Splenectomy complicated by sustained extreme thrombocytosis and extensive portosplenomesenteric vein thrombosis in pyrimidine 5′-nucleotidase deficiency

Al-Jafar, Hassan; Taqi, Ali; Madda, John Patrick; A.Abdullah, Thamer

doi:10.1136/bcr-2013-201061

Cited by 2 publications

(1 citation statement)

References 18 publications

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“… 64 – 67 Portosplenomesentric venous thrombosis was described in one patient with pyrimidine-5′-nucleotidase following splenectomy due to trauma. 68 As there are insufficient data regarding the efficacy and complications of splenectomy in this disorder no recommendations could be made.…”

Section: Disease-specific Recommendationsmentioning

confidence: 99%

Recommendations regarding splenectomy in hereditary hemolytic anemias

et al. 2017

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Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children.

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Section: Disease-specific Recommendationsmentioning

confidence: 99%

Recommendations regarding splenectomy in hereditary hemolytic anemias

et al. 2017

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Peripheral Blood Cells Changes After Two Groups of Splenectomy and Prevention and Treatment of Postoperative Complication

Lv¹,

Gong²,

Han³

et al. 2018

J Hypertens

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Objective: This study aimed to investigate the changes in peripheral blood cells after two groups of splenectomy in patients with traumatic rupture of the spleen and portal hypertension group, as well as causes and prevention and treatment of splenectomy related portal vein thrombosis. Methods:Clinical data from 109 patients with traumatic rupture of the spleen who underwent splenectomy in our hospital from January 2001 to August 2015 were retrospectively analyzed, and compared with those from 240 patients with splenomegaly due to cirrhotic portal hypertension who underwent splenectomy over the same period.Results: After splenectomy, peripheral platelet (PLT) count was significantly increased in both groups (P<0.01), and the increase was significantly greater in the traumatic rupture group than in the portal hypertension group (P<0.05). The red blood cell (RBC) count remained unchanged (P>0.05), while the white blood cell (WBC) count was significantly decreased (P<0.05) in the traumatic rupture group. Both WBC and RBC counts were increased significantly (P<0.05) in the portal hypertension group. Both groups received routine anticoagulant therapy after splenectomy. The incidence of portal vein thrombosis was 8.26% and 6.67% in the traumatic rupture and portal hypertension groups (P>0.05), respectively, which was alleviated with urokinase. Conclusion:The significant increase of peripheral PLT count in both groups after splenectomy might be caused by the removal of the site that stored blood cells. The more significant increase of PLT in the traumatic rupture group might be related to a constant production and release of thrombopoietin by the normal liver into blood circulation. The lack of increase of RBC count in the traumatic rupture group indicated no storage of RBCs in the spleen, and the significant increase of WBC count was related to the control of inflammation. The significant increase of WBC and RBC counts in the portal hypertension group was related not only to the elimination of spleen storage, but also to the elimination of hypersplenism. Postoperative routine anticoagulant and thrombolytic therapy could prevent and treat portal vein thrombosis.

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Splenectomy complicated by sustained extreme thrombocytosis and extensive portosplenomesenteric vein thrombosis in pyrimidine 5′-nucleotidase deficiency

Cited by 2 publications

References 18 publications

Recommendations regarding splenectomy in hereditary hemolytic anemias

Recommendations regarding splenectomy in hereditary hemolytic anemias

Peripheral Blood Cells Changes After Two Groups of Splenectomy and Prevention and Treatment of Postoperative Complication

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