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La rate occupe un rôle central dans le système de filtration et de défense sanguin. La pulpe rouge permet notamment l'élimination des globules rouges altérés, via son réseau microcirculatoire unique ; tandis que la pulpe blanche est un organe lymphoïde secondaire, directement connecté à la circulation sanguine, dont la spécificité est la défense contre les bactéries encapsulées via la production d'IgM « naturelles » dans la zone marginale. Une altération acquise de la fonction splénique (ou hyposplénisme) peut résulter de diverses maladies et/ou d'une splénectomie thérapeutique. L'hypo/asplénie se complique d'une susceptibilité accrue aux infections à germes encapsulés, mais un excès de risque de thrombose et d'hypertension pulmonaire est également rapporté après une splénectomie chirurgicale. La drépanocytose homozygote est la plus fréquente des maladies associée à une asplénie fonctionnelle. Cette dernière apparaît précocement, dans l'enfance, vraisemblablement du fait d'altérations ischémiques répétées dues à la falciformation des hématies. De plus, des complications spécifiques telles que l'hypersplénisme et les séquestrations spléniques aiguës peuvent survenir et menacer le pronostic vital. Nous proposons ici une mise au point sur le rôle et la physiologie de la rate, ce qui permettra de mieux comprendre la physiopathologie de l'atteinte splénique et ses conséquences au cours de la drépanocytose. The spleen filters blood cells and contributes to the immune defense. The red pulp clears the blood from altered red blood cells via its unique microcirculatory network ; while the white pulp is a secondary lymphoid organ, directly connected to the bloodstream, whose specificity is the defense against encapsulated bacteria through the production of "natural" IgM in the marginal zone. Various health conditions can cause acquired impairment of the splenic function (or hyposplenism) directly and/or through therapeutic splenectomy. Hypo/asplenia is complicated by an increased susceptibility to encapsulated germ infections, but an increased risk of thrombosis and pulmonary hypertension has also been reported after surgical splenectomy. Homozygous sickle cell disease is the most common disease associated with functional asplenia. The latter appears early in childhood likely through repeated ischemic alterations caused by the sickling of red blood cells. In addition, specific complications such as hypersplenism and acute splenic sequestration can occur and may be life-threatening. We provide here an update on the role and physiology of the spleen, which will allow a better understanding of the pathophysiology of spleen damage and its consequences in sickle cell disease. [ABSTRACT FROM AUTHOR]

Immune thrombocytopenia (ITP) is an acquired autoimmune bleeding disorder characterized by antibody mediated platelet destruction and impaired platelet production. Adult patients often present with a chronic disease, displaying a stable platelet count and a low risk for bleeding. However, there is a small category of ITP patients who have instable platelet count, are at high risk for bleeding and are refractory to all forms of treatment. Immune thrombocytopenia is a diagnosis of exclusion; extensive diagnostic work is needed in order to classify a patient as having refractory ITP. Refractory patients do not respond to steroids, i.v. immunoglobulins, thrombopoietin receptor agonists (TPO-RA) or rituximab, or to any other form of treatment. Combination therapy has been evaluated in small cases, with varying results. Newer treatments – fostamatinib, FcRn or Bruton kinase (BTK) inhibitors – appear to be more effective. Exploring the biology of refractoriness is essential for understanding the complex pathology of this disease and for finding ways to stop its natural evolution. In summary, refractory immune thrombocytopenia is a complex condition that requires precise diagnosis and the initiation of an effective combination treatment from onset. [ABSTRACT FROM AUTHOR]

Immune thrombocytopenia is an autoimmune disorder that involves the platelet destruction in the spleen. Often, patients do not respond to conventional therapy. The management of refractory or relapsed immune thrombocytopenia poses a challenge for hematologist. We report the case of a 43-year-old patient in whom conventional treatment was not successful but who responded to TPO agonist eltrombopag [ABSTRACT FROM AUTHOR]

Résumé: L'hypertension portale segmentaire (HTPS) est une entité rare, compliquant principalement des maladies pancréatiques, bénignes ou malignes. Les patients sont le plus souvent asymptomatiques. La survenue de douleurs abdominales chroniques pouvant résulter de la splénomégalie et/ou de la maladie sous-jacente responsable de l'HTPS ont été décrites. Cependant, la complication majeure, et parfois fatale, de l'HTPS est l'hémorragie digestive sur rupture de varices œsophagiennes ou plus fréquemment de varices gastriques. Plusieurs modalités thérapeutiques peuvent être proposées, mais le traitement de référence reste la splénectomie. Plus récemment, des techniques de radiologie interventionnelle avec notamment des embolisations de l'artère splénique ± couplées à une embolisation splénique permettent de réaliser une « splénectomie non chirurgicale » et moins invasive. Enfin, l'endoscopie joue un rôle important dans la prise en charge de ces malades, car elle contribue d'une part au diagnostic de l'HTPS permet dans le même temps une approche thérapeutique avec également la possibilité de réaliser une hémostase endoscopique de varices par ligature élastique et/ou encollage. Left-sided portal hypertension (LSPH) is a rare entity caused by various entities, pancreatic diseases (mainly chronic pancreatitis) being the most common cause. LSPH is asymptomatic in most of the cases, but non-specific chronic abdominal pain have also been described. In some cases, LSPH may present with gastro-intestinal bleeding from ruptured oesophageal or gastric varices, which could be life-threatening. Different treatment options exist, but surgical splenectomy remains the treatment of choice as it cures the LSPH, by removing the blood outflow. Recently, interventional radiology procedures have been developed, such as the splenic artery embolization. Splenic artery embolization can be associated with a partially splenic embolization to potentialized the efficacity of the procedure, enabling a "non-surgical splenectomy", while maintaining normal immune function of spleen. Endoscopy also plays a crucial part in the therapeutic management of LSPH patients, by allowing the diagnosis of the causal lesion and its treatment (e.g., elastic band ligation for the oesophageal varices or tissues adhesive injection for gastric varices) at the same time. It is important to notice that LSPH is a curable cause of portal hypertension. [ABSTRACT FROM AUTHOR]

L'hyperkaliémie nécessite une prise en charge rapide, notamment symptomatique à visée cardio-protectrice. À côté des causes facilement évoquées telles l'insuffisance rénale, l'insuffisance surrénalienne, la lyse cellulaire ou les causes iatrogènes, celle d'une fausse ou pseudo-hyperkaliémie l'est moins et ne doit pas être oubliée. Trois patients (1 homme, 2 femmes, âgés de 78, 84, 88 ans) pris en charge pour thrombocytose (entre 1306 et 2404 G/L) et hyperkaliémie non symptomatique (entre 6,1 et 7,7 mmol/L) sont rapportés. La kaliémie de contrôle sur tube hépariné était normale (entre 4,4 et 4,6 mmol/L). Il n'a donc pas été nécessaire d'instaurer de traitement spécifique à cette pseudo-hyperkaliémie. L'association thrombocytose et hyperkaliémie non symptomatique doit faire évoquer le diagnostic de pseudo-hyperkaliémie et faire impérativement réaliser un contrôle de la kaliémie sur tube hépariné. La reconnaissance de ce diagnostic est importante afin de ne pas instaurer un traitement non nécessaire et potentiellement délétère. Hyperkalemia is common in medicine and requires rapid management. Besides the easily evoked causes such as renal failure, adrenal insufficiency, cell lysis or iatrogenic causes, false or pseudo-hyperkalemia should not be forgotten. Three patients (1 man, 2 women, aged 78, 84, 88) were managed for thrombocytosis (between 1306 and 2404 G/L) and non-symptomatic hyperkalemia (between 6.1 and 7.7 mmol/L) are reported. Kalemia on blood collected in heparin tube was normal (4.4–4.6 mmol/L). Therefore, no specific treatment for this pseudohyperkalemia was required. The combination of thrombocytosis and non-symptomatic hyperkalemia should suggest the diagnosis of pseudohyperkalemia and should prompt for a control of kalemia on blood collected in heparin tube. The recognition of this diagnosis is important in order to avoid unnecessary and potentially deleterious treatment of hyperkalemia. [ABSTRACT FROM AUTHOR]

Introduction: Splenectomy is a surgical procedure that involves the removal of the spleen. Ils indications are diverse. The aim of this study conducted at the Surgery Department of Libreville University Hospital in Gabon, was to determine the indications of splenectomies and to evaluate the outcome. This was an observational retrospective descriptive study covering a period of 14 months, from October 2016 to January 2018. All records of patients undergoing splenectomy in isolation or associated with other surgical procedures were included. The parameters studied were: age, sex, circumstances of discovery and diagnosis, indication of splenectomy, type of prophylactic anti-infectious vaccines, type of incision and surgical procedure, histological diagnosis, postoperative morbidity and mortality. Fourteen cases were identified, including 9 men and 5 women. The mean age was 20.8 ± 17.7 years [3-58 years]. Eight patients were aged less than 16 years. Sickle cell disease was found in 7 patients. Antibiotic prophylaxis was continued for 1 month or more in 11 patients. The main indications were splenomegaly with hypersplenism and traumatic hemoperitoneum by splenic rupture. No immediate postoperative complication was observed. One death was recorded. In our context, total splenectomy is the rule. Immediate postoperative morbidity and mortality is low. [ABSTRACT FROM AUTHOR]

Résumé: La cirrhose n'est plus une contre-indication formelle à la chirurgie abdominale extra-hépatique mais celle-ci reste grevée d'une très forte morbidité (> 50 %), ainsi qu'une mortalité comprise entre 10 % et 50 %. Les scores de Child, et plus récemment de MELD, permettent d'anticiper le risque de décompensation post-opératoire. Le risque de complications est acceptable en cas de Child-A (pas d'ascite), important en cas de score MELD > 15, et majeur pour un score MELD > 25. L'indication chirurgicale repose plus que jamais sur la balance bénéfices/risques de l'intervention (le contexte d'urgence notamment) et requiert une information complète auprès du patient et/ou de son entourage. Les chirurgies biliaires et pariétales représentent les indications les plus fréquentes dans ce contexte d'hypertension portale (notamment par voie laparoscopique), suivies ensuite des colectomies pour cancer. Il n'y a pas de modification notable des indications de voie d'abord ni de drainage chez le cirrhotique. Les chirurgies pancréatiques, gastriques et œsophagiennes doivent rester en revanche exceptionnelles sur ce terrain et nécessitent une prise en charge multidisciplinaire avec optimisation pré-opératoire. Si une prise en charge médicale (nutritionnelle, hépatologique, cardiologique, kinésithérapeutique) optimale peut être recommandée, les autres mesures médicales (pharmacologiques), chirurgicales ou radiologique (TIPS) pour diminuer le degré d'hypertension portale n'ont pas été validées hors chirurgie hépatique. Cirrhosis is no longer a formal contraindication to extrahepatic abdominal surgery, but is still burdened with very high morbidity (>50%) and mortality between 10% and 50%. The Child, and more recently MELD, scores predict the risk of postoperative decompensation. The risk of complications is acceptable in case of Child A (no ascites), important in case of MELD score >15, and major for a MELD score >25. The surgical indication is based on the benefit-risk balance of the intervention (emergency context), and requires complete information from the patient and/or his entourage. Biliary and parietal surgeries are the most frequent indications in this context of portal hypertension (especially laparoscopic approach), followed by colectomies for cancer. There is no significant change in surgical approaches or drainage indications in cirrhotic patients. On the other hand, pancreatic, gastric and esophageal surgeries must remain exceptional and require multidisciplinary management with preoperative optimization. If an optimal medical (nutritional, hepatological, cardiological, physiotherapist) treatment can currently be recommended, other medical, surgical or radiological (TIPS) measures to decrease the degree of portal hypertension have not been validated except for liver surgery. [ABSTRACT FROM AUTHOR]

Immune thrombocytopenia (ITP) is an acquired haemorrhagic disease characterized by the presence of isolated thrombocytopenia (platelet count in peripheral blood <100,000 / mm3). Corticotherapy is recommended as the first-line ITP treatment, but splenectomy remains, although it has the greatest chances to cure the disease, a second-line option in ITP therapeutic management. Classic surgical spleen removal can be associated with several complications: infection, cardiovascular events and cancer. The modern surgical approach consists of laparoscopic splenectomy, being preferred to the classical method due to reduced complications, faster recovery and is both a palliative and a curative method. Palliative irradiation therapy of spleen is another option to annihilate this organ in ITP. [ABSTRACT FROM AUTHOR]

Trombocitoza reprezintă creşterea numărului de trombocite peste 500.000/mm³. Obiectiv. Obiectivul acestui studiu este de a evalua frecvenţa şi severitatea trombocitozei reactive la pacienţii pediatrici splenectomizaţi. Material şi metodă. Am realizat un studiu retrospectiv în care au fost incluşi 20 de pacienţi (4-16 ani) care au suferit o intervenţie de splenectomie în intervalul 2006-2015. Criteriile de includere în studiu au fost: pacienţi cu vârsta sub 18 ani, care au fost splenectomizaţi şi care au dezvoltat trombocitoză ulterior intervenţiei. Rezultate. În lotul studiat, 80% dintre pacienţii splenectomizaţi (16) au dezvoltat o formă de trombocitoză; în 4 cazuri formă severă (trombocite > 1milion/mm3), iar în 7 cazuri formă uşoară. Nu s-au observat diferenţe semnificative în repartiţia pe sexe a pacienţilor (9 de sex feminin şi 11 de sex masculin). La 13 dintre pacienţi trombocitoza s-a remis după 30 de zile şi doar în 3 dintre cazuri episodul a durat >360 de zile. Fenomene trombotice s-au observat la un singur pacient din lotul studiat. Toţi pacienţii au beneficiat de măsuri de tromboprofilaxie şi hidratare, iar 2 cazuri au necesitat asociere terapeutică de Hidoxiuree. Concluzii. Studiul nostru relevă o frecvenţă crescută a trombocitozei după splenectomie (80%), cu un vârf maxim de incidenţă la 2-10 zile postchirurgical, trombocitoza fiind în general benignă şi cu remisie spontană; evenimente trombotice s-au semnalat într-un singur caz, iar trombocitoza severă a fost mai frecventă după splenectomia de cauză traumatică.

Résumé Un patient âgé de 62 ans a subi une splénectomie en urgence suite à un accident de la voie publique. Porteur d’une prothèse valvulaire mécanique depuis trois ans, il suit un traitement anticoagulant chronique. Summary A 62-year-old patient has undergone an emergency splenectomy following a road traffic accident. Fitted with a mechanical prosthetic valve three years ago, he has been receiving chronic anticoagulant therapy. [ABSTRACT FROM AUTHOR]

Hereditary spherocytosis (HS) is the most frequent cause of constitutional hemolytic anemia among Caucasians. It is due to a quantitative or qualitative defect of some of the proteins composing the skeleton of the red blood cell membrane. Destabilization of this membrane causes spherisation of the red blood cells and hemolysis, occurring mainly in the spleen. Splenectomy is consequently very efficient in this disease. Recent guidelines aimed at defining the place of splenectomy in HS: it is always indicated in severe cases, preferentially after the age of 6 years old; it should be discussed in mild presentations, and is never indicated in asymptomatic cases. Subtotal splenectomy may be an interesting option in children under 6 years old with severe presentation, but its place is not well defined yet. However, splenectomy is associated with long-term complications. Infectious complications are well known, and can be prevented with vaccination, education of the patient and his family, and with the systematic use of prophylactic antibiotics after splenectomy. It is noteworthy that the risk of overwhelming post-splenectomy infection persists all life long in splenectomized patients. There is also an increased thrombo embolic risk after splenectomy, including early splenic and portal thrombosis (often asymptomatic), and delayed arterial or venous events. A slightly increased risk of cancer has also been described in splenectomized patients, but this is still controversial, and has not been studied specifically in HS patients. All these complications should be taken into account when a splenectomy is discussed, but keeping in mind its clear benefits in symptomatic forms of HS. [ABSTRACT FROM AUTHOR]

L'hypertension portale non cirrhotique est une affection décrite pour la première fois par Guido BANTI en 1898 comme une affection associant une hypertension portale avec splénomégalie et anémie sur foie sain. Le diagnostic repose sur l'échographie abdominale, la splénoportographie et la biopsie hépatique. Le but de notre travail est d'évaluer la place de la splénectomie dans l'hypertension portale non cirrhotique à travers une étude rétrospective portant sur 3 malades dont 2 femmes et un homme pris en charge dans notre formation entre Janvier 2010 et Septembre 2016. Le diagnostic de l'hypertension portale idiopathique a été basé sur les critères suivants: une hypertension portale, la présence des varices ésophagiennes avec une splénomégalie, l'absence de cirrhose ou d'autres affections hépatiques responsables de l'hypertension portale. La splénectomie a été réalisée chez les 3 malades. L'évolution après la splénectomie était marquée par la normalisation des signes cliniques, radiologiques et biologiques de cette affection, avec absence de récidive des varices ésophagiennes. La splénectomie associée à la ligature des varices ésophagiennes pourraient être suffisantes pour traiter ce syndrome et surtout ses conséquences sans avoir recours à une dérivation spléno-rénale.

Non-cirrhotic portal hypertension was first described by Guido BANTI in 1898 as a condition characterized by the association of portal hypertension with splenomegaly, anemia and healthy liver. The diagnosis was based on abdominal ultrasound, splenoportography and liver biopsy. Our study aimed to evaluate the role of splenectomy in non-cirrhotic portal hypertension. We conducted a retrospective study of 3 patients (2 women and 1 man) treated by our staff over the period January 2010-September 2016. The diagnosis of idiopathic portal hypertension was based on the following criteria: portal hypertension, the presence of oesophageal varices associated with splenomegaly, the absence of cirrhosis or of other liver disorders responsible of portal hypertension. All patients underwent splenectomy. Outcome after splenectomy was marked by the standardization of clinical, radiological and biological signs of this disease associated with the absence of oesophageal varices recurrence. Splenectomy associated with ligation of oesophageal varices may be sufficient to treat this syndrome and especially its consequences without using splenorenal bypass. [ABSTRACT FROM AUTHOR]

Résumé Introduction L’incidence des infections après splénectomie est mal établie sur le long terme. L’objectif de cette étude est de décrire les complications infectieuses et les facteurs associés à la survenue d’une complication infectieuse chez les patients ayant bénéficié d’une splénectomie depuis plus de dix ans. Méthodes Étude rétrospective monocentrique de patients adultes splénectomisés entre le 1 er janvier 1997 et le 31 décembre 2004 dans un centre hospitalier universitaire. Les données sur l’âge, le sexe, l’indication de la splénectomie, les événements infectieux, la mortalité, le statut vaccinal et l’antibioprophylaxie ont été collectées en janvier 2015. Résultats Cent soixante-cinq patients ont été inclus. Les hémopathies malignes représentent l’indication principale de la splénectomie (37,5 %). Quatre-vingt-dix-sept pour cent des patients ont bénéficié d’une vaccination contre le pneumocoque. Soixante-dix-huit pour cent ont reçu une antibioprophylaxie dont les modalités sont hétérogènes. Trente-sept patients ont présenté 42 complications infectieuses survenant en médiane 4 ans après la splénectomie (2 jours à 12 ans). Cinquante-sept pour cent des infections sont survenues au-delà de la 2 e année, et 14,3 % au-delà de la 10 e année. Il s’agit majoritairement d’infections pulmonaires et/ou microbiologiquement documentées à pneumocoque. La survenue d’une complication infectieuse est liée significativement à l’âge (HR = 6,2 ; IC95 % : 1,4–27,1 ; après 65 et à la cause de la splénectomie [ p = 0,002]). Les différentes modalités de prescription de l’antibioprophylaxie ne sont pas significativement associées à la survenue d’infection. Conclusion Après splénectomie, le risque infectieux persiste au long cours avec d’authentiques infections à germes encapsulés survenant parfois après plus de 10 ans. La survenue d’une complication infectieuse est liée à l’âge et à la cause de la splénectomie. Introduction Although most infections occur within the first 2 years after splenectomy, the relatively short follow-up reported in many studies may underestimate the frequency of infections. The objective of the study was to determine the incidence of infective outcomes and factors associated with infection after splenectomy by studying a group of patients who underwent splenectomy over a 10-year period. Methods A retrospective and monocentric study of patients who underwent splenectomy between January 1st, 1997 and December 31st, 2004 in a French university hospital. Age, sex, indication for splenectomy, infectious events, death, vaccination and antibiotic prophylaxis were collected in January 2015. Results One hundred and sixty-five patients were included. The most common reasons for splenectomy were therapeutic hematological indications (37.5%). Ninety-seven per cent received pneumococcal vaccine. Prophylactic antibiotics were prescribed in 78% of patients. Thirty-seven patients had 42 severe infections with a median incidence rate of 4 years after splenectomy (2 days–12 years). The rate of infection after splenectomy declined over time but 57% occurred after 2 years and 14.3% after 10 years. Respiratory infections were the most common sites of infections. The incidence of infection differed according to age was highest among the elderly (HR = 6.2; 95%CI: 1.4–27.1; after 65 years old) and underlying reason for splenectomy ( P = 0.02). There is no difference with or without prophylactic antibiotics. Conclusion After splenectomy, the incidence of severe infection declined over time but can occur after 10 years. The onset of infection is linked to age and reason for splenectomy. [ABSTRACT FROM AUTHOR]

Résumé Introduction La thrombose veineuse portale et/ou splénique (TVSP) survient dans 5–55 % des splénectomies. Elle comporte un risque d’ischémie mésentérique veineuse et d’hypertension portale. Dans la mesure où le traitement médical est efficace, un diagnostic précoce semble important afin d’éviter des complications parfois irréversibles. Il n’y a pas de consensus concernant l’intérêt et les modalités d’un dépistage éventuel et la prise en charge de la TVSP. Nous avons mis en place un protocole systématique de dépistage des TVSP après splénectomie élective depuis janvier 2012. Le but de ce travail est d’évaluer les résultats de ce dépistage systématique. Patients et méthodes Depuis janvier 2012, tous les patients ayant eu une splénectomie programmée ont eu un dépistage de TVSP par imagerie à j7. Les données démographiques, les indications opératoires, le type d’intervention, les chiffres de plaquettes pré- et postopératoires, les complications, les résultats de l’imagerie et les modalités de prise en charge des TVSP ainsi que le résultat du traitement ont été colligés. Résultats Sur une période de 3 ans, 52 patients ont eu une splénectomie totale élective. Tous ont eu un scanner injecté au 7 e jour postopératoire (j7). Onze TVSP (21,2 %) ont été diagnostiquées. Aucun patient n’était symptomatique au moment du diagnostic. En analyse univariée, les facteurs associés à la survenue d’une TVSP étaient un diagnostic de lymphome et la splénomégalie. Un traitement anticoagulant curatif était débuté dès le diagnostic. Aucune TVSP ne s’est compliquée d’ischémie mésentérique ou d’hypertension portale. Le traitement s’est avéré efficace chez tous les patients avec disparition du thrombus à l’imagerie de contrôle. Conclusion Le dépistage systématique de la TVSP après splénectomie permet de débuter rapidement un traitement efficace et d’éviter ses potentielles complications. Le risque de TVSP est plus élevé en cas de splénectomie pour lymphome ou de splénomégalie. Background Portal and/or splenic vein thrombosis (PSVT) is common after splenectomy. It can be a life-threatening complication, with a risk of bowel ischemia and portal hypertension. An early diagnosis allows an effective medical treatment and prevents life-threatening complications. There is no consensus regarding the benefit of systematic screening of patients after splenectomy for PSVT. We started in January 2012 a routine screening of PSVT after elective splenectomy. The aim of this study was to assess this policy. Methods Since January 2012, all patients undergoing an elective splenectomy had an abdominal CT-scan on postoperative-day 7. Demographic data, pathology, type of surgery, platelet counts before and after surgery, outcome, results of medical imaging, and management of PSVT and its results were recorded. Results Over 3 years, 52 patients underwent an elective splenectomy. All of them had a CT-scan at postoperative-day 7. A PSVT was found in 11 patients (21.2 %). They were all asymptomatic. Lymphoma and splenomegaly were the main factors associated with PSVT in the univariate analysis. All patients with PSVT were treated with anticoagulation and no complication of PSVT occurred. The follow-up CT confirmed the efficacy of anticoagulation therapy in all patients. Conclusions Routine screening of PSVT after elective splenectomy is warranted because it allows to start anticoagulant therapy and avoid further life-threatening complications. The incidence of PSVT is particularly high among patients operated on for lymphoma or with splenomegaly. [ABSTRACT FROM AUTHOR]

The spleen filters blood cells and contributes to the immune defense. The red pulp clears the blood from altered red blood cells via its unique microcirculatory network ; while the white pulp is a secondary lymphoid organ, directly connected to the bloodstream, whose specificity is the defense against encapsulated bacteria through the production of "natural" IgM in the marginal zone. Various health conditions can cause acquired impairment of the splenic function (or hyposplenism) directly and/or through therapeutic splenectomy. Hypo/asplenia is complicated by an increased susceptibility to encapsulated germ infections, but an increased risk of thrombosis and pulmonary hypertension has also been reported after surgical splenectomy. Homozygous sickle cell disease is the most common disease associated with functional asplenia. The latter appears early in childhood likely through repeated ischemic alterations caused by the sickling of red blood cells. In addition, specific complications such as hypersplenism and acute splenic sequestration can occur and may be life-threatening. We provide here an update on the role and physiology of the spleen, which will allow a better understanding of the pathophysiology of spleen damage and its consequences in sickle cell disease., (Copyright © 2023. Published by Elsevier Masson SAS.)

La rate baladeuse ou errante est une anomalie rare, qui est le plus souvent décrite chez l'enfant. Ses complications parmi lesquelles figure la torsion de son pédicule sont fréquentes et peuvent engager le pronostic vital du patient. Nous rapportons un cas de torsion chronique du pédicule d'une rate baladeuse chez un patient de 17 ans, au long passé de douleurs épigastriques. Le tableau clinique était marqué par une masse épigastrique spontanément douloureuse, évoluant depuis 48 heures. L'échographie abdominale objectivait une rate ectopique hétérogène, hypertrophiée, en position épigastrique et un hématome sous capsulaire. Au doppler, on notait une torsion du pédicule splénique à deux tours de spires et un petit flux sur l'artère splénique. La tomodensitométrie abdominale avec injection de produit de contraste montrait un défaut de rehaussement parenchymateux d'une grosse rate ectopique épigastrique et un hématome sous capsulaire. Le diagnostic de torsion chronique du pédicule d'une rate baladeuse, compliquée de nécrose et d'hématome sous capsulaire était retenu. Il a été réalisé une splénectomie. Les suites opératoires étaient simples. Nous discutons l'apport de l'échographie et de la TDM dans le diagnostic de la torsion chronique du pédicule d'une rate baladeuse.

La coloscopie à visée diagnostique et/ou thérapeutique est un examen invasif fréquemment pratiquée de nos jours. La perforation colique et l'hémorragie digestive en sont les principales complications. La survenue d'un hémopéritoine par rupture d'un hématome sous-capsulaire splénique est une complication extrêmement rare et potentiellement mortelle de la coloscopie. Un traumatisme splénique minime passé inaperçu et la prise d'anticoagulant en sont des facteurs favorisants. Nous présentons le cas d'une rupture d'un hématome sous-capsulaire de la rate après une coloscopie, survenue chez un patient de 70 ans porteur d'une valve mitrale mécanique sous acénocoumarol à dose hypocaogulante. La nécessité d'obtention d'une anti coagulation rapidement efficace et l'instabilité hémodynamique avaient justifiée la réalisation d'une splénectomie. L'évolution était favorable. A travers cette observation clinique nous discutons les mécanismes et les modalités de prise en charge devant cette complication.

Wandering spleen is an unusual entity. Spleen infarction which results from the torsion of the pedicle is his main complication. We report a case of the torsion of wandering spleen diagnosed by computed tomography. We have done a splenectomy because of splenic infarction. Nowadays, splenopexy is the best treatment if the diagnosis is done early. [ABSTRACT FROM AUTHOR]

Marginal zone malignant lymphoma is a non-Hodgkin lymphoma, with B-cell proliferation, with indolent evolution, representing about 5-17% of all non-Hodgkin malignancies in adult patients. Etiologically, there is a constant association with chronic infections, but also with inflammation; in this case, chronic immune antigenic stimulation results in a lymphoid increase, from which the further oncological events occur, involving chromosomal abnormalities that activate intracellular signaling pathways, leading to antigen independent lymphoproliferation. The MALT subtype is the most common form of marginal zone non-Hodgkin lymphoma; in 50-70% of cases there is also an association with autoimmune disorders such as Hashimoto's thyroiditis, Wegener's granulomatosis, Sjögren's syndrome, and systemic lupus erythematosus. For diagnostic certainty of this type of lymphocytes, is required histopathological examination of spleen after surgical splenectomy or morphological/immunophenotype examination of circulating lymphocytes if there splenic lymphoma with villous lymphocytes. [ABSTRACT FROM AUTHOR]

Wandering or migrating spleen is a rare anomaly which is usually described in children. Complications, which include pedicle torsion, are common and can be life-threatening. We report the case of a 17 year-old patient with a long past medical history of epigastric pain suffering from wandering spleen with chronic torsion of the pedicle. The clinical picture was marked by spontaneously painful epigastric mass, evolved over the past 48 hours. Abdominal ultrasound objectified heterogeneous hypertrophied ectopic spleen in epigastric position and a subcapsular hematoma. Doppler showed a torsion of splenic pedicle which was untwisted 2 turns and a small blood stream on the splenic artery. Abdominal CT scan with contrast injection showed a lack of parenchymal enhancement of large epigastric ectopic spleen and a subcapsular hematoma. The diagnosis of wandering spleen with chronic torsion of the pedicle complicated by necrosis and subcapsular hematoma was confirmed. The patient underwent splenectomy. The postoperative course was uneventful. We here discuss the contribution of ultrasound and CT scan in the diagnosis of wandering spleen with chronic torsion of the pedicle. [ABSTRACT FROM AUTHOR]

Résumé Introduction Le déficit en sphingomyélinase acide conduit à une maladie sévère de l’enfant (maladie de Niemann-Pick A) ou à une forme plus atténuée observée à l’âge adulte (Niemann-Pick B) associant une fibrose pulmonaire et une splénomégalie. Observation Un homme de 52 ans atteint de la maladie de Niemann-Pick B se présentait avec une rupture splénique. Une embolisation de l’artère splénique était initialement réalisée. À 3 mois, le volume splénique avait augmenté et il existait une asplénie fonctionnelle. La scintigraphie splénique montrait cependant 20 % de parenchyme fonctionnel. Une splénectomie était finalement décidée en raison d’une nécrose complète de la rate. Conclusion Malgré sa contre-indication théorique dans la maladie de Niemann-Pick en raison du risque d’aggravation respiratoire, la splénectomie doit parfois être réalisée. Introduction Acid sphingomyelinase deficiency leads to a severe infantile disease (Niemann-Pick disease type A) or an attenuated form of the disease encountered in adults (Niemann-Pick type B), including pulmonary fibrosis and splenomegaly. Case report A 52-year-old man with Niemann-Pick disease type B was admitted with splenic rupture. Embolization of the splenic artery was initially performed. Three months later, the splenic volume had increased and functional asplenia was diagnosed. Splenic scintigraphy showed 20% of functional splenic tissue. Splenectomy was finally performed because of complete necrosis of the spleen. Conclusion Despite its theoretical contra-indication in Niemann-Pick disease due to a risk of respiratory insufficiency, splenectomy must sometimes be considered. [ABSTRACT FROM AUTHOR]

La coloscopie à visée diagnostique et/ou thérapeutique est un examen invasif fréquemment pratiquée de nos jours. La perforation colique et l'hémorragie digestive en sont les principales complications. La survenue d'un hémopéritoine par rupture d'un hématome sous-capsulaire splénique est une complication extrêmement rare et potentiellement mortelle de la coloscopie. Un traumatisme splénique minime passé inaperçu et la prise d'anticoagulant en sont des facteurs favorisants. Nous présentons le cas d'une rupture d'un hématome sous-capsulaire de la rate après une coloscopie, survenue chez un patient de 70 ans porteur d'une valve mitrale mécanique sous acénocoumarol à dose hypocaogulante. La nécessité d'obtention d'une anti coagulation rapidement efficace et l'instabilité hémodynamique avaient justifiée la réalisation d'une splénectomie. L'évolution était favorable. A travers cette observation clinique nous discutons les mécanismes et les modalités de prise en charge devant cette complication. [ABSTRACT FROM AUTHOR]

Splenectomy is a therapeutic procedure for a wide range of conditions, mainly hematological disorders. From the hematological disorders we can mention the red cell disorders: haemolytic anemia, hereditary spherocytosis and thalassemia, that have indication for splenectomy in case of failure to the drug treatment. A wide variety of thrombocytopenic disorders are improved by splenectomy, and the most common indicatios is for Idiopatic Thrombocitopenic Purpura (ITP). However, splenectomy imparts a high risk of fulminant infections that can be avoided with proper prophylaxis. Although in ITP splenectomy in not curative, it does not remove the autoimmune component, it will lead to relief of symptoms, improved platelet count with the cessation of the immunosuppressant treatment and better quality of life. [ABSTRACT FROM AUTHOR]

For hematologic disorders, splenectomy has a wide range of applications: diagnosis, palliative or curative. The most frequent indication is for red cell disorders: hereditary spherocytosis, autoimmune hemolytic anemia or thalassemia. For the platelet disorders, the greatest benefit is achieved in idiopathic thrombocytopenic purpura. In case of limfoproliferative disorders (leukemia, lymphoma), splenectomy plays a role in diagnosis, palliative care (lowering of tumor burden) and in some cases as a cure. In patients with hematologic disorders, splenectomy is associated with an elevated risk of infections that can be avoided with proper antibiotic prophylaxis and vaccination. Due to the expanding list of applications for splenectomy in hematologic disorder, it is necessary to establish a work protocol that covers all aspects and particularities of these disorders. [ABSTRACT FROM AUTHOR]

Résumé Dans les thalassémies dites intermédiaires car l’anémie est moins sévère que dans les formes majeures, les transfusions ne sont pas en règle indiquées ou de manière occasionnelle. Les thalassémies non dépendantes des transfusions comportent trois formes cliniques principales : les bêta-thalassémies intermédiaires, les hétérozygoties composites HbE/bêta-thalassémie qui leur sont proches et les hémoglobinoses H ou alpha-thalassémies avec hémoglobine H. Avec l’âge, l’anémie secondaire à la dysérythropoïèse et à l’hémolyse périphérique s’aggrave et se complique. Surviennent également les conséquences de la splénectomie fréquemment pratiquée dans les thalassémies intermédiaires (complications thromboemboliques et hypertension artérielle pulmonaire notamment). La fréquence des complications observées à l’âge adulte conduit actuellement à limiter les indications de splénectomie et à discuter plus précocement les traitements transfusionnels et chélateurs. Par ailleurs, la meilleure connaissance des mécanismes de la dysérythropoïèse thalassémique et de la surcharge en fer qu’elle induit par hyperabsorption digestive du fer a ouvert la voie à de nouvelles perspectives thérapeutiques. Thalassemia intermedia is a clinical entity where anemia is mild or moderate, requiring no or occasional transfusion. Non-transfusion-dependent thalassemia encompasses 3 main clinical forms: beta-thalassemia intermedia, hemoglobin E/beta-thalassemia and alpha-thalassemia intermedia (HbH disease). Clinical severity of thalassemia intermedia increases with age, with more severe anemia and more frequent complications such as extramedullary hematopoiesis and iron overload mainly related to increased intestinal absorption. Numerous adverse events including pulmonary hypertension and hypercoagulability have been associated with splenectomy, often performed in thalassemia intermedia patients. The potential preventive benefit of transfusion and chelation therapies on the occurrence of numerous complications supports the strategy of an earlier therapeutic intervention. Increasing knowledge about pathophysiological mechanisms involved in thalassemia erythropoiesis and related iron overload is currently translating in novel therapeutic approaches. [ABSTRACT FROM AUTHOR]

Introduction: Spleen is the largest lymphoid organ in the body. Splenomegaly is the increase in spleen volume, in varying degrees of clinical detection by palpation of the abdomen or by various imaging investigations. Aim of the paper: The present paper aims to review the etiology of splenomegaly in children (hematological, oncological, digestive, infectious, metabolic diseases, etc.) and to point out the steps necessary to evaluate the pediatric patient with splenomegaly, starting from anamnesis, clinical examination, laboratory and imaging investigations, as well as the therapeutic options. Conclusion: Therapeutic management in the child with splenomegaly includes the treatment of the underlying disease. Splenectomy is required in symptomatic patients or in the presence of complications caused by enlarged spleen. [ABSTRACT FROM AUTHOR]

Introduction: Although most infections occur within the first 2 years after splenectomy, the relatively short follow-up reported in many studies may underestimate the frequency of infections. The objective of the study was to determine the incidence of infective outcomes and factors associated with infection after splenectomy by studying a group of patients who underwent splenectomy over a 10-year period., Methods: A retrospective and monocentric study of patients who underwent splenectomy between January 1st, 1997 and December 31st, 2004 in a French university hospital. Age, sex, indication for splenectomy, infectious events, death, vaccination and antibiotic prophylaxis were collected in January 2015., Results: One hundred and sixty-five patients were included. The most common reasons for splenectomy were therapeutic hematological indications (37.5%). Ninety-seven per cent received pneumococcal vaccine. Prophylactic antibiotics were prescribed in 78% of patients. Thirty-seven patients had 42 severe infections with a median incidence rate of 4 years after splenectomy (2 days-12 years). The rate of infection after splenectomy declined over time but 57% occurred after 2 years and 14.3% after 10 years. Respiratory infections were the most common sites of infections. The incidence of infection differed according to age was highest among the elderly (HR=6.2; 95%CI: 1.4-27.1; after 65 years old) and underlying reason for splenectomy (P=0.02). There is no difference with or without prophylactic antibiotics., Conclusion: After splenectomy, the incidence of severe infection declined over time but can occur after 10 years. The onset of infection is linked to age and reason for splenectomy., (Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Background: Portal and/or splenic vein thrombosis (PSVT) is common after splenectomy. It can be a life-threatening complication, with a risk of bowel ischemia and portal hypertension. An early diagnosis allows an effective medical treatment and prevents life-threatening complications. There is no consensus regarding the benefit of systematic screening of patients after splenectomy for PSVT. We started in January 2012 a routine screening of PSVT after elective splenectomy. The aim of this study was to assess this policy., Methods: Since January 2012, all patients undergoing an elective splenectomy had an abdominal CT-scan on postoperative-day 7. Demographic data, pathology, type of surgery, platelet counts before and after surgery, outcome, results of medical imaging, and management of PSVT and its results were recorded., Results: Over 3 years, 52 patients underwent an elective splenectomy. All of them had a CT-scan at postoperative-day 7. A PSVT was found in 11 patients (21.2 %). They were all asymptomatic. Lymphoma and splenomegaly were the main factors associated with PSVT in the univariate analysis. All patients with PSVT were treated with anticoagulation and no complication of PSVT occurred. The follow-up CT confirmed the efficacy of anticoagulation therapy in all patients., Conclusions: Routine screening of PSVT after elective splenectomy is warranted because it allows to start anticoagulant therapy and avoid further life-threatening complications. The incidence of PSVT is particularly high among patients operated on for lymphoma or with splenomegaly., (Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Wandering spleen is a rare pathology. Spleen infarction which results from the torsion of the pedicle is its main complication. We report a case of torsion of a wandering spleen diagnosed by computed tomography. The therapeutic sanction was a splenectomy because of the infarction. Nowadays, splenopexy is the best treatment if the diagnosis is done early., (Le comitée de rédaction se réserve le droit de revoyer aux auteurs avant toute soumission à l'avis des lecteurs les manuscrits qui ne seraient pas conformes à ces modalités de présentation. En outre il leur conseille de sonserver un examplaire du manuscrit, des figures et des tableaux.)

Introduction: Acid sphingomyelinase deficiency leads to a severe infantile disease (Niemann-Pick disease type A) or an attenuated form of the disease encountered in adults (Niemann-Pick type B), including pulmonary fibrosis and splenomegaly., Case Report: A 52-year-old man with Niemann-Pick disease type B was admitted with splenic rupture. Embolization of the splenic artery was initially performed. Three months later, the splenic volume had increased and functional asplenia was diagnosed. Splenic scintigraphy showed 20% of functional splenic tissue. Splenectomy was finally performed because of complete necrosis of the spleen., Conclusion: Despite its theoretical contra-indication in Niemann-Pick disease due to a risk of respiratory insufficiency, splenectomy must sometimes be considered., (Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)