Signs :

• palpation
• Bailey's bimanual palpation
• hooking palpation (accrochage) : patient with left arm over head
• percussion
• arose obtuseness (above upper border of 9^th rib)
• descended obtuseness (below lower border of 11^th rib)
• splenectopia
• splenoptosis
• tumor lienis => splenomegaly

• splenectopia / splenectopy : displacement of the spleen; wandering or floating spleen
• splenoptosis / splenoptosia : prolapse or downward displacement of the spleen.
• splenonephroptosis : downward displacement of the spleen and kidney on the same side
• tumor lienis : enlargement of the spleen less in degree than splenomegaly.
• splenomegaly / splenomegalia / splenoparectasis / splenauxe : enlargement of the spleen.
• splenohepatomegaly / splenohepatomegalia : enlargement of both spleen and liver (hepatomegaly).
Aetiology :
• chronic myeloid leukemia (CML)
• congestive splenomegaly / Banti's disease / splenic anemia : enlargement of the spleen occurring secondary to portal hypertension (cirrhosis, obstruction of splenic or extrahepatic portal axis), with ascites, anemia, thrombocytopenia, leukopenia, and episodic hemorrhage from the gastrointestinal tract
• thrombophlebitic splenomegaly / Opitz's disease : enlargement of the spleen due to thrombosis of the splenic vein
• Gaucher's disease or splenomegaly
• hemolytic splenomegaly : that associated with hemolytic anemia
• infectious or infective splenomegaly : splenomegaly associated with an infections.
• Egyptian splenomegaly : that caused by Schistosoma mansoni
• myelophthisic splenomegaly : enlargement of the spleen marked by decrease in myeloid tissue and by fibrosis
• siderotic splenomegaly / Gandy-Nanta disease / Gandy-Gamna spleen : splenomegaly characterized by marked fibrosis with deposit of iron and calcium (Gandy-Gamna nodules / nodules tabac : brown or yellow pigmented nodules seen in the spleen in certain cases of enlargement, such as Banti's disease and siderotic splenomegaly)
• spodogenous splenomegaly : enlargement of the spleen attributed to accumulation of erythrocytes in the organ.
• tropical splenomegaly syndrome (TSS) :
• visceral leishmaniasis
• malarial hyperreactive spleen syndrome / hyperreactive malarious splenomegaly (HMS) : a syndrome of massive splenomegaly, hepatomegaly, anemia, and elevated serum IgM levels that occurs in some areas where malaria is endemic, such as parts of sub-Saharan Africa and New Guinea. Lymphocytic infiltrates are present in hepatic sinusoids, and the association with chronic malarial infection is suggested by a polyclonal increase in IgM, a very high titer of IgM antibodies to Plasmodium falciparum, and often by a therapeutic response to malarial chemoprophylaxis
Symptoms & signs : the involvement of left hemidiaphragm may cause pain in homolateral shoulder due to involvementof phrenic nerve. Spontaneous pain is generally accentuated by abdominal palpation, expecially in concomitant acute perisplenitis; a true defensive contracture occurs only in splenic infarction, acute abscess or torsion. The spleen becomes palpable below the left costal arch as mobile tumefaction during respiratory cycles if the increase in volume is > 1/3. At percussion the lower border of obtusity extends below rib X-XI along left mid-axillary line
Laboratory examinations (splenometry) :
• echography : increase of at least 2 out of 3 diameters (normally 12-14 cm for longitudinal diameter, 12 cm for anteroposterior diameter, and 8 cm for transverse diameter)
• CT is used to monitor engraftment of autografts and in differential diagnosis between lymphadenopathy and accessory spleen (angio-CT)
• MRI has poor specificity and is used only to study hematomas
• splenic portography is no longer used
• selective angiography is used only for diagnosis of doubtful masses and preoperatory embolization
• laparoscopic or echo-assisted FNAC or FNAB (contraindicated in severe thrombocytopenia, acute perisplenitis, polycythemia and thrombocytemia (urgent splenectomy could increase circulating platelets)
• splenic scintigraphy with ^99mTc autologus denatured RBC (to study culling) or ^99mTc-colloidal sulfur (to study pitting of the 15-20% of particles which deposit into the spleen parenchyma). Platelet half-life can be studied with ¹¹¹In-autologous platelets to assess the main sequestration location (liver, spleen or both)
‡
• hypersplenism (Chauffard, 1907) : increase in activity of RES cells => pancytopenia

Aetiology :
• primary / idiopathic (more common in females)
• secondary
• inflammation (infectious mononucleosis, bacterial endocarditis, acute infections, tuberculosis, brucellosis, Felty's syndrome, SLE)
• increased erythrocatheresis (hereditary spherocytosis, thalassemia major)
• congestive splenomegaly
• acute or chronic leukemia, lymphomas, polycythemia
• neoplasia (splenic hamartomas, metastatic carcinomas)
• infiltrates (sarcoidosis, Gaucher's disease, amyloidosis)
Pathogenesis : pancytopenia (anemia, thrombocytopenia, leukopenia)
Symptoms & signs : not related to pancytopenia or spleen size
Therapy : splenectomy
• hyposplenism (Dameshek, 1955) : a condition characterized by diminished functioning of the spleen.

Aetiology :
• operative splenectomy : surgical removal of the spleen is performed for severe splenic trauma, for splenic cysts, or as part of a resective procedure for tumours of the spleen or adjacent organs (Jackson JW. Operations for carcinoma of the thoracic oesophagus and cardia. In: Dudley H, Pories W, Carter D, eds. Rob and Smith's operative surgery. Alimentary tract and abdominal wall. 4th ed. Vol 1. London: Butterworths, 1983:177). Partial splenectomy with retention of some splenic tissue is increasingly practised^ref. However, in view of the uncertainty of the level of splenic function achieved by partial splenectomy or autotransplantation of splenic tissue it is prudent to institute similar measures to prevent infection in these patients as for asplenic subjects. Though there has been a reduction in the use of splenectomy for staging haematological malignancies such as Hodgkin's disease, the procedure may still be used in hereditary spherocytosis, immune thrombocytopenic purpura, and autoimmune haemolytic anaemia. Patients receiving immunosuppressive chemotherapy or radiotherapy or both are at greatest risk of serious infection after splenectomy^ref
• functional hyposplenism may be detected on a blood film as red cells containing Heinz and Howell-Jolly bodies, thrombocytosis, and monocytosis (Hoffbrand AV, Lewis SM. Postgraduate haematology. 3rd ed. Oxford: Blackwell Scientific, 1989:20). Splenic dysfunction may occur secondary to sickle cell anaemia (HbSS, HbSC), thalassaemia major, essential thrombocythemia, and lymphoproliferative diseases (Hodgkin's disease, non-Hodgkin's lymphoma, and chronic lymphocytic leukaemia). Functional hyposplenism may also occur in celiac disease^ref, inflammatory bowel disease (Jewell DP. Crohn's disease. In: Weatherall DJ, Ledingham JGG, Warrell DA. The Oxford textbook of medicine. 2nd ed. Oxford: Oxford University Press, 1987;12:122-3), and dermatitis herpetiformis (Losowsky MS. Malabsorption. In: Weatherall DJ, Ledingham JGG, Warrell DA, eds. The Oxford textbook of medicine. 2nd ed. Oxford: Oxford University Press, 1987;12:106). Bone marrow transplantation is a further cause of functional hyposplenism, leading to an increased incidence of pneumococcal bacteraemia^ref and H influenzae type b pneumonia^ref. Though most studies have shown an association of infections with chronic graft versus host disease^ref, in some patients there is also impaired splenic function, with Howell-Jolly bodies^{ref1, ref2}. Infections occur six months after bone marrow transplantation, after co-trimoxazole prophylaxis against pneumocystis pneumonia has been stopped^ref. More detailed recommendations for managing patients receiving bone marrow transplantation have been given by Fielding^ref. All patients having bone marrow transplantation should be immunised against pneumococcal infection 9-12 months after trans-plantation, and those with chronic graft versus host disease should receive appropriate long term prophylactic antibiotics, especially if the spleen has been removed or irradiated before transplantation.
• hyperthryoidism
• sarcoidosis
• systemic lupus erythematosus
• amyloidosis
• congenital asplenia is associated with cardiac abnormalities and biliary atresia^ref
• effect of age : asplenic children under 5--and especially infants splenectomised for trauma--have an infection rate of over 10%, much higher than in adults (<1%)^ref. Children with sickle cell anaemia (HbSS, HbSC) are at especially high risk of overwhelming infection^ref. Other groups of patients may for other reasons be considered at risk of infection with encapsulated organisms--for example, patients with chronic lymphocytic leukaemia or multiple myeloma^ref and patients with HIV related disease or other immunodeficiency states. Local or national guidelines for the prevention of infection in these groups should be consulted (Gibb D, Walters S. Guidelines for management of children with HIV infection. 2nd ed. Horsham: AVERT, 1993:29pp)
Laboratory examinations : Howell-Jolly and Heinz bodies, siderocytes, pit cells, target cells, acanthocytes, transient thrombocytosis, persistent lymphocytosis and monocytosis, IgM and tuftsin deficiencies
Complications :
• immune deficiency => bacterial sepsis / overwhelming postsplenectomy infection (OPSI)^{ref1, ref2, ref3}

Epidemiology : existing published data suggest an incidence of OPSI varying from 0.18 to 0.42 cases/100 person years^{ref1, ref2}; the highest OPSI risk lies within the first few years after surgery^ref. There are approximately 50 000 asplenic individuals living in the UK
Aetiology : capsulated bacteria (Streptococcus pneumoniae^{ref1, ref2, ref3} (90%, with a mortality of up to 60%), Neisseria meningitidis^{ref1, ref2}, Listeria monocytogenes, Escherichia coli^ref, Klebsiella spp., Salmonella typhimurium, Haemophilus influenzae^ref, Staphylococcus aureus, Capnocytophaga canimorsus^ref, Pseudomonas aeruginosa, Plasmodium spp.^ref, Babesia spp.^ref)
Risk factors :
• age < 1 yr : 15%
• age 1-5 yrs : 10%
• splenic rupture : 0.5-2%
• spherocytosis : 3%
• chronic idiopathic thrombocytopenic purpura (1.5-2.5%)
• lymphoma (7%)
• thalassemia (20-25%)
Pathogenesis : splenic macrophages have an important filtering and phagocytic role in removing bacteria and parasitised red blood cells from the circulation^ref. Though the liver can perform this function in the absence of a spleen, higher levels of specific antibody^ref and an intact complement system are probably required. The ability of an asplenic patient to mount an adequate protective antibody response may relate more to the indication for or age at splenectomy and to the presence of underlying immune suppression than to the absence of the spleen.
Symptoms & signs : hyperpirexia, vomiting, hypotension, CID, adrenal necrosis and sudden death
Duration of risk : though most infections occur within the first 2 years after splenectomy, up to a third may be manifested at least 5 years later. Cases of fulminating infection have been reported > 20 years after splenectomy^ref. The risk of dying of serious infection, though unquantifiable^ref, is clinically significant and almost certainly lifelong. Patients falling into all the above categories, once identified, should receive appropriate vaccination and advice about lifelong antibiotic prophylaxis (Chief Medical Officer. Asplenic patients and immunisation. Update 1. London: HMSO, 1994:3)
Prevention^ref :
• immunisation has become accepted as an integral part of the preventive strategy against serious infection in the asplenic population^ref, but OPSI episodes classified as vaccine failures are regularly described^ref. Asplenia in itself is not a contraindication to routine immunisation. Normal inoculations, including live vaccines, can be given safely to children and adults with absent or dysfunctional spleens.
• anti-pneumococcal vaccination (mandatory in babies aged < 2 yrs and recommended in adults) : under use of the pneumococcal vaccine has been attributed to controversy about its effectiveness in high risk patients^ref. Few controlled studies have demonstrated vaccine efficacy in asplenic patients but 2 recent reports support its use :
• a programme of pneumococcal vaccination and prophylactic antibiotics effectively prevented serious infection in a population of 280 children for an average of 4.3 years after splenectomy^ref
• in a series of over 200 adult asplenic haematology patients who had been immunised, only 4 episodes of pneumococcal sepsis were seen in 13 years and in all 4 episodes the infecting serotype was not included in the vaccine^ref.
Up to 20% of asplenic individuals may show a poor immunological response, with antibody values declining more rapidly than in those still possessing a spleen^ref. Second, in contrast to the accepted protective antibody value for H influenzae type b, there is no agreed protective titre against S pneumoniae, and the current measurement of pneumococcal antibodies gives a total titre against the 23-valent polysaccharide vaccine, whereby a good response against one serotype might mask a failed or poor response to other serotypes. Decisions on the timing of booster immunisations are also difficult but the proposal to base this on antibody measurement has been made. The optimal frequency for checking antibody titres has yet to be established but 2 recommendations have been published. The first suggested antibody measurement preimmunisation, 1 month after, and then at 3 and 5 years postimmunisation^ref, whereas the second group advocated a 3 week postimmunisation check then measurement at yearly intervals^ref. A poor response to vaccination was observed in 28% of patients and a good response in 72%, respectively, determined using an ELISA before vaccination, at peak, and follow-up. During the follow-up period of 7.5 years (range 3.5-10.5 years) after vaccination, and despite repeated revaccination in many cases, a total of 5 episodes (in 3 patients) of pneumococcal infections were reported, all confined to the poor responder group. Revaccination did not improve antibody levels in this group. The median age at vaccination was significantly higher in the group of poor responders (p=0.0006). None of the following factors could predict a poor antibody response: gender, disease activity or aggressiveness in hematological malignancies, previous radiotherapy and/or chemotherapy, time between splenectomy and pneumococcal vaccination, time between chemotherapy/radiotherapy and study pneumococcal vaccination (1 year), or the presence of hypogammaglobulinemia^ref. Individuals who fail to respond could be targeted for other measures such as maintaining lifelong antibiotic prophylaxis.
Current vaccine : the currently available polyvalent pneumococcal vaccine contains purified capsular polysaccharide from the 23 most prevalent serotypes^ref. The vaccine is > 90% effective in healthy adults under the age of 55^ref. Once vaccinated, failure of protection may relate to waning specific antibody levels or be due to infection with serotypes not represented in the vaccine. Children under 2 years of age have inherently reduced ability to mount an antibody response to polysaccharide antigens. However, pneumococcal vaccine may have some (if reduced) efficacy for particular serotypes. The same considerations apply to other patients who have functional hyposplenism because of an underlying disorder^ref. The vaccine is best avoided in pregnancy (Walker G. ABPI data sheet compendium 1994-95. London: ABPI, 1995:986).
Timing : the vaccine should be given a minimum of 2 weeks before elective splenectomy in order to ensure an optimal antibody response. If this is not practicable the patient should be immunised as soon as possible after recovery from the operation and before discharge from hospital. The general practitioner should be notified of the splenectomy and vaccinations given in order to avoid potential reactions due to premature reimmunisation. Unimmunised patients splenectomised some time earlier should be immunised at the first opportunity. Immunisation, however, should be delayed at least six months after immunosuppressive chemotherapy or radiotherapy, during which time prophylactic antibiotics should be given. Hyposplenic patients should be immunised as soon as the diagnosis is made, though because of the reduced efficacy in young children (Department of Health. Immunisation against infectious disease. London: HMSO, 1992:102) it may be better to rely initially on prophylactic antibiotics and immunise after the second birthday. Reimmunisation of asplenic patients is currently recommended every 5-10 years. It may be necessary to revaccinate more frequently, particularly if there is an underlying disease causing immunosuppression. Antibody levels may decline more rapidly than expected in asplenic patients^ref and reimmunisation may be required as early as three years after the first dose, especially in lymphoproliferative disorders or sickle cell anaemia^ref
Adverse reactions : side effects of immunisation are usually self limiting hypersensitivity, with pain and swelling at the site of the injection after 24 hours. Much less commonly fever, malaise, and generalised aches disappearing after 48-72 hours may occur. Occasional patients with chronic immune thrombocytopenic purpura may have a relapse of their thrombocytopenia after immunisation^ref
• anti-Hemophilus influenzae : most children in Britain up to 4 years of age will have received H influenzae type b vaccine. Over 18 years most patients will have acquired some immunity through natural exposure but this may not provide adequate protection in the context of an absent or dysfunctional spleen. The vaccine has been shown to be immunogenic in patients with impaired splenic function associated with sickle cell anaemia^ref. The level of antibodies to H influenzae type b required for protection is known for people with an intact spleen. There is evidence that a higher specific antibody level is required in patients lacking a spleen^ref. The need for reimmunisation is unclear.
• anti-Neisseria mengitidis vaccination : in Britain meningococcal infection is most commonly due to a group B strain^ref. The present meningococcal vaccine covers groups A and C, which occur more commonly abroad. As the protection conferred with the current vaccine is of short duration, meningococcal immunisation is not routinely recommended for asplenic patients except when travelling to areas where there is an increased risk of group A infection. Otherwise the vaccine should be restricted to groups for whom it is already specifically recommended--that is, close contacts of cases due to group A or C disease and outbreaks in closed or semiclosed institutions^ref.39 Reimmunisation should be considered after 2 years in those remaining at risk, especially children. A forthcoming conjugated vaccine should provide longer lasting immunity
• anti-influenza vaccine is recommended yearly for patients with "immunosuppression due to disease or treatment" (Chief Medical Officer. Influenza immunisation. London: HMSO, 1993. (PL/CMO (93)13.)) and may be of value to asplenic patients by reducing the risk of secondary bacterial infection.
• the use of antibiotic prophylaxis remains controversial because there is a lack of good clinical data demonstrating its efficacy in asplenic patients^ref.In a series, S pneumoniae caused 9 of 10 infections in patients undergoing antibiotic prophylaxis and prophylactic oral penicillin or amoxicillin failed to prevent sepsis in 6 episodes, despite the isolates being sensitive to penicillin in vitro. The specific prophylactic regimen was not reported for 2 patients, but in one case, prophylactic erythromycin was being taken and the infecting pneumococcal strain was resistant to the macrolide agent. Increasing antimicrobial resistance, especially to commonly used antibiotics, such as the penicillins or macrolides, has provoked concerns over continuous antibiotic prescription. This issue, together with patient compliance difficulties, has influenced some to advocate a policy of holding a reserve antibiotic supply for self prescribing at the earliest signs of infection^{ref1, ref2}. Prophylactic oral phenoxymethylpenicillin has been used effectively for years in children with sickle cell anaemia^ref. Though amoxicillin has been recommended more recently^ref, this drug may be less well tolerated in young children and is more expensive. The advantages of amoxicillin over penicillin in adults are that it is better absorbed as an oral preparation and it has a broader spectrum and a longer shelf life^ref. Patients who are allergic to penicillin should be offered erythromycin. Lifelong prophylactic antibiotics should be offered in all cases, especially in the first two years after splenectomy, for all children aged up to 16,30 and when there is underlying impaired immune function^ref. In addition, for patients not allergic to penicillin a supply of amoxicillin should be kept at home (and taken on holiday) and used immediately should infective symptoms of raised temperature, malaise, or shivering develop. In such a situation the patient should seek immediate medical help. In the event of a feverish illness patients taking erythromycin as prophylaxis should increase the dose to a therapeutic level or change to an alternative broader spectrum preparation and seek medical advice immediately. Antibiotic prophylaxis may not prevent sepsis. Indeed, phenoxymethylpenicillin does not cover H influenzae and neither does amoxycilin reliably. The emergence of antibiotic resistant bacterial strains^ref must be considered if empirical treatment of sick patients is to be used. Local resistance patterns may dictate the need to use other antibiotics.
• recommendations for travellers : asplenic patients should be strongly advised of the increased risk of severe falciparum malaria. Scrupulous adherence to antimalarial prophylaxis cannot be overemphasised, and specialist advice from an infectious disease or tropical disease unit or the local consultant in communicable disease control should be sought. Meningococcal A plus C vaccine is recommended for all those travelling to sub-Saharan Africa, India, and Nepal^ref. Patients who are not otherwise taking antibiotic prophylaxis should do so during periods of travel^ref and should keep a therapeutic course of antibiotics with them for the duration of the holiday, taking into account resistance patterns such as the high incidence of penicillin resistant pneumococci in Spain and some other European countries.
• other measures : it is essential to educate patients regarding the risk and the importance of prompt recognition and treatment of infections. Asplenic patients should be encouraged to wear a Medic-Alert disc (Medic-Alert Foundation (a registered charity), 156 Caledonian Road, London N1 9UU) and carry a card with information about their lack of a spleen, other clinical details, and contact telephone numbers. In an emergency this information may be life saving.
• environmental : protective clothing and washing after potential exposure in endemic areas for histoplasmosis, babesiosis, and malaria may be beneficial.
• animal bites : ensure adequate antibiotic cover after dog (and other animal) bites, as asplenic patients are particularly susceptible to infection by Capnocytophaga canimorsus and should receive a five day course of co-amoxiclav (erythromycin in allergic patients).
• tick bites : babesiosis is a rare tickborne infection, and patients (especially those in contact with animals) should be warned of the danger of tick bites transmitting the disease. Clinical presentation is with fever, fatigue, and haemolytic anaemia. Diagnosis is made by identifying parasites within red cells on blood films and by specific serology. Quinine (with or without clindamycin) is usually effective treatment^ref
• mosquito bites : travel to areas where malaria is endemic should be discouraged. Patients should be made aware of their increased risk and advised about chemoprophylaxis relevant to local patterns of resistance and measures to reduce exposure to malaria parasites.
Treatment of acute infection : in suspected pneumococcal, meningococcal, or other serious infection immediate medical attention is required. Primary care physicians attending a known asplenic patient with clinically significant infection should (provided there is no history of penicillin allergy) give an immediate dose of intramuscular or intravenous benzylpenicillin before transfer to hospital. The intravenous route is preferable. For adults and children over 10, 1200 mg (2 MU) benzylpenicillin should be dissolved in 10 ml water for injection and injected over three to four minutes. A blood sample can be taken for culture immediately before giving the penicillin but the injection should not be delayed if facilities are not immediately to hand. Once the patient has been admitted to hospital blood samples should be taken and intravenous benzylpenicillin continued--but for patients who have been receiving antibiotic prophylaxis, patients allergic to penicillin, patients with possibly resistant organisms, and children under 5 cefotaxime or ceftriaxone should be given instead. Patients allergic to penicillin who are also allergic to cephalosporins may be given chloramphenicol after taking expert advice. Dosing regimens for antibiotic prophylaxis and treatment :

antibiotic		oral prophylaxis	treatment for suspected infection (established infection may require much higher doses given in hospital)
penicillin	adult	250-500 mg 12 hourly (if compliance is a problem 500 mg once daily is acceptable) (phenoxymethylpenicillin (oral))	1.2 g 4-6 hourly (benzylpenicillin (intravenous))
	child aged 5-14 years	250 mg 12 hourly (phenoxymethylpenicillin (oral))	200-300 mg/kg per day in 6 divided doses (maximum 6 g) (benzylpenicillin (intravenous))
	child under 5 years (seek expert advice for neonatal doses)	125 mg 12 hourly (phenoxymethylpenicillin (oral))
erythromycin (base)	adult+child over 8 years	250-500 mg daily	0.5-1.0 g 6 hourly by mouth or intravenously by infusion in 4 divided doses
	child aged 2-8 years	250 mg daily	250 mg 6 hourly by mouth infusion in 4 divided doses 12.5 mg/kg/day intravenously by infusion in 4 divided doses
	child under 2 years	125 mg daily	12.5 mg/kg/day by mouth or intravenously by infusion in 4 divided doses
amoxycillin/co-amoxiclav (doses according to amoxycillin content)	adult	250-500 mg daily	0.5-1.0 g 8 hourly by mouth or intravenously
	child aged 5-14 years	125 mg daily	250 mg 8 hourly by mouth 90 mg/kg/day intravenously in three divided doses
	child aged 1-5 years	10 mg/kg/day	125mg 8 hourly by mouth 90 mg/kg/day intravenously in three divided doses
	child under 1 year	10 mg/kg/day	62.5mg 8 hourly by mouth 90 mg/kg/day intravenously in three divided doses
cefotaxime	adult	Not suitable	2 g 8 hourly intravenously
	ahild under 14 years	Not suitable	100 mg/kg/day intravenously in three divided doses (maximum 12 g)
ceftriaxone	adult	Not suitable	1-2 g once daily intravenously
	child under 14 years	Not suitable	80 mg/kg/day intravenously in a single dose (maximum 4 g)
chloramphenicol (only patients allergic to penicillins and cephalosporins)	all patients	Not suitable	Expert advice

The advice for asplenic individuals to be issued with a form of medical alert, such as a card or a bracelet^ref (Mayon-White R. Protection for the asplenic patient. Prescribers Journal 1994;34:165–70), has 2 purposes. First, it should provide a constant reminder to the individual of their underlying condition and, second, knowledge of their asplenic state might be vital for medical attendants in the event of any medical emergency. 3 groups have published results on the foundation of asplenic patient registers at district or regional level^{ref1, ref2, ref3} : an assessment of the patients' own knowledge about their condition^{ref1, ref2} can help in the formulation of educational programmes. Once agreed policies are introduced into a district or region, it is important to evaluate compliance by performing audit^{ref1, ref2}
Key guidelines (A = based on published evidence; B = expert opinion; NB: there are no randomised controlled trials or case controlled studies on this issue) :
• all splenectomised patients and those with functional hyposplenism should receive pneumococcal immunisation (A, B)
• documentation, communication, and reimmunisation require attention (A, B)
• patients not previously immunised should receive Haemophilus influenzae type b vaccine (A, B)
• meningococcal immunisation is not routinely recommended (B)
• influenza immunisation may be beneficial (B)
• lifelong prophylactic antibiotics are recommended (oral phenoxymethylpenicillin or an alternative) (A, B)
• asplenic patients are at risk of severe malaria (A)
• animal and tick bites may be dangerous (A)
• patients should be given a leaflet and a card to alert health professional to their risk of overwhelming infection (A, B)
• patients developing infection despite measures must be given a systemic antibiotic and urgently admitted to hospital (A, B)
Prognosis : mortality = 2.5%, 100% in babies with hematological diseases, so regulated splenectomy is recommended in this group; death rates from OPSI have been reported to be up to 600 times greater than in the general population, with an estimated lifetime risk for OPSI of approximately 5%^ref
• sissorexia : a tendency of the spleen to accumulate blood corpuscles
• splenemphraxis : congestion of the spleen.
• splenemia : congestion of the spleen with blood.
• cyanosis lienis : passive congestion of the spleen
• cyanotic spleen : a contracted form of spleen due to passive congestion.
• splenic infarction

Aetiology :
• cardiac embolism
• extrinsic compression
• acute pancreatitis / chronic pancreatitis
• locoregional neoplasm
• advanced lymphomas and leukemia
Symptoms & signs : aspecific pain in left hypochrondrium or acute abdomen
Therapy :
• medical (recovery within 3-5 months or evolution to pseudocyst or abscess)
• total splenectomy
• splenic artery aneurysm

Epidemiology : more common in females aged 50-80 or at third trimester of pregnancy
Aetiology :
• atherosclerosis (60%)
• congenital hypoplasia (30%)
• post-traumatic or inflammatory (10%)
Localization : main trunk (60-70%), branches (30-40%)
Symptoms & signs : only in hemoperitoneum or in arteriovenous fistula with portal hyperafflux
Laboratory examinations : echocolor Doppler
Therapy : aneurysmectomy + splenectomy + pancreatic tail resection
• spleen rupture

Aetiology :
• traumatic (the commonest : see blunt abdominal trauma (BAT))
• iatrogenous (20%)
• diagnostic interventions : biopsy, laparoscopy, splenic portography
• therapeutic interventions : laparotomic surgery (usually left hemicolectomy and gastrectomy)
• spontaneous (excideengly rare) : in hyperreactive malarious splenomegaly (HMS), sarcoidosis, congestive splenomegaly, acute and chronic leukemia, hemolytic anemia, infectious mononucleosis (at day 10-30 : the commonest cause of death)
Grading  :
• intraparenchymal or subcapsular hematoma
• capsular laceration
• marginal parenchymal rupture (< 1 cm in depth)
• deep parenchymal rupture (> 1 cm) without hilar lesion
• hilar rupture or injury
• fragmentation
Timing :
• acute
• cataclismatic
• in 2 times
Symptoms & sign :
• splenorrhagia : hemorrhage from a ruptured spleen => hemoperitoneum => hypovolemic shock
• pain may be absent in polytrauma patients with neurological damage
• Kehr's sign : severe pain in the left shoulder in some cases due to irritation of homolateral phrenic nerve
• Saegesser's sign : compression of phrenic point (along the border of sternocleidomastoideal muscle, 2-4 cm above the collar-bone) causes sudden contraction of homolateral hemidiaphragm and severe pain along the lateral border of rectus muscle of the abdomen
• acute abdomen in epigastrium and left hypochondrium
• Douglas' pouch occupied and painful at DRE
• Ballance's sign : reduction of gastric resonance over Traube's space of right flank when the patient lies on the left side and, if massive, in flanks; said to be present in splenic rupture
Laboratory examinations :
• anemia, leukocytosis
• abdomen X-rays : attenuation of kidney and ileopsoas shadows, medial dislocation of gastric bulla, lowering of splenic colon flexure, and ileal hydroaereal level due to reflexed functional ileus
• CXR : left pleural effusion, disapperance or discontinuity of left diaphragmatic cupula, fracture of lower left ribs
• echography : high sensitivity in detection of hemoperitoneum, but imaging of spleen is difficult (25-35%) as polytrauma patients cannot maintain deep inspiration
• CT has poor sensitivity (40-80%) in detection of hemoperitoneum but specificity and sensitivity > 90% for imaging of spleen
• diagnostic peritoneal lavage (DPL)
Therapy :
• total splenectomy
• splenorrhaphy in capsular and marginal parenchymal injuries
• regulated splenic resection along avascular borders (usually superior or inferior polar resections, or inferior subtotal resection) : discouragad in hilar injuries
• expectant therapy in people aged < 40 years, no trauma in different areas, and no coagulopathy, no shock, HGB > 8 g/dl. Follow-up for 7-10 days (every 4-6 hours in the first 12 hours, then every 8 hours, every 12 hours since day 2, every 24-36 hours since day 4) and hospitalization for 3-4 weeks
• splenic cysts^ref

Epidemiology : young and middle aged adults, with 60% being women in the fertile age. The reason for this female prevalence is unknown. Hormonal influences causing splenic infarction, and microtrauma to the more vulnerable spleen in pregnant women have been suggested as possible causes.
Aetiology :
• primary : epithelial lining toward the cystic lumen
• parasitic (most often multilocular) : Echinococcus spp. cysts (60-70% in countries endemic for hydatidosis (South America and Mediterranean area), 2-3% of all patients with hydatidosis)
• non-parasitic (most often unilocular)
• congenital
• epidermoid cysts (90% of primary non-parasitic) : mesothelial and with focal squamous metaplasia; foci of transitional epithelium at risk of neoplastic trasformation. They can result from ... :
• embryonic inclusion of epithelial cells from adjacent structures followed by cystic dilatation
• invagination of the capsulat surface mesothelium
• trauma with metaplasa within mesothelial cysts
• dermoid cysts (extremely rare) : cystic teratomas
• endodermoid cysts are not true cysts, but rather a cystic vascular lesion composed of several ectatic vessels. They should be classified as a cystic lymphangioma or a hemangioma (ranging from 5-8 cm to 300-400 g), isolated or disseminated to other organs
• simple cysts
• neoplastic
• secondary : without an epithelial lining
• posttraumatic / splenic pseudocyts (75% of all non-parasitic cysts in countries nonendemic for hydatidosis, following splenic infarction) : organization, liquefation, resorption and encapsulaion of an intraparenchymal or subcapsular hematoma following abdominal trauma (although 30% of patients do not recall any trauma). Secondary cysts might also develop because of splenic infarcts or infections (e.g. mononucleosis, tuberculosis or malaria), which enlarges and makes the spleen more vulnerable. The pseudocyst contains a liquid mixture of blood and necrotic debris. The wall of the posttraumatic cyst does not have an epithelial lining and deposited hemosiderin is often detected microscopically. However, hemorrhage may also occur in the case of primary cysts. Furthermore, the epithelial lining of the primary cyst can be atrophic, which some times make the primary cysts difficult to be distinguished from secondary cysts
Symptoms & signs : painless abdominal mass as well as typical splenomegaly, early satiety, nausea, vomiting, flatus, and weight loss are frequently present. Some patients present with continuous or inconsistent left-sided abdominal or epigastric pain, as well as radiating left shoulder pain, or involving the abdomen like a "tight belt". The pain is due to distension of the capsule, or the mass effect of the cyst, such as dislocation and compresion of the adjavent organs (occupation syndrome : stomach, colon, ureter, diaphragm). If the left kidney is affected, the result might be proteinuria and systemic arterial hypertension. Thrombocytopenia, granulocytopenia and anemia might appear as a result of the relation of the spleen to the blood circulation (hyper/hyposplenism). Cought and dyspnea may occur as well as pleural exudates and empyema due to a transdiaphragmatic and bronchial fistula.
Complications : spleen rupture (severe in hydatid cyst; 25% rate of spontaneous rupture with a mortality rate = 20-25%) => hemoperitoneum, peritonitis, abscess, anaphylactic shock, and empyema; abscessualization, intracystic or endoperitoneal hemorrhage (sometimes dramatic in large hemangiomas). Clinically, it is not possible to distinguish between primary and secondary cysts, although adhesions are reported to be associated with secondary cysts.
Laboratory examinations :
• abdominal echography
• CT/MRI
• doppler ultrasound/angiography
• cystic puncture/blood samples
• biomarkers : raised CA19-9 in epidermoid cysts. However, CA19-9 and CEA are also sensitive tumor markers in differentiating benign pseudocyst from mucinous cystic neoplasm of the pancreas, althought the specificity is low. The CA72-4 tumor marker can be used to detect mucinous pancreatic tumors as it has both a high sensitivity and specificity. This is interesting due to the possible splenic pseudocyst communication of the pancreas.
Differential diagnosis :
• splenomegaly
• cysts, abscess and tumors in the surrounding organs, such as pancreatic pseudocyst extending into the splenic paranchyma. Calcifications of both the primary and secondary cysts are frequently found, which are useful in diagnosing cysts from causes other than splenomegaly.
Therapy :
• < 5 cm :
• non-parasitic : conservative : US follow-up once a year for the following 5 years
• resolved
• progression => surgery
• parasitic : delay surgery to prevent anaphylactic shock
• single or only a few cysts : medical treatment and cyst evacuation is recommended to achieve splenic salvage. Centrimide (cetyltrimethylammonium bromide) 0.1% is the potent chemical agent used to ensure the death of all viable scolices. It is used to fill in the cavity and left in place for 10 minutes, followed by aspiration and opening of the cyst. A known complication to this drug is methemoglobinemia and peritoneal irritation. Methemoglobinemia is easily treated by intravenous methylene blue, and peritoneal irritation is tested by a saline wash of the abdomen after treatment.
• multiple : chemical sterilization followed by laparoscopic complete splenectomy
• > 5 cm : surgery and post-operative US follow up every 3 months. 25% of the splenic parenchyma, if irrigated by splenic vessels, is sufficient to achieve immunologic protection. Partial splenectomy satisfies this goal and has proven to be better in outcome compared with splenic autotransplantation. Incide the capsule of the spleen witrh harmonic scalpel on the ischemic cystic side. The communicating branches between the neraly avascular cyst area and the viable portion of the spleen are divided and controlled
• polar/deep cyst (higher risk of recurrence) : laparoscopic/needlescopic hemisplenectomy
• single/superficial cyst : laparoscopic/needlescopic marsupialization or fenestration (removal of the thinnest part of the cyst wall and attachment of the omentum over the resulting parenchyma defect)
• polycystic unaccessible for fenestration or marsupialization or giant cyst or almost entirely covered by splenic parenchyma (risk of intractable bleeding from the spleen) : vaccination against Neisseria meningitidis, Haemophilus influenzae, and Streptococcus pneumoniae => laparoscopic total splenectomy (or regulated splenic resection ?) with harmonic scalpel
Percutaneous drainage, with or without sclerosing, is followed by a high incidence of recurrence. Furthermore, it may result in adherences to the surrounding organs. A dense inflammatory response around the spleen is another complication, rendering any subsequent operation difficult.
Complications to laparoscopic treatment :
• perforation of hollow organs when establishing the pneumoperitoneum
• bleeding from the major vessel caused by puncture from the Veress needle or laparoscopic/camera trocar
• thermal damage to the intestine from electrocautery resulting in early or late perforation
• bleeding from the spleen
• subcutaneous emphysema
• wound infection
• undetected injury to the colon accompanied by abscess and peritonitis
• thrombosis or embolism
Contraindications for laparoscopic treatment :
• coagulopathy
• infection (abscess)
• demonstrated or suspected neoplastic cyst
• perisplenitis or other disorders with extensive, broad adhesions
• bacon spleen : a spleen with areas of amyloid degeneration, giving its cut surfaces the appearance of fried bacon.
• diffuse waxy spleen : amyloid degeneration of the spleen involving especially the coats of the venous sinuses and the reticulum of the organ.
• flecked spleen of Feitis / speckled spleen : multiple necroses of the spleen, characterized by nonembolic multiple areas of anemic necrosis
• floating, movable or wandering spleen : a spleen congenitally displaced and preternaturally movable

Epidemiology : more common in female sex
Aetiology : agenesis or excessive laxity of phrenocolic ligament
Differential diagnosis : renal ptosis, bowel, mesenterial, uterus or ovarian tumors
Symptoms & signs : intermittent weight sensation or pain
Therapy : splenopexy
Complications : spleen peduncle torsion : strangulation of vessels => sudden abdominal defense and pain, vomiting, shock
Therapy : urgent total splenectomy
• splenelcosis : ulceration of the spleen.
• splenic abscess :

Aetiology :
• primary (rare) : generalized sepsis, immunodepression, primary splenic tuberculosis, hyperreactive malarious splenomegaly (HMS)
• secondary : superinfections of infarcted areas
Therapy : rapid total splenectomy
• splenitis : inflammation of the spleen, a condition usually produced by pyemia. It is attended by enlargement of the organ with pus, and is marked by much local pain.
• splenicterus : inflammation of the spleen associated with jaundice.
• spodogenous splenitis : that due to accumulation of foreign particles in the spleen.
• acute splenic tumour : acute splenitis, enlargement, and softening of the spleen, usually due to bacteraemia or severe bacterial toxaemia
• perisplenitis : inflammation of the peritoneal coat of the spleen and of the structures around it.
• perisplenitis cartilaginea : inflammatory overgrowth of the capsule of the spleen, causing a thickening of cartilaginous hardness.
• splenocele : hernia of the spleen
• lardaceous or waxy spleen : a spleen affected with amyloid degeneration
• porphyry spleen : a spleen which is the seat of nodular infiltration.
• sago spleen : a spleen having on its cut surface the appearance of grains of sago; due to amyloid infiltration
• splenocleisis : irritation of the surface of the spleen to induce the development of new fibrous tissue.
• splenodynia : pain in the spleen.
• splenalgia : neuralgic pain in the spleen.
• splenolysis : destruction of spleen tissue.
• splenoma / splenoncus : general non-specific term for an enlarged spleen
• splenokeratosis / splenceratosis : hardening of the spleen
• splenomalacia : abnormal softness of the spleen; softening of the spleen.
• splenomyelomalacia : softening of the spleen and bone marrow.
• splenosis : the spontaneous transplantation of splenic tissue to unusual sites after open splenic trauma, e.g., after automobile accidents, gunshot or stab wounds. The splenic pulp implants appear as red-blue nodules on the peritoneum, omentum, and mesentery, morphologically similar to multifocal pelvic endometriosis.
• acquired intrathoracic splenosis after diaphragm rupture^ref
• polysplenia : the presence of multiple spleens or splenuli
• lien or splen accessorius / accessory spleen / splenculus / spleneolus / splenulus / splenule / splenunculus : a connected or detached outlying portion, or exclave, of the spleen
• polysplenia syndrome
• splenatrophy : atrophy of the spleen.
• asplenia : absence of the spleen.
• functional asplenia : impaired reticuloendothelial function of the spleen, as in children with sickle-cell anemia
• Polhemus-Schafer-Ivemark syndrome / asplenia syndrome
Laboratory examinations : isolated asplenia or hyposplenia is often diagnosed only after the patient has had a serious, fulminant, and often fatal infection. These conditions are extremely difficult to diagnose in the absence of other indicators. Morphologic anomalies of peripheral blood erythrocytes, such as Howell-Jolly (HJ) bodies, may be the only evidence of the presence of a nonfunctional spleen
• inflammatory pseudotumor (IPT) of the spleen is a reactive lesion, probably of multiple etiologies, characterized by a mixture of inflammatory cells and a minor, disorganized component of spindle cells. The latter include fibroblasts, SMA⁺ myofibroblasts, and CD68⁺ spindled histiocytes, establishing a close similarity with the IPT of the lymph node. This benign process needs to be distinguished from 2 others that have a predominant spindle cell component arranged in parallel bundles: the IPT-like follicular dendritic cell tumor, which is consistently associated with HHV-4 / EBV; and the inflammatory myofibroblastic tumor, also often HHV-4 / EBV-related and similar to those of the soft tissues, lung and other organs. These 2 lesions are neoplastic and therefore have a potentially worse prognosis than IPT^ref
• splenic tumors :
• benign
• hemangiomas
• lymphangiomas
• splenic hamartoma represents an abnormally formed red pulp and is characterized by the presence of sinus-like structures lined by CD8⁺ endothelia. The great variety of its morphologic appearances may result from the preponderant growth of one or another of the several components of the red pulp, ie, CD34⁺ capillaries, myoid cells and macrophages. Therefore, it is proposed that cord capillary hemangioma, myoid angio-endothelioma, and histiocyte-rich tumors are part of the spectrum of splenic hamartoma.
• malignant : angiosarcoma
• metastases (rare due to immunosurveillance) : mammary carcinoma, lung carcinoma, melanoma
Symptoms & signs : only from occupation syndrome (stomach, colon, ureter, diaphragm), hyper/hyposplenism, and apperance of complications such as spleen rupture, abscessualization, intracystic or endoperitoneal hemorrhage
Laboratory examinations : laparoscopic or echo-assisted FNAC or FNAB
Therapy : total splenectomy

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