What Are the Different Types of Spleen Disorders?

Spleen diseases refer to the spleen itself and diseases related to the spleen. It mainly includes hematopoietic system diseases, infectious diseases, congestive splenomegaly, spleen injury, spleen occupying lesions, deformities, vascular lesions, and some rare diseases.

Basic Information

Chinese name

Spleen disease

Foreign name

spleen diseases

Classification

Hematological diseases, infectious diseases, deformities, etc.

Classification of Spleen Diseases

1. Spleen and hematopoietic system diseases The spleen and hematopoietic system are closely related to various diseases, such as hemolytic anemia, thrombocytopenic purpura, chronic leukemia, lymphoma, and myelodysplastic syndrome. Different blood diseases have different effects on the spleen.
(1) Hemolytic anemia: Usually related to congenital or hereditary factors and autoimmune disorders. The spleen is mainly involved in the pathogenesis of blood cell destruction or the production of autoantibodies. Congenital anemia mainly includes hereditary spherocytosis, hereditary oval erythrocytosis, pyruvate kinase deficiency, sickle cell anemia, anemia of globin production, etc. The main clinical manifestations are anemia, jaundice, and spleen Big. Autoimmune hemolytic anemia is caused by the abnormal destruction of red blood cells caused by the body's production of autoantibodies. According to serological characteristics, it can be divided into warm antibody type and cold antibody type. The former is more common. Splenectomy is effective for warm antibody type.
(2) Thrombocytopenic purpura: It is a systemic hemorrhagic disease caused by thrombocytopenia caused by autoantibodies. Primary thrombocytopenic purpura is common.
(3) Chronic leukemia: Chronic myelogenous leukemia caused severe pain in the spleen area and decreased platelets due to spleen infarction and peri-spleenitis. Swollen spleen may rupture or be insensitive to chemotherapy. Try splenectomy if systemic conditions allow. Chronic lymphocytic leukemia has similar indications for splenectomy.
(4) Lymphoma: It is a malignant tumor that originates from lymph nodes or other lymphoid tissues. It is often divided into Hodgkin disease and non-Hodgkin lymphoma. The clinical manifestations are painless lymph nodes. Swelling, spleen often swollen, cachexia, fever, anemia and other manifestations in late stage. Determining the histological type and clinical stage of lymphoma is of great significance in determining treatment options and prognosis.
(5) Myelodysplastic syndrome: also known as bone marrow fibrosis, is a diffuse fibrous tissue hyperplasia in the bone marrow of the whole body, accompanied by extramedullary hematopoiesis in the spleen, liver, lymph nodes, etc., mainly manifested as anemia, splenomegaly, fever, Bone marrow pain, bleeding, etc.
(6) Spleen-associated genetic metabolic disease: It is a class of disorders of lipid metabolism. Lipid storage disorders involving the monocyte-macrophage system mainly include glucocerebrosidosis (Gaucher disease) and sphingomyelin Disease (Niemman-Pick disease).
1) Gaucher's disease: It is an autosomal recessive disease. It is caused by the lack of -glucosidase and the accumulation of a large number of glucocerebrosides in monocytes and macrophages, mainly involving the liver, spleen, bone marrow, and lymph nodes. Clinical manifestations include anemia, splenomegaly, bleeding tendency, and bone pain. The indications for splenectomy include hypersplenism, extremely reduced platelets, and significant swelling of the spleen to affect cardiopulmonary function.
2) Niemman-Pick disease: It is also an autosomal recessive disease, which is very rare.
2. Infectious diseases (1) Acute infectious diseases: such as sepsis, typhoid fever, infectious mononucleosis, subacute bacterial endocarditis, etc. may be accompanied by increased destruction of red blood cells in the blood circulation, causing splenomegaly and spleen function. Hyperactive. After primary disease control, secondary hypersplenism can be relieved. Except for spleen rupture and spleen abscess, there is no indication for splenectomy.
(2) Chronic infections: such as recurrent malaria, tuberculosis, and black fever, etc., may be accompanied by varying degrees of splenomegaly and hypersplenism. Human immunodeficiency virus (HIV) infection can be accompanied by thrombocytopenia and easily cause bleeding.
3. Congestive splenomegaly Hepatic cirrhosis and portal hypertension caused by congestive splenomegaly and hypersplenism. Most of the western countries are alcoholic cirrhosis, and most of our country are post-hepatic cirrhosis and schistosomiasis cirrhosis.
4. Splenic mass lesions (1) Splenic cyst: can be divided into true and false. The former cyst wall is lined with endothelial or epithelial cells, such as skin-like cysts, epidermal-like cysts, lymphatic cysts and simple cysts, single or multiple. Occasionally congenital polycystic liver and polycystic kidney complicated with polycystic spleen. Parasitic splenic cysts are also true, mostly spleen hydatid disease, accounting for 2% to 3% of all hydatid disease. Pseudocysts are mostly formed by spleen injury due to old hematomas or spleen infarcts. Small cysts are often asymptomatic, and large cysts often cause left upper abdominal discomfort and indigestion due to mass effects. Ultrasound of the abdomen can detect the dark area of the splenic fluid. CT examination shows that the spleen has a clear and sharp circular low-density space. Large cysts can be treated with cystectomy, partial splenectomy, splenectomy or laparoscopic drainage as appropriate. Small non-parasitic cysts can be clinically observed and generally do not require treatment.
(2) Splenic abscess: It is often a complication of systemic infection, and infection by blood. In addition, spleen central rupture, splenic infarction, splenic artery ligation, or splenic artery embolization may be secondary to infection and form an abscess. The pathogenic bacteria are Staphylococcus and Streptococcus. The clinical manifestations were chills, high fever, pain in the left upper quadrant, increased white blood cell count, tenderness in the left upper abdomen, and abdominal muscle tension. X-ray examination showed enlarged spleen shadow, left diaphragm elevation, etc., B-mode examination showed fluid level. In addition to antibiotic treatment, splenectomy should be selected. Abdominal abscess can be used for drainage and drainage when the spleen adhesions are tight and difficult to remove.
(3) Spleen tumors: Primary splenic tumors are rare, and benign tumors are mostly hemangiomas, lymphangiomas, hamartomas, fibroids, and lipomas. Small tumors have no obvious symptoms due to slow growth, and large tumors may have symptoms such as localized space compression. Splenic hemangioma is more common, showing nodular or diffuse type, which can be secondary to infection, infarction, fibrosis, and calcification. Due to the role of arteriovenous communication, once spontaneous rupture, bleeding is more serious, and diagnostic splenic puncture should be contraindicated. Clinically, it is difficult to identify benign and malignant tumors of the spleen. Total splenectomy is usually used. Splenectomy for benign spleen tumors is effective.
1) Primary malignant tumors of the spleen: Lymphosarcoma, reticulosarcoma, fibrosarcoma, angioendothelial sarcoma, and lymphoma. The spleen often swells rapidly, causing discomfort, pain, and compression of the adjacent organs in the left upper abdomen. Due to the rapid progress and early transfer, the prognosis is poor. Primary splenic lymphomas include Hodgkin's lymphoma and non-Hodgkin's lymphoma, and their prognosis is also poor. The splenectomy plus radiotherapy or chemotherapy is the preferred treatment for splenic primary malignant tumors.
2) Metastatic tumor of the spleen: Usually refers to a malignant tumor derived from the non-hematopoietic system. The routes of metastasis are hematogenous, lymphatic and direct invasion. Hematogenous metastases usually occur in lung cancer, breast cancer, ovarian cancer, and malignant melanoma. Lymphatic metastasis mostly comes from abdominal organs; direct invasion comes from nearby organs, such as stomach and colon. Imaging studies can help improve the rate of diagnosis. When spleen metastatic cancer occurs, the primary disease is usually advanced, and the surgical treatment has lost its timing and significance.
5. Spleen injury The cause of spleen injury is spleen rupture caused by trauma in the first place, while iatrogenic spleen injury and spontaneous spleen rupture are less than 15%.
6. Other rare spleen diseases (1) Splenic aneurysm: It is the most common splanchnic aneurysm. There are more women than men, and the incidence of elderly people over 60 is higher than in other age groups. It has been confirmed that the occurrence of splenic aneurysms is related to a variety of factors. Local lesions of blood vessels, systemic diseases and local environment can affect the occurrence and development of splenic aneurysms, such as arteriosclerosis, portal hypertension, structural defects of the arterial wall, primary Hypertension, women and pregnancy, spleen artery trauma or iatrogenic spleen injury, splenic artery inflammation, infection or necrosis, orthotopic liver transplantation, etc. Clinical manifestations are often left quarter rib discomfort or pain, nausea, belching, anorexia, etc. When the tumor is large, there may be left shoulder or left back radiation pain. On examination, if the tumor is large, the mass can be touched, and vascular noise can be heard in the left upper abdomen. Massive intra-abdominal hemorrhage and acute hemorrhagic shock may occur during rupture, and gastrointestinal bleeding may occur when gastrointestinal tract is broken. Abdominal angiography, CT, MRI, ultrasound, X-ray and other imaging examinations can help confirm the diagnosis. Surgery is the preferred treatment. The surgical method can be selected according to the situation of simple splenic aneurysm resection, tumor ligation, tumor resection and splenic artery anastomosis or reconstruction, tumor and spleen resection. Interventional therapies such as arterial embolism can also be used.
(2) Spleen infarction: Splenic infarction is the ischemic necrosis of the distal part of the splenic artery due to blockage of the main or branch vessels of the splenic artery. It is often complicated by hematological diseases such as acute leukemia, chronic myeloid leukemia, myelofibrosis, and non-Hodge Gold lymphoma, polycythemia vera, sickle cell anemia, etc., cardiovascular diseases such as atrial fibrillation, infective endocarditis, and infectious diseases such as sepsis, malaria, typhoid, etc. The embolization of the small arterial branch is often asymptomatic, while the embolization of the larger arterial branch may cause severe left upper abdominal pain or tear-like pain, which radiates to the left shoulder with nausea and vomiting, and has obvious peritoneal irritation. Abdominal puncture may have dark red thin bloody fluid. The treatment of splenic infarction is mainly non-surgical treatment. Splenectomy can be performed when secondary infection leads to spleen abscess.
(3) accessory spleen: refers to spleen tissue that exists outside the normal spleen and has a similar structure to the main spleen and has a certain function, with an incidence of more than 10%. The paraspleen is mostly located near the spleen hilum, about 1/4 of which is located around the blood vessels of the spleen and the tail of the pancreas. It has a dark purple sphere or hemisphere, ranging in size from several millimeters to several centimeters. Asymptomatic patients do not need to be treated, and should be surgically removed when intestinal obstruction, parasplenic torsion, rupture and bleeding occur.
(4) Wandering spleen: The spleen that moves away from the normal anatomical position and moves to other parts of the abdominal cavity is called the wandering spleen. Mostly caused by congenital spleen pedicle or spleen ligament is too long, or spleen ligament is absent, or the enlarged spleen stretches the ligament to relax or weak abdominal muscles. The main clinical manifestations are abdominal masses, which often cause compression symptoms of adjacent organs. About 20% of the migratory spleen has severe abdominal pain when the spleen is twisted, which can be accompanied by shock.
(5) Splenic implantation: Also known as spleen implantation, refers to the splenic tissue cell clusters that spontaneously scatter when a damaged spleen is ruptured to re-establish blood circulation on the surface of one or more organs and grow to the size of an envelope Varying nodules. The main sites of spleen tissue implantation are the small intestine serosa, the omentum, the parietal peritoneum, the mesentery, and the diaphragm. Spleen tissue implantation is usually asymptomatic.
(6) Splenic peliosis: It is a rare spleen vascular disease, often accompanied by liver purpura. Related to the use of steroid hormones, oral contraceptives, previous tuberculosis and tumor history. Androgens may also play a role in the pathogenesis. Solitary spleen purpura is often asymptomatic. Larger cases may cause rupture and bleeding and require surgery.