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Africa
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Gaucher disease
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Thrombocytopenia is a common hematologic manifestation of Gaucher disease [26]. It may be accompanied by leukopenia and anemia, the full picture of hypersplenism, and resolves with splenectomy [28]. Late hematologic dysfunction in splenectomized patients may result from replacement of normal marrow with Gaucher cells. There may be bleeding, petechiae, and easy bruising.
Haematological malignancy
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
There might be bone marrow failure resulting in anaemia, reduced resistance to infection and a bleeding tendency from thrombocytopenia. This can be exacerbated by the effects of gross splenomegaly, causing the phenomenon of hypersplenism with pooling of blood within the large spleen. Massive splenomegaly can also cause local pain and might impair gastric emptying. Local areas of infarction can occur within the spleen, causing acute pain.
Pathology of the Spleen
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Hypersplenism is characterized by cytopenias, compensatory bone marrow hyperplasia, splenomegaly, and correction of cytopenia after splenectomy. The differential diagnosis of splenomegaly is shown in Table 1. The pathogenesis is attributed to splenic sequestration and destruction of trapped blood elements. Sequestration is caused by abnormalities in the blood cells or of the spleen itself. Abnormalities of red cells are either congenital or acquired. Congenital red cell abnormalities include hereditary spherocytosis, hemoglobinopathies, and hereditary hemolytic anemias. Acquired red cell disorders include malaria and autoimmune hemolytic anemia. Splenic causes of hypersplenism are due to disorders of cordai macrophages or secondary to infiltrative diseases. Disorders of cordai macrophages include the storage diseases, Langerhans cell histiocytosis, and hemophagocytic syndromes. In these disorders, the cords are widened and macrophages are abundant. Infiltrative neoplastic diseases and vascular abnormalities may also cause hypersplenism.
Phenotypic variation in sickle cell disease: the role of beta globin haplotype, alpha thalassemia, and fetal hemoglobin in HbSS
Published in Expert Review of Hematology, 2022
The strength of the Jamaican cohort is the opportunity to collate hematological and clinical observations in the same environment, conducted by dedicated staff with defined protocols over periods up to 48 years but the disadvantage is the relatively small number of subjects. Within these limitations, low HbF levels were associated with dactylitis [70], gallstones [19], acute splenic sequestration (ASS) [71], and an earlier clinical presentation [72]. Lower HbF levels at 2 years was also related to an increase in deaths [73], and applying survival curve analysis in terciles of the HbF distribution at 10 years [74] found that low HbF levels increased the risk of acute chest syndrome, dactylitis, bone pain crisis, and ASS but that these effects were confined to males who tended to have lower HbF levels. This approach also found no relationship with chronic hypersplenism suggesting that this may have different risk factors from ASS. Data from the non-Cohort clinic in Jamaica also showed relationships between low HbF and splenomegaly [59] and priapism [75].
A rare vascular tumor of the spleen: Littoral cell angioma
Published in Acta Chirurgica Belgica, 2021
Littoral cell angioma (LCA) is a rare primary vascular tumor of the spleen arising from the littoral cells lining the splenic red pulp sinuses. LCA is usually benign and is seen in males and females at equal rates and at any age. The etiology of LCA remains unknown [1]. Patients are generally asymptomatic, but signs of hypersplenism including anemia, thrombocytopenia and pancytopenia, and systemic symptoms such as fever, chills, weakness, fatigue, and abdominal pain may exist. In physical examination, an abdominal mass associated with splenomegaly can be palpated. Usually discovered incidentally, LCA may involve nonspecific findings in the form of splenomegaly and multiple masses of similar size and appearance in the spleen with imaging techniques such as abdominal ultrasonography (USG), computerized tomography (CT) and magnetic resonance imaging (MRI), and these may contribute to the diagnosis of LCA. The final diagnosis of LCA requires histopathological and immunohistochemical analyses of the splenectomy material and splenectomy is a gold standard also for the treatment [2].
Improvement of human platelet aggregation post-splenectomy with paraesophagogastric devascularization in chronic hepatitis B patients with cirrhotic hypersplenism
Published in Platelets, 2020
Hui Zhang, Shaoying Zhang, Jian Zhang, Rui Zhou, Yongzhan Nie, Song Ren, Jun Li, Keping Feng, Fanpu Ji, Guangyao Kong, Zongfang Li
Hypersplenism encompasses a group of syndromes that can be caused by a variety of diseases. In China, hypersplenism is most commonly secondary to hepatitis virus-related cirrhosis [6]. The syndrome, in general, is closely associated with PH and the transition from compensation to decompensation in the progressive stage of cirrhosis [7,8]. Hypersplenism is also associated with bleeding tendency and pancytopenia in cirrhotic patients, and splenectomy can reduce the portal blood flow and improve the pancytopenia [9]. SPD has emerged as an efficacious surgical therapy for PH. Indeed, it has been recommended as the first-choice therapy in China for a long time. As a conventional and classic surgical procedure, SPD can produce a decrease in portal vein pressure, ultimately reducing the risk of variceal hemorrhage.