spleen disease.ppt
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1、,Spleen Disease, The splenic primordium becomes evident during the fifth week of gestation as an outgrowth of the dorsal mesogastrium, which migrates to the left upper quadrant. The gross appearance of the spleen is the result of its development from multiple anlage, resulting in an organ with multi
2、ple clefts.,Embryoiogy, 12 to 15 cm in length, 4 to 8 cm in width and 3 to 4 cm in thickness, Average weight is about 150 to 250 g, lies in the shelter of the 9th to 11th rib at the left side of the abdominal cavity,Anatomy,A pancreas B spleen C duodenum 5 Hepatic portal vein 8 celiac artery (trunk
3、) 9 superior mesenteric artery 13 superior mesenteric artery, The spleen receives its arterial supply from the splenic artery, which originates in the celiac axis. After its origin, the splenic artery courses along the superior edge of the pancreas, with multiple branches into the pancreatic parench
4、yma. The artery then g ives off several branches into the spleen, the first being the superior polar artery. There are other arterial vessels to the spleen from the left gastroepiploic artery and the short gastric artery., The splenic veins follow the arterial distribution closely, and the main sple
5、nic vein emerges from the spleen following a course to join the superior mesenteric vein, forming the portal vein.,Functions of the Spleen,Circulation through the spleen is about 150 to 200 ml/min, or about 5% of the cardiac output. The spleen has traditionally been ascribed four functions : filtrat
6、ion immunological reservoir hematopoietic, Filtration abnormal or senescent red blood cells , particulate antigens such as microorganisms or antigen-antibody complexes., Immunological trapping of antigens, homing of lymphocytes, antibodyand lymphokine production, microphage activation, immunoglobuli
7、n and antibody synthesis , affects the capability of cellular populations in other lymphoid organs, Reservoir, Hematopoietic, Spleen harbors about one-third of the total platelet mass and a large number of granulocytes., The hematopoietic functions are minimal in humans and much more prominent in ot
8、her species.,Pathological Conditions of the Spleen, Splenic Rupture, Benign Lesions of the Spleen, Maligant Lesions of Spleen,Splenic Rupture,The spleen can rupture from three underlying causes: trauma, spontaneous rupture, and pathological rupture.,Traumatic rupture of the spleen remains the most f
9、requent indications for splenectomy. Pathologicalcauses of splenic rupture include infiltration of the spleen by reactive lymphoid cells or by neoplastic cells. Most cases attributed to spontaneous rupture of the spleen are actually due to an undiagnosed pathological process.,Conditions associated w
10、ith defective or absent splenic function are grouped together as being conditions of hyposplenism. Those conditions are characterized by the presence of Howell-Jolly bodies in the peripheral circulation. Conditions associated with hypersplenism remain the most frequent indication for elective splene
11、ctomy ,these can be divided into those conditions in which the spleen is normal but increased destruction of abnormal blood elements causes hypersplenism, and those in which there is a primary disorder of the spleen that results in increased destruction of abnormal blood cells.( Table 1).,Pathologic
12、al Conditions of the Spleen,TABLE 1 Disorders Associated With Hypersplenism 1.Disorders associated with sequestration of abnormal blood cells in an intrinsically normal spleen. A. Congenital disorders of erythrocytes 1. Hereditary spherocytosis 2. Hereditary elliptocytosis 3. Hemoglobinopathies B. A
13、cquired disorders of erythrocytes 1.Autoimmune hemolytic anemia 2.Parasitie disease(e.g.,malaria,babesiosis) C. Autoimmune thrombocytopenia D. Autoimmune neutropenia 2. Disorders of spleen resulting in sequestration of normal blood cells A. Disorders of cordal macrophages: Bantis syndrome, storage d
14、iseases, parasitie diseases(e.g.,kala-azar),Langerhans cells histiocytosis, malignant histiocytosis B. Infiltrative disorders:leukemias,lymphomas,plasma cell dyscrasias, myeloid metastatic carcinnoma C. Vascular abnormalities D. Splenic cysts E. Hamartomas 3. Miscellaneous conditions A. Hyperthyroid
15、ism B. Hypogammaglobulinemia C. Progressive multifocal leukoencephalopathy Source: Reiman (1997).3,Benign Lesions of the Spleen, Hemangioma Lymphangioma Peliosis Hemangioendothelioma Hamartomas Other Benign Lesions Nonparasitic Cysts Parasitic Cysts, Hemangioma, This is the most common benign primar
16、y neoplasm of the spleen and can present with splenomegaly, Lesions can be solitary or multiple, and are usually blue-red, well-circumscribed nodules., Microscopically,they usually appear as endothelium-lined spaces, and are known as cavernous hemangiomas., Treatment of these lesions is usually sple
17、nectomy,Benign Lesions of the Spleen,Gross images: It weighed 425 grams and measured 15 x 9 x 7 cm. A splenectomy was performed.,Micro images:representative section of tumor, Lymphangioma, These lesions are less common than hemangiomas, and are usually subcapsular, appearing as soft, compressible, m
18、ulticystic lesions on the splenic surface., When large, they present with splenomegaly as an indication., There are case reports of patients presenting with hypersplenic syndromes, consumptive coagulopathy, and even portal hypertensionwith these lesions.,Benign Lesions of the Spleen, Peliosis, These
19、 rare lesions bear a superficial resemblance to vascular neoplasms of the spleen., They consist of blood-filled cysts distributed in patches or diffusely, and can result in splenomegaly., Intraperitioneal hemorrhage can result from the rupture of these lesions.,Benign Lesions of the Spleen, Hemangio
20、endothelioma, These rare lesions is thought to be intermediate between hemangioma and angiosarcoma., They usually contain cellular atypia, differentiating them from hemangiomas., These lesions may present with splenomegaly or rupture, and should suggest the possibility of a malignabt vascular neopla
21、sm., Hamartomas, These are focal developmental abnormalities within the normal spleen, rather than being true neoplasms., They consist of normal cellular elements in disarray, and are usually found incidentally,Benign Lesions of the Spleen,Maligant Lesions of Spleen,There are many malignancies that
22、affect the spleen.,. Lymphoproliferative disorders a. Non-Hodgkins lymphoma b. Hodgkins disease c. Chronic lymphocytic leukemia d. Hairy cell leukemia e. plasmacytoma f. Waldenstrms macroglobulinemia . Myeloproliferative disorders a. Chronic myelogenous leukemia b. polycythemia vera c. Myelofibrosis
23、 (agnogenic myeloid metaplasia) d. Essential thrombocythemia .Vascular tumors a. Hemangiosarcoma b. Lymphangiosarcoma . Metastatic tumors: breast,lung,melanoma,etc .Other lesions a. Sarcoma: fibrosarcoma, leiomyocarcoma, Kaposis sarcoma Source: Adapted from Giles and Lim(1997). 7,Maligant Lesions of
24、 Spleen,Non-Hodgkins Lymphoma, Non-Hodgkins lymphoma is a diverse group of disease with a wide range of biological behaviors., They may be very aggressive and rapidly fatal, or may behave as one of the most indolent and well-tolerated malignancies afflicting man., The role of the surgeon in Non-Hodg
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