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111 2 3 10 4 5 6 Benign Diseases of the Spleen 7 8 Refaat B. Kamel 9 1011 1 2 3 4 5 6 7 8 9 2011 1 Aims ● Splenic conservation, various tech- 2 niques. 3 ● Identifying the value and functions of the ● Splenic injuries and management. 4 spleen in health and diseases. 5 ● The role of spleen in haematological dis- 6 orders (sickle cell disease, thalassaemia, Introduction 7 spherocytosis, idiopathic thrombocy- 8 topenic purpura). The spleen has always been considered a mys- 9 terious and enigmatic organ. Aristotle con- ● Haematological functions of the spleen 3011 cluded that the spleen was not essential for life. (haemopoiesis in myeloproliferative 1 As a result of this, splenectomy was undertaken disorders, red blood cell maturation, 2 lightly, without a clear understanding of subse- removal of red cell inclusions and 3 quent effects. Although Hippocrates described destruction of senescent or abnormal red 4 the anatomy of the spleen remarkably accu- cells) and immunological functions 5 rately, the exact physiology of the spleen con- (antibody production, removal of partic- 6 tinued to baffle people for more than a 1000 ulate antigens as well as clearance of 7 years after Hippocrates. The spleen was thought immune complex and phagocytosis 8 in ancient times to be the seat of emotions but (source of suppressor T cells, source 9 its real function in immunity and to remove of opsonin that promotes neutrophil 4011 time-expired blood cells and circulating phagocytosis and production of 1 microbes, has only recently been recognised. “tuftsin”). 2 3 ● Effects of splenectomy on haematologi- 4 cal and immunological functions. Anatomy of the Spleen 5 ● Complications and sequelae of splenec- 6 tomy including overwhelming post- The development of the spleen begins in the 7 splenectomy infection (OPSI). fifth week of intrauterine life. Mesenchymal 8 ● Hyposplenism, asplenia and associated cells, between the two mesothelial layers of the 9 manifestations. mesogastrium, aggregate and differentiate as 5011 ● Indications for splenectomy whether the anlage of the spleen. Primitive vessels, 1 therapeutic or diagnostic. during the second month of gestation, vascu- 2 ● Alternatives to total splenectomy. larise these cellular aggregates to form a lobu- 311 lated embryonic spleen. Continued growth and 127 128 10 · UPPER GASTROINTESTINAL SURGERY formation occurs during fusion of the splenic Lymphatic vessels in the red pulp or white 1111 lobules. From the fourth to eighth months, pulp of the human spleen are few. Lymphatic 2 the spleen participates with the liver in haemo- capillaries originating in the capsule and tra- 3 cytopoiesis. After the eighth month and beculae converge on lymph nodes of the hilum 4 throughout postnatal life, the spleen resumes and pancreaticoduodenal lymph nodes. Nodes 5 haemocytopoiesis only when bone marrow is in the splenic hilum are often involved in 6 incapable of meeting the demands of the disease processes such as lymphoma, when the 7 body (extramedullary haemocytopoiesis), or in spleen is involved. Accessory spleens may be 8 pathological circumstances. confused with these lymph nodes, whose 9 The parenchyma of the spleen appears as appearance is vascular (haemolymph) on gross 1011 greyish-white areas, the white pulp scattered examination. 1 in a spongy deep-reddish-purple substance, 2 the red pulp. The white pulp consists of 0.2– 3 0.8 mm masses of diffuse and nodular lymphatic Physiology 4 tissue surrounding small arteries called central 5 The spleen is the largest mass of lymphoid tissue arteries. The white pulp undergoes involution 6 in the body. Like a lymph node, the spleen pro- between the ages of 10 and 14. After the age of 7 vides for the storage of lymphocytes and their 60, the spleen as a whole undergoes involution. 8 production; it removes foreign matter in the The red pulp possesses unique venous sinuses 9 blood by the reticular cells; it prolongs the life supported in a spongy reticular stroma con- 2011 of red cells by providing temporary shelter from taining free erythrocytes, macrophages, reticu- 1 certain ionic changes to which they are exposed lar cells and other cells. 2 in the circulation; it stores blood and can expel 3 the contents into the circulation during haem- 4 orrhage, exercise or at high altitudes. Not only 5 Blood Supply is the spleen involved in many systemic diseases 6 but unsuspected splenic abnormalities may The blood supply to the spleen is provided by 7 produce widespread effects. Unlike the lymph the splenic artery, the largest of the three 8 nodes, which are interposed in chains of lym- branches of the coeliac artery. During its course, 9 phatic vessels to filter lymph, the spleen is situ- it sends branches to (1) the stomach (via the 3011 ated in the course of the blood vascular system left gastroepiploic artery and a short gastric 1 to filter blood. Added to this, the spleen receives artery), (2) the pancreas (via the pancreatic 2 a disproportionate amount of the circulating artery) and (3) the spleen (via the end of the 3 blood volume for its relatively small size. Hence, splenic artery). About 3.5 cm from the spleen, 4 it becomes involved secondarily in a wide range the splenic artery divides into superior and infe- 5 of haematological disorders. rior terminal branches, each of which further 6 subdivides into several smaller branches prior 7 to penetrating the hilum of the spleen. Haematological Functions of 8 On the basis of comparative anatomy, the the Spleen 9 spleen has been divided into segments sepa- 4011 rated by fibrous septa [1]. Gupta et al. [2] Because of the peculiar anatomical arrangement 1 inferred segmentation of the spleen on the of its blood vessels, the spleen is ideally suited 2 basis of avascular planes. In one of our studies, as a site for fine quality control of the erythro- 3 we showed the parenchymal distribution of cyte population. It removes fragmented, 4 the splenic artery -and clarified the avascular damaged or senescent red cells from the circu- 5 planes in the human spleen. The mode of lating blood, a process known as “culling”. It 6 termination of the splenic artery was studied also plays a role in remodelling the surface of 7 in 25 cadavers. Observation of the parenchymal the maturing erythrocytes and in preserving the 8 distribution of the artery in 17 cases revealed normal relationship between their membrane 9 avascular planes that divided the spleen into surface area and volume. Target cells, which 5011 lobes, inside which other avascular planes have a relatively high ratio of membrane to 1 separated the lobes into segments [3] (Figure intracellular content, appear in the peripheral 2 10.1). blood soon after splenectomy. 311 128 129 BENIGN DISEASE OF THE SPLEEN 111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 9 Figure 10.1. Arterial supply of the spleen. Arrows indicate avascular planes coinciding with surface notches. (S, Splenic; ST, superior 3011 terminal, MT, middle terminal; IT, inferior terminal, LP, lower polar; SG, segmented.) 1 2 3 A variety of intra-erythrocyte inclusions are to sequester a significant number of platelets, 4 removed by the spleen (through a process however, and after splenectomy there is nearly 5 known as pitting), after which the red cells are always a transient thrombocytosis so that the 6 returned to the circulation. Among the inclu- need for preoperative platelet transfusion is not 7 sions removed are Howell–Jolly bodies, which important. The increase in platelet count occurs 8 are probably nuclear remnants, siderotic gran- intraoperatively shortly after splenic artery 9 ules, which are haemosiderin aggregates laid ligation during splenectomy. 4011 down during normal erythroid maturation, and 1 Heinz bodies, which are pathological aggregates Splenic Pooling and Hypervolaemia 2 of denatured haemoglobin (normally the per- 3 centage of these abnormal cells and inclusions It has been known for many years that plasma 4 does not exceed 3%). Thus after splenectomy, volume is increased in patients with splen- 5 Howell–Jolly bodies and siderotic granules may omegaly, while the red cell mass is normal or 6 be seen in the peripheral blood and the red cells even increased, despite the venous haematocrit 7 show striking changes in shape and size with the being depressed. Anaemia is to a large extent 8 appearance of acanthocytes, irregularly cre- due to haemodilution. Similar observations 9 nated cells and target cells (their percentage were reported in patients with Gaucher’s disease 5011 may reach up to 20–25%) (Figures 10.2 to 10.6). and massive splenomegaly. In patients with cir- 1 The human spleen unlike that of many rhosis of the liver, expansion of the plasma 2 animals contains relatively little blood and volume is common and is not closely related to 311 hence has no important storage role. It appears splenic enlargement. On the other hand, with 129 130 10 · UPPER GASTROINTESTINAL SURGERY 1111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 Figure 10.2. Target cells in the blood film. 9 3011 1 2 moderate to massive splenomegaly, the spleen very contractile and serve as a reservoir of blood 3 size does play a role, as there is a decrease in at high haematocrit. In times of “fight or flight”, 4 the plasma volume following splenectomy, splenic contraction, which is produced by 5 although it may remain above normal. myoepithelial cells in the capsule or trabeculae, 6 Some authors have postulated that increased transfers blood from the reservoir into the cir- 7 blood flow through an enlarged spleen acts as culation, and the splenic filtration function is 8 a functional arteriovenous shunt, the increased put “on hold”, since all blood flows via the fast 9 venous return to the heart causing a high pathways in contracted spleens, until the organ 4011 cardiac output together with an increase in relaxes again.
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