated by a bleeding esophagus that may cause vomiting of blood and the passage of dark stools composed of decomposing blood. Ultimately, in some cases, the liver itself becomes enlarged and subdivided by fibrous tissue (cirrhosis). However, the spleen enlargement in Banti syndrome is the primary symptom.
Patients with Banti syndrome bruise easily, are more likely to contract bacterial infections, and carry a fever for longer periods.
Symptoms may also include abnormal accumulation of fluid in the abdominal cavity (ascites), weakness, fatigue; abnormally low levels of circulating red blood cells (anemia), white blood cells (leukopenia), and/or platelets (thrombocytopenia), and/or episodes of bleeding (hemorrhage) from the gastrointestinal tract.
Causes
Banti syndrome may occur due to several different factors obstructing, and abnormally increased blood pressure (hypertension) within, certain veins of the spleen (splenic veins) or the liver (e.g., hepatic or portal veins). These may include abnormalities present at birth (congenital) of such veins, blood clots, or various underlying disorders causing inflammation and obstruction of veins (vascular obstruction) of the liver, such as cirrhosis. Increased arsenic intake has also been implicated in some cases. Also, cases have occurred in patients taking long-term azathioprine, particularly after kidney transplantation.
Diagnosis
The diagnosis of Banti syndrome may is confirmed by a thorough clinical evaluation and a variety of specialized tests, particularly advanced imaging techniques such as splenic venography and magnetic resonance imaging (MRI). During MRI, a magnetic field and radio waves are used to create cross-sectional images of targeted parts of the body.
A thorough clinical evaluation and a variety of specialist testing, notably advanced imaging techniques such as splenic venography and magnetic resonance imaging, may be used to confirm the diagnosis of Banti’s syndrome (MRI). A magnetic field and radio waves are utilized in MRI to obtain cross-sectional images of certain body areas.
Treatment
Treatment of Banti syndrome is dependent upon the cause. If a contributing factor such as arsenic or azathioprine is identified, the exposure should be stopped.
The main clinical problem is bleeding from esophageal or gastric swollen blood vessels (varices). Active bleeding may be treated with vasoconstrictor drugs or other methods used to treat portal hypertension. Recurrent bleeding may require that blood flow be rerouted by a surgical shunt.
The treatment of gastrointestinal bleeding caused by portal hypertension and the therapy of hypersplenism are the mainstays of the management of Banti’s syndrome. Variceal ligation and endoscopic sclerotherapy were shown to be equally effective in 95 percent of patients with acute gastrointestinal bleeding, with a recurrence rate of roughly 20% and 3% recurrent bleeding, respectively. Some patients may also require shunt procedures as a last resort.
Patients with symptomatic hypersplenism, severe anemia needing repeated blood transfusions, or recurrent splenic infarction should consider surgical management. Both open and laparoscopic splenectomy procedures are available. In the open approach, the spleen is mobilized to the midline by dividing the lateral and superior pole attachments or a left sub-coastal or midline incision is made.
Banti’s syndrome has a favorable prognosis, with a 5-year survival rate of 100% after successful varices ligation. A case report published in the International Journal of Case Reports in Surgery demonstrated that the post-operative recovery was excellent in the patient. Due to severe anemia requiring repeated transfusions and large splenomegaly posing a risk of traumatic rupture, the patient had a splenectomy. During the follow-up period, the post-operative recovery was excellent, with a rapid increase in platelet level and a steady rise in hemoglobin level.
Banti’s syndrome and COVID-19
There hasn’t been any evidence of COVID producing Banti’s syndrome in the literature. A case of a 32-year-old male who presented with hematemesis was reported in a recent research publication. Subsequent follow-up revealed that the patient was suffering from ascites, splenomegaly, and bleeding varices, eventually completing the signs and symptoms of Banti’s syndrome.
Banti’s syndrome should be considered in a patient with hematemesis and splenomegaly, although it is a rare illness. The patient suffered from flu-like symptoms for three weeks. He did not seek any medical attention, ultimately developing Banti’s syndrome. His COVID-19 serology was positive.
COVID-19 is recognized to cause hypercoagulability, but the exact mechanism by which it causes Banti’s syndrome is unknown. It could be caused by thrombosis of the portal arteries or embolization of the portal circulation. There is still a lot of research to be done to figure out what causes Banti’s syndrome in COVID-19 positive patients.
References
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5385310/
- https://en.wikipedia.org/wiki/Banti%27s_syndrome
- https://jamanetwork.com/journals/jama/article-abstract/308163
- https://www.ncbi.nlm.nih.gov/books/NBK525984/
- https://www.ncbi.nlm.nih.gov/books/NBK1488/
- https://medlineplus.gov/download/genetics/condition/bannayan-riley-ruvalcaba-syndrome.pdf
- https://www.fda.gov/medical-devices/
- https://www.fda.gov/science-research/womens-health-research/owh-funded-research-autoimmune-diseases
- https://www.niams.nih.gov/health-topics/autoimmune-diseases
- https://www.ncbi.nlm.nih.gov/books/NBK459447/
- https://www.ncbi.nlm.nih.gov/books/NBK459452/
- https://www.ncbi.nlm.nih.gov/books/NBK1370/
- https://www.ncbi.nlm.nih.gov/books/NBK576418/
- https://www.ncbi.nlm.nih.gov/books/NBK459479/
- https://www.rarediseaseadvisor.com/disease-info-pages/
- https://www.clinicaltrials.gov/
- https://rarediseases.org/for-patients-and-families/information-resources/rare-disease-information/
- https://rarediseases.org/organizations/genetic-and-rare-diseases-gard-information-center/
- https://rarediseases.org/organizations/nihnational-institute-of-arthritis-and-musculoskeletal-and-skin-diseases/
- https://www.ncbi.nlm.nih.gov/books/NBK459433/
- https://www.ncbi.nlm.nih.gov/books/NBK580299/
- https://www.ncbi.nlm.nih.gov/books/NBK580299/
- https://www.ncbi.nlm.nih.gov/books/NBK10757/
- https://www.ncbi.nlm.nih.gov/books/NBK459459/
- https://en.wikipedia.org/wiki/Autoimmune_disease
- https://en.wikipedia.org/wiki/List_of_autoimmune_diseases
- https://en.wikipedia.org/wiki/Immune_disorder
- https://www.niaid.nih.gov/diseases-conditions
- https://www.niehs.nih.gov/health/topics/conditions/autoimmune/
- https://www.cancer.gov/publications/dictionaries/cancer-terms/def/autoimmune-disease
- https://medlineplus.gov/autoimmunediseases.html
- https://www.cancer.gov/publications/dictionaries/cancer-terms/def/autoimmune-disease
- https://clinicaltrials.gov/ct2/show/NCT04995783
- https://www.sciencedirect.com/topics/medicine-and-dentistry/autoimmune-disease
- https://www.gene.com/stories/autoimmune-disease-101
- https://www.genome.gov/Genetic-Disorders
- https://www.sciencedirect.com/book/9780123849298/the-autoimmune-diseases
- https://autoimmune.org/disease-information/
