[Splenic dysfunction in sickle cell disease: An update]. / Dysfonction splénique au cours de la drépanocytose : mise au point.

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.

[Pseudohyperkalemia and thrombocytosis]. / Pseudo-hyperkaliémie et thrombocytose.

INTRODUCTION: 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. OBSERVATIONS: 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 7.7mmol/L) are reported. Kalemia on blood collected in heparin tube was normal (4.4-4.6mmol/L). Therefore, no specific treatment for this pseudohyperkalemia was required. CONCLUSION: 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.

[Long-term infectious risks after splenectomy: A retrospective cohort study with up to 10 years follow-up]. / Complications infectieuses à long terme des patients splénectomisés : cohorte rétrospective avec un suivi de plus de 10 ans.

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.

Indications et résultats de la splénectomie chez le drépanocytaire au Centre Hospitalier Universitaire de Libreville / Indications and results of splenectomy in sickle cell patients at the University Hospital Center of Libreville

Introduction : la drépanocytose est une affection génétique héréditaire responsable d'une déformation des hématies en «faucilles¼. Elle peut engendrer de nombreuses complications parmi lesquelles la séquestration splénique et l'hypersplénisme. La splénectomie fait partie de l'arsenal thérapeutique, induisant un état d'hyposplénie ou d'asplénie aux conséquences parfois mortelles, surtout chez le sujet drépanocytaire. Le but de ce travail mené au service de chirurgie du centre hospitalier universitaire de Libreville au Gabon, est de déterminer les indications et les résultats à moyen terme de la splénectomie chez le drépanocytaire.Matériel et méthodes : il s'agissait d'une étude rétrospective, descriptive et monocentrique allant d'octobre 2016 à avril 2019. Tous les dossiers des patients drépanocytaires opérés d'une splénectomie seule ou associée à un autre geste chirurgical étaient retenus.Résultats : au total treize patients ont été inclus dans notre étude. L'âge moyen des patients était de 10,7 ans [3-25 ans]. L'indication opératoire était l'hypersplénisme (8 cas), la séquestration splénique (3 cas), et l'infarctus splénique (2 cas). Tous les patients étaient opérés d'une splénectomie totale par laparotomie. La vaccinothérapie et l'antibioprophylaxie post-opératoire étaient réalisées chez tous les patients. Aucune complication post-opératoire immédiate ni aucun décès n'avaient été noté chez tous les patients.Conclusion : l'indication des splénectomies chez le drépanocytaire est dominée par l'hypersplénisme. Dans notre context la splénectomie totale par laparotomie semble être la règle. La vaccination prophylactique est bien administrée. La morbi-mortalité post-opératoire immédiate est nulle.

Introduction: Sickle cell disease is a genetic inherited disorder responsible for the deformation of red blood cells into"sickles". It can lead to many complications including splenic sequestration and hypersplenism. Splenectomy is the indicated gesture. It induces a state of hyposplenia or asplenia with dreadful consequences, sometimes fatal especially in this sickle cell patient. The aim of this work, conducted at the Surgery Department of Libreville University Hospital Center in Gabon, is to determine the indications and results of splenectomy in sickle cell patients.Material and methods: this was a retrospective, descriptive and single-center study from October 2016 to April 2019.All records of sickle cell patients undergoing splenectomy alone or with other surgical procedures were included.Results: a total of thirteen patients were included in our study. The average age of the patients was 10.7 years [3-25years]. The operative indication was hypersplenism (8 cases), splenic sequestration (3 cases), and splenic infarction(2 cases). All patients underwent total splenectomy under laparotomy. Vaccine therapy and postoperative antibiotic prophylaxis were performed in all patients. No immediatepostoperativecomplications or deaths were noted in all patients. Conclusion: the indication of splenectomies in sickle cell disease is dominated by hypersplenism. In our context, total splenectomy under laparotomy was the rule. Prophylactic vaccination is well administered. Immediate postoperative morbidity and mortality is nil.

[Routine screening of splenic or portal vein thrombosis after splenectomy]. / Dépistage systématique d'une thrombose veineuse splénique ou portale après splénectomie.

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.

[Torsion of wandering spleen: a case report in the Kara University Hospital (Togo)]. / Torsion d'une rate ectopique: a propos d'un cas au CHU Kara (Togo).

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.

La rate ectopique ou baladeuse est une entité pathologique rare. Sa complication majeure est la torsion de son pédicule pouvant conduire à un infarctus splénique. Nous rapportons un cas de torsion de rate ectopique dont le scanner avait permis de poser le diagnostic. La splénectomie a été la sanction thérapeutique à cause de son infarcissement. Actuellement, la splénopexie est proposée quand le diagnostic est fait précocement.

[Acid sphingomyelinase deficiency and spleen trauma: splenectomy or not splenectomy?]. / Déficit en sphingomyélinase acide et rupture splénique : splénectomie ou traitement conservateur ?

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.

[Osteolytic bone lesion: vertebral alveolar echinococcosis in a patient with splenectomy]. / Lésion ostéolytique chez une patiente splénectomisée : à propos d'un cas d'échinococcose alvéolaire vertébrale.

INTRODUCTION: Osteolytic lesions are not always related to malignancies. CASE REPORT: We report an 82-year-old woman suffering from subcostal pain. The patient underwent a splenectomy 40 years previously. CT-scan and MRI highlighted a calcified hepatic lesion associated with an osteolytic lesion of the L5 vertebra. Osteolytic and hepatic lesions were attributed to an alveolar echinococcosis based on positive serological assays. CONCLUSION: To our knowledge, this is the first report of an alveolar echinococcosis in a patient with splenectomy and secondary lesions. We suggest that the splenectomy could have promoted the parasite spreading to vertebra.

[Post-splenectomy complications in primary immune thrombocytopenia. Literature review and preventive measures]. / Complications de la splénectomie au cours du purpura thrombopénique immunologique. Revue de la littérature et mesures de prévention.

Management of primary immune thrombocytopenia (ITP) has changed, and clinical practice broadens the use of thrombopoietin receptor agonists and anti-CD20 antibody as options for second-line therapy, as alternative to splenectomy. Splenectomy remains a successful, definitive curative treatment. The purpose of this review about the complications of the splenectomy, in the context of ITP, is to increase the awareness of clinicians towards the preventive measures, which are often not correctly applied.

[Current management of thalassemia intermedia]. / Prise en charge actuelle des thalassémies intermédiaires.

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.