1/16. splenectomy in haemophagocytic lymphohistiocytosis: report of histopathological changes with CD19 B-cell depletion and therapeutic results.The pathogenesis of haemophagocytic lymphohistiocytosis (HLH) in children without a known familial pattern of inheritance is often difficult to establish. Splenic enlargement, one of the main clinical findings in this disorder, has led to the use of splenectomy for uncontrollable coagulopathy, persistent cytopenia or both. This procedure is also thought to be a useful tool in making a differential diagnosis in cases of the immunochemotherapy-resistant HLH. We report here five cases of splenectomized childhood HLH, in which subsets of mononuclear spleen cells were analysed either by flow cytometry or immunohistochemistry, and the results were compared with those from cases of hereditary spherocytosis (controls). There was a statistically significant depletion of CD19 B cells in the HLH cases (3.8 /- 3.2% vs. 52.6 /- 4.5%, P < 0. 0001) associated with an increase of T cells in three cases and of natural killer cells in another. The histopathological findings included atrophic white pulps, B-cell depletion with fibrosis and haemosiderosis in all five cases. Despite temporary therapeutic benefits, three of the HLH patients had a rapidly deteriorating post-splenectomy course and all three eventually died. These results demonstrate striking depletion of B cells in the enlarged spleens of children with HLH, which may be an intrinsic feature of HLH pathogenesis. Further study is needed to establish the therapeutic value of splenectomy in this disease.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
2/16. splenosis in a port site after laparoscopic splenectomy.splenosis, the autotransplantation of splenic tissue, is most commonly seen after traumatic splenic rupture and splenectomy. It also can occur during embryonic development. Intraperitoneal, intrathoracic, and retroperitoneal sites have been reported. Although the presence of the splenic tissue often is asymptomatic and an incidental finding, it may present with pain or be confused with various pathologies including neoplasia. Because most pediatric splenectomies are performed for hemolytic disorders, parenchymal disruption must be contained to avoid recurrent disease. We present a case in which the devascularized spleen was contained in a bag and fragmented in situ. splenosis developed in the retrieval port site after laparoscopic splenectomy and cholecystectomy. Port-site splenosis needs to be considered in the differential diagnosis of port-site pain and a palpable nodule postsplenectomy.- - - - - - - - - - ranking = 0.5keywords = spleen (Clic here for more details about this article) |
3/16. Extramedullary hematopoiesis in hereditary spherocytosis deficient in ankyrin: a case report.Hereditary spherocytosis (HS) is a common inherited hemolytic anemia due to red cell membrane defects. Extramedullary hematopoiesis is a compensatory response to insufficient bone marrow blood cell production. The preferred sites of extramedullary hematopoietic involvement are the spleen, liver, and lymph nodes, but in HS the posterior paravertebral mediastinum is also commonly involved. A nonsplenectomized 74-year-old man with mild HS, with primary deficiency in ankyrin, was found by magnetic resonance imaging to have thoracic paravertebral hematopoietic masses. The patient showed high serum levels of erythropoietin, which may have played a role in the development of extramedullary hematopoietic masses through a continuous hematopoietic stimulus to erythroid cells in the propositus. The long-standing history of respiratory infections and of hypoxia in the propositus may have been an additional etiological factor.- - - - - - - - - - ranking = 0.5keywords = spleen (Clic here for more details about this article) |
4/16. erythropoiesis inhibitor in a patient with hereditary spherocytosis.The studies dealt with the effect of plasma of a patient with congenital haemolytic anaemia on the erythropoiesis in mice. The materials included the plasma from the patient before and after splenctomy and the spleen homogenate and the spleen subcellular fractions. The effect of the materials was evaluated with the amount of the 59Fe taken up by the erythrocytes of the mice. The erythropoiesis was found to be inhibited by the plasma before splenectomy and by the spleen homogenate and its subcellular fractions. The inhibition was the highest in cases when the mice was given the spleen homogenates previously incubated with plasma of healthy persons.- - - - - - - - - - ranking = 2keywords = spleen (Clic here for more details about this article) |
5/16. Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration.The association of sickle cell trait (SCT) and hereditary spherocytosis (HS) has been reported in only 18 patients. Three of these 18 patients experienced splenic infarct or acute splenic sequestration. We report here a 46-year-old African-American male, the oldest reported case to date, who experienced episodes of hemolysis and severe left upper quadrant pain for the past 26 years. The patient had compensated hemolysis with splenomegaly. A CT scan of the abdomen revealed a large infarct in the spleen. The diagnosis of SCT was confirmed with isoelectric focusing, cation exchange and reverse-phase HPLC. The presence of a silent, interacting globin variant as the cause of hemolysis and sickling in the spleen was ruled out by sequencing of the alpha1-, alpha2- and beta-globin genes. The diagnosis of HS was established by an osmotic fragility test. The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
6/16. Pelvic extramedullary haematopoiesis associated with hereditary spherocytosis.Extramedullary haematopoiesis (EMH) is a rare disorder and is characterised by the appearance of haemopoietic tissue outside of the bone marrow. The most common of the previously recognised sites of EMH are the spleen and the liver. This case-report describes a unique case of pelvic EMH secondary to herditary spherocytosis with regression of the lesion following splenectomy. Current principles of managing EMH are also discussed.- - - - - - - - - - ranking = 0.5keywords = spleen (Clic here for more details about this article) |
7/16. Splenic sequestration associated with sickle cell trait and hereditary spherocytosis.Coexistence of sickle cell trait and hereditary spherocytosis (HS) is unusual, and only 16 cases have been reported in the literature. These patients have the same clinical and hematological features as individuals having HS alone. We report a serious complication, acute splenic sequestration crisis (ASSC), occurring in two patients with sickle cell trait and HS. One patient experienced four episodes of ASSC during an 11-year span, while the other had two episodes of this complication during a 4-year period. Red blood cell studies and membrane protein analysis confirmed the diagnosis of HS as a consequence of spectrin deficiency. splenectomy resulted in marked clinical and hematological improvement in both patients. Histological examination of spleens following splenectomy confirmed that significant erythrostasis and sickling had indeed occurred. ASSC can occur in patients with coexistence of sickle cell trait and HS, and this potentially life-threatening complication should be considered in this condition.- - - - - - - - - - ranking = 0.5keywords = spleen (Clic here for more details about this article) |
8/16. A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis.BACKGROUND: Partial splenectomy is sometimes used for children with hereditary spherocytosis (HS) to reduce hemolysis while retaining some splenic immune function. Previous reports have described a partial splenic resection through a laparotomy incision. Whereas laparoscopic total splenectomy for HS is well-established, laparoscopic partial splenectomy (LPS) has not been described. The authors have developed a novel LPS technique that combines the benefits of partial splenectomy with those of a laparoscopic approach. methods: A chart review was conducted for three children with HS who underwent LPS, with approximately one-fourth of the spleen left on the basis of the short gastric arterial supply. RESULTS: The mean preoperative spleen size was 17.6 cm. The mean preoperative hemoglobin count was 100 g/l, and the postoperative hemoglobin count was 133 g/l. All three patients reported reduced malaise and increased energy levels. There was no recurrent anemia at the 1- to 2-year follow-up evaluation. CONCLUSION: The LPS procedure is a safe and effective approach to HS that resolves anemia, potentially retains some splenic immunity, and confers the benefits of a minimal access technique.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
9/16. Preoperative computed tomography and scintigraphy to facilitate the detection of accessory spleen in patients with hematologic disorders.Accessory spleens of 1.5 cm in size were preoperatively identified by the combined use of computerized tomography and splenic scintigraphy in two patients with hematologic diseases. After the accessory spleen had been removed from the first patient, who had persistent hereditary spherocytosis and had undergone a splenectomy 15 months before, a postoperative decrease in hyperbilirubinemia was noted. In the other patient who had idiopathic thrombocytopenic purpura, a successful accessory splenectomy was done at the same time as her splenectomy, and was followed by 6 months' complete remission. These events indicate that preoperative investigations using computerized tomography and scintigraphy are indispensable for ruling out an accessory spleen in those patients for whom splenectomy needs to be done in order to alleviate hematologic disorders.- - - - - - - - - - ranking = 3.5keywords = spleen (Clic here for more details about this article) |
10/16. Embolisation of the splenic artery as an alternative to splenectomy for treating hereditary spherocytosis.In 48 years old men with microspherocytosis constantly refusing splenectomy an experimental embolisation of the spleen artery with Spongostan was performed. 5 years of follow-up confirm the efficiency of this method of management. Further observations, however, are needed to formulate the indication for this method of treatment.- - - - - - - - - - ranking = 0.5keywords = spleen (Clic here for more details about this article) |
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