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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.)

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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]

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]

Copyright of Journal Africain D'Hépato-Gastroentérologie is the property of Lavoisier and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction. – Splenic tuberculosis is rare. The aim of this study was to remind diagnostic problems in this disease, and to evaluate the value of surgery in its management.Matériel et méthodes. – Retrospective study of data of six patients (three male and three female, whose mean age was 50 years) admitted in surgical department from 1980 to 2000 for splenic tuberculosis.Results. – In six cases, symptoms were poorly specific; splenomegaly and anemia were constant. In imaging studies, splenomegaly was homogeneous in four cases and heterogeneous in two. Diagnosis of tuberculosis was done without need for splenectomy in four patients. Five patients were operated on: two had diagnostic laparotomy without splenectomy, two underwent diagnostic splenectomy and one had splenectomy indicated for splenic abcess refractory to medical treatment. Four of the five operated patients recovered. The two other patients were lost of follow-up.Conclusions. – In our experience, surgery is useful in diagnosis and treatment of splenic tuberculosis. [Copyright &y& Elsevier]

This study reports 3 new cases of spontaneous (or atraumatic) splenic rupture of the spleen, including two with massive hemoperitoneum and one with a secondary rupture of splenic infarct, and reviews the literature about this rare disease. These spontaneous ruptures are rare and potentially fatal. They result from infectious diseases (mainly mononucleosis and and paludism) and hematological diseases (mainly malignant hemopathies) in more than 50% of cases. Mortality is close to 20%, and includes some deaths occuring before diagnosis was established and postoperatives deaths, which can result from delayed management and bad general condition of the patients. Splenectomy is usually mandatory. Non-surgical treatment can be indicated only in young and stable patients. [Copyright &y& Elsevier]

Abstract: In haemodynamically stable children with splenic trauma, conservative treatment is recommended to preserve the spleen and prevent potentially lethal post-splenectomy infectious complications. We report on the case of a 11-year-old child who suffered a fistula of a huge subcapsular splenic hematoma into the colon, 16 days after the traumatism. Decision to sustain the non-operative treatment allowed the preservation of the spleen without complications. [Copyright &y& Elsevier]

Splenic rupture associated with anticoagulant therapy is a rare entity. We report a case of spontaneous splenic rupture in a 62-year-old man who was treated by acenocoumarol orally after an aortic-valve replacement. Unrecognised, minor trauma may lead to splenic haemorrhage in patients receiving anticoagulant therapy. The symptoms and signs may mimic those of acute myocardial infarction and cardiogenic shock. Early diagnosis is essential. Reversal of the anticoagulant and emergent splenectomy are the treatments of choice. [Copyright &y& Elsevier]