Search results for "Splenectomy"

Cholelithiasis in Patients with Gaucher Disease type 1: Risk Factors and the Role of ABCG5/ABCG8 Gene Variants

2016

Background & Aim: Patients with Gaucher disease type 1 (GD1) show an altered lipid profile and a certain degree of insulin resistance, which might contribute to cholelithiasis (CL) and could possibly be associated with ABCG5/ABCG8 gene variants. We aimed to investigate the prevalence of CL in Caucasian adult patients with GD1 and the possible risk factors, including gene variants of the ABCG5/ABCG8 genes.
 Methods: 61 Caucasian patients with GD1 (38 female/23male), aged 18-62 years and 61 healthy subjects matched for age, gender and BMI, without CL, for comparison of lipid profiles. Data before start of enzyme replacement therapy (ERT) were recorded: clinical, haematological, sever…

How I diagnose and treat splenic lymphomas.

2011

Abstract The incidental finding of an isolated splenomegaly during clinical assessment of patients evaluated for unrelated causes has become increasingly frequent because of the widespread use of imaging. Therefore, the challenging approach to the differential diagnosis of spleen disorders has emerged as a rather common issue of clinical practice. A true diagnostic dilemma hides in distinguishing pathologic conditions primarily involving the spleen from those in which splenomegaly presents as an epiphenomenon of hepatic or systemic diseases. Among the causes of isolated splenomegaly, lymphoid malignancies account for a relevant, yet probably underestimated, number of cases. Splenic lymphoma…

Efficacy of bendamustine and rituximab in splenic marginal zone lymphoma: results from the phase II BRISMA/IELSG36 study.

2018

Splenectomy in addition to immunotherapy with rituximab can provide quick and sometimes durable disease control in patients with splenic marginal zone lymphoma (SMZL). However, systemic chemotherapy is ultimately required in many cases. The BRISMA (Bendamustine-rituximab as first-line treatment of splenic marginal zone lymphoma)/IELSG (International Extranodal Lymphoma Study Group)36 trial is an open-label, single arm phase II study designed by the IELSG in cooperation with the Fondazione Italiana Linfomi and the lymphoma Study Association according to Simon's two-stage method. The primary endpoint was complete response rate. Fifty-six patients with SMZL diagnosis confirmed on central revis…

Intrasinusoidal bone marrow infiltration: a possible hallmark of splenic lymphoma.

1996

A particular type of lymphomatous bone marrow infiltration defined as intrasinusoidal and occuring in seven patients with splenomegaly and mild to moderate thrombocytopenia is reported. Immunocytochemistry highlighted the intrasinusoidal pattern and showed a mature B-cell phenotype. Four patients underwent splenectomy and a diagnosis of splenic marginal cell lymphoma was made. The disease course seems to be chronic and quite indolent. All patients are alive and well ; follow-up ranges from 2 to 42 months. The value of bone marrow biopsy as a primary diagnostic tool in splenic marginal zone lymphomas is discussed.

Splenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment

2022

Although splenectomy is still considered the most effective curative treatment for immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the approval of thrombopoietin receptor agonists (TPO-RAs). The main objective of the study was to determine whether splenectomy was still as effective nowadays, particularly for patients with failure to respond to TPO-RAs. Our secondary objective was to assess, among patients who relapsed after splenectomy, the pattern of response to treatments used before splenectomy. This multicentre retrospective study involved adults who underwent splenectomy for ITP in France from 2011 to 2020. Response status was defi…

Splenic marginal zone lymphoma with or without villous lymphocytes

2004

BACKGROUND Splenic marginal zone lymphoma (SMZL) is a well defined pathologic entity. However, questions regarding the bone marrow infiltration rate, the minimal diagnostic data set, and therapy remain unanswered. METHODS Clinical-pathologic features and outcomes of 57 consecutive patients who had splenomegaly with no clinically significant lymphadenomegaly and who were diagnosed with SMZL with or without (±) villous lymphocytes (VL) were reviewed. RESULTS SMVL ± VL occurred mostly in elderly males (median age, 62 years ± 10 years; male-to-female ratio, (1.85). Anemia was recorded in 49% of patients, and 30% of patients had moderate thrombocytopenia. Leukocytosis and leukopenia were found i…

EP1224 Predictors and clinical outcome of pancreatic fistula in patients receiving splenectomy for advanced or recurrent ovarian cancer: a large mult…

2019

Introduction/Background The aim of the surgical treatment of advanced ovarian cancer is to completely remove all macroscopic disease.To achieve optimal cytoreduction, the use of extensive upper abdominal surgery has become widely accepted.Although the disease doesn’t usually invade the splenic parenchyma, the capsule may commonly be infiltrated. These metastases may be difficult to be dissected from the pancreatic tail and a partial pancreatic resection en bloc with spleen may be required. The objective of this study is to evaluate predictors and clinical outcome of pancreatic fistula in patients receiving splenectomy for advanced or recurrent ovarian cancer. Methodology Data of women recei…

Outcome of type III Gaucher disease on enzyme replacement therapy: review of 55 cases.

2007

The European Task Force for Neuronopathic Gaucher Disease (NGD) met in 2006 to review its 2001 guidelines. Fifty-five patients from five European countries were reviewed; 29 were male and 26 female. The majority of the patients were homozygous for the L444P mutation. All had been on enzyme replacement therapy (ERT). However, there was considerable variation in the dose of ERT, as well as an uneven distribution of risk factors. Thus, the oldest patients were on the lowest doses, and several had had a total splenectomy, while the youngest patients had a high proportion of compound heterozygosity and were on the highest doses, and very few had had a splenectomy. This heterogeneity rendered ana…

Schistosomiasis and antiviral treatment of chronic hepatitis C

2003

Solitary splenic metastasis from nasopharyngeal carcinoma: a case report and systematic review of the literature

2016

Background: Solitary splenic metastases are a rare occurrence, and the nasopharyngeal carcinoma represents one of the most uncommon primary sources. The present study aimed to describe a rare case of a solitary single splenic metastasis from nasopharyngeal carcinoma and to assess the number of cases of isolated nasopharyngeal carcinoma metastases to the spleen reported in the literature. Main body: We describe the case of a 56-year-old man with a history of nasopharyngeal carcinoma and complete remission after chemo-radiotherapy. Three months after complete remission, positron emission tomography/ computed tomography scan revealed a hypermetabolic splenic lesion without increased metabolic …