Surgical Indications in Idiopathic Splenomegaly

Carr, John Alfred; Shurafa, Muhammad; Velanovich, Vic

doi:10.1001/archsurg.137.1.64

Cited by 30 publications

(34 citation statements)

References 16 publications

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“…According to a surgical classification [39], the removed spleen had a moderately increased size, and its gross appearance was essentially congestive and fibrotic. On histological examination, there was no evidence of any of the haematologic, infectious or inflammatory diseases most commonly associated with spleen enlargement [21,40,41]. The diagnostic yield of splenectomy is very high [40,41] and, as occurred in our patient, up to 40% of all In these analyses, a patient was only represented once at each eGFR stage, using the smallest haemoglobin level, WBC or platelet count available at that particular level of renal function.…”

Section: Discussionmentioning

confidence: 68%

Splenomegaly, hypersplenism and peripheral blood cytopaenias in patients with classical Anderson–Fabry disease

et al. 2008

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A 39-year-old male with classical Anderson-Fabry disease (AFD) and long-standing idiopathic splenomegaly, who had been on haemodialysis since the age of 24, was splenectomised for symptomatic pancytopaenia. Spleen enlargement was first noted at clinical presentation, at age 16, but despite thorough investigation its cause remained unclear. Anaemia, leukopaenia and thrombocytopaenia were first observed a few years thereafter, but well before the start of dialytic treatment. On gross pathological examination the spleen weighed 700 g and had a fibrocongestive appearance. Histologically, it showed expansion of the red pulp and decreased white pulp. Some histiocytes and many of the endothelial cells lining the sinusoids had vacuolated cytoplasm with argyrophilic material within, suggesting their involvement in the storage pathology of AFD. In a retrospective review of our cohort of patients with classical AFD (n = 10), complete blood counts showing anaemia, leukopaenia or thrombocytopaenia were found in five, two and four patients, respectively, including a 6-year-old boy, whose spleen was also enlarged. Data from AFD international registries show that peripheral blood cytopaenias, particularly anaemia, are prevalent among these patients. Sinusoidal endothelial involvement resulting in compromise of splenic blood flow may be the cause of congestive splenomegaly and hypersplenism in classical AFD.

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Section: Discussionmentioning

confidence: 68%

Splenomegaly, hypersplenism and peripheral blood cytopaenias in patients with classical Anderson–Fabry disease

et al. 2008

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“…5 In the subset of patients in which splenomegaly remains "unexplained," lymphomas account for the leading cause (Table 3). [6][7][8] When approaching patients with suspect SLs, our primary effort is to achieve a diagnosis by integrating presenting clinical and laboratory data with imaging, PB, and BM assessment, thus trying to avoid splenectomy for diagnostic purposes. The main steps of our diagnostic workup of SLs are schematically represented in Figure 1.…”

Section: Diagnostic Workupmentioning

confidence: 99%

How I diagnose and treat splenic lymphomas

Iannitto

Tripodo

2011

Blood

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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 lymphomas constitute a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. Such a clinical heterogeneity is paralleled by the high degree of biologic variation in the lymphoid populations from which they originate. Nevertheless, the presenting clinical, laboratory, and pathologic features of these diseases often display significant overlaps. In this manuscript, we present our approach to the diagnosis and treatment of these rare lymphomas, whose complexity has been so far determined by the lack of prospectively validated prognostic systems, treatment strategies, and response criteria. (Blood. 2011;117(9):2585-2595)

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“…2) CD1 0 ( D) , b c l -2 , a n d b c l - (1)(2)(3)(6)(7)(8)(9)(10)(11)(12). Moreover, other benign diseases such as tuberculosis, Castleman disease, sarcoidosis and adult onset still disease have also been diagnosed by splenectomy (2,3,7,8 (2,7,(9)(10)(11)(12). Therefore, splenectomy is useful not only for diagnosis but also for treatment underlying hematologic malignancy.…”

Section: F I G U R E 1 P L a I N Ct S H O We D Ma S S I V E S P L Ementioning

confidence: 99%

Localized, Splenic, Diffuse Large B-Cell Lymphoma Presenting with Hypersplenism: Risk and Benefit of Splenectomy

et al. 2010

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Surgical Indications in Idiopathic Splenomegaly

Cited by 30 publications

References 16 publications

Splenomegaly, hypersplenism and peripheral blood cytopaenias in patients with classical Anderson–Fabry disease

Splenomegaly, hypersplenism and peripheral blood cytopaenias in patients with classical Anderson–Fabry disease

How I diagnose and treat splenic lymphomas

Localized, Splenic, Diffuse Large B-Cell Lymphoma Presenting with Hypersplenism: Risk and Benefit of Splenectomy

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