China
Africa
Explore chapters and articles related to this topic
Haematology and oncology
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
Hereditary spherocytosis is autosomal dominant. As the majority of cells are destroyed by the spleen, splenectomy relieves the symptoms. Although gallstones have been reported in children as young as 4-5 years of age, in most cases they occur in late childhood or adolescence. Haemolysis occurs because of the abnormality of the cells and not due to oxidizing agents. Red cell morphology remains unchanged after splenectomy.
The Spleen
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
This is a rare familial condition, transmitted as autosomal dominant trait. It is similar to hereditary spherocytosis, however, the red cells are oval and rod shaped. The anemia is usually mild. However, severe hemolysis is rare, but it can occur causing anemia and jaundice. This condition also responds to splenectomy.
Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
In hereditary spherocytosis, in addition to the above-described signs and symptoms of chronic hemolysis, splenomegaly, hepatomegaly, and cholelithiasis are frequently observed. The osmotic fragility of RBCs is increased, the Coombs test is negative.
Severe autoimmune hemolytic anemia complicating hereditary spherocytosis treated successfully with glucocorticoids and cyclosporine: a case report
Published in Hematology, 2023
Na Wang, Hongkai Lu, Linzhang Li, Ming Gong, Yongtong Cao
Hereditary spherocytosis (HS) encompasses a heterogeneous group of inherited anemias characterized by the presence of spherical RBCs on peripheral blood smears. It is prevalent worldwide, with a high incidence of 1:2000 in northern Europeans [6] and an incidence of about 1:100,000 in the Chinese population [7]. HS is characterized by anemia, jaundice, splenomegaly, and cholelithiasis [8]. In addition, abnormalities in the quantity and/or quality of erythrocyte membrane proteins can lead to the reduced deformability of erythrocytes, which can easily break down and cause hemolysis [9,10]. Five genes are associated with HS and involved in the interaction between the erythrocyte membrane and lipid bilayer: ankyrin (ANK1); β-spectrin (SPTB); α-spectrin (SPTA1); solute carrier family 4, member 1 (SLC4A1); and erythrocyte membrane protein 4.2 (EPB42) [11–13]. Mutations in one or more of these genes can cause membrane protein deficiency, leading to HS [14,15].
Successful treatment of a patient with refractory immune thrombocytopenic purpura in systemic lupus erythematosus with rituximab
Published in Immunological Medicine, 2019
Kazuya Abe, Yuichi Ishikawa, Junichi Ishikawa, Michio Fujiwara, Yasuhiko Kita
CSs are first-line therapy in SLE-ITP treatment; most patients respond relatively well and approximately 20% achieve remission after CS monotherapy [4]. When SLE-ITP is resistant to CS monotherapy, splenectomy or IVIG are sometimes used, similar to primary ITP; however, this has not been well-studied and randomized controlled trials are lacking [8]. In SLE-ITP patients with a high risk of hemorrhage, IVIG is a good therapeutic option because it results in a rapid increase in platelet count [9]. However, its effectiveness may be temporary and induce difficulty of tapering CSs [10]. In addition, IVIG is expensive and carries a small risk of anaphylactic and anaphylactoid reaction. On the other hand, splenectomy may induce a lupus flare or lupus nephritis, given that the spleen is responsible for clearance of circulating immune complexes [11]. In addition, patients who undergo splenectomy are at risk for postsplenectomy infection. A previous study has reported a lifelong risk of severe and potentially fatal infection in patients with hereditary spherocytosis after splenectomy [12]. This is a particular concern in SLE patients who may require long-term immunosuppressive therapy. Moreover, the splenectomy procedure itself may have a high risk in SLE patients receiving CSs because CSs increase the risk of perioperative infection and gastrointestinal hemorrhage and impair wound healing [13–15].
Spleen is cool
Published in Pediatric Hematology and Oncology, 2019
Red cell membrane mediates passive transport functions for flux of monovalent cations and acts as a skeleton conferring mechanical resilience and deformability. Hereditary Spherocytosis (HS) is a disorder of red cell mechanics accompanied with osmotic changes and the most common inherited anemia among Caucasians affecting about 1:2000. Splenectomy remains the mainstay of management of HS to this day unlike disorders of the passive flux of cations leading to red cell stomatocytoses where splenectomy is contraindicated.6In this issue of PHO Tripodi et al compare the clinical and laboratory outcome in 79 patients with HS who underwent total (n = 33) or partial splenectomy (n = 46). Though short-term postsurgical complications were more frequent after partial splenectomy, both modalities relieved symptoms and hemoglobin levels were not notably different between total and partial splenectomy cohorts upon long term follow up (24.9 and 59.6 months respectively).