Cases reported "Hypersplenism"

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1/10. portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy.

    A 12-year-old girl underwent laparoscopy-assisted splenectomy and cholecystectomy with removal of her spleen through a small Pfannenstiel incision. She had an unremarkable postoperative course but returned 16 days later because of increasing right-sided abdominal pain. The pain was constant, sharp, and stabbing without radiation. Abdominal examination showed diffuse right upper quadrant and epigastric tenderness without peritoneal irritation. Laboratory test results included white blood cell count, 14.4 x 10(9)/mm3; hemoglobin, 8.5 g/dL; platelets, 1,483,000; and normal values for lipase, amylase, aspartate transaminase, and alanine transaminase. Evaluation with ultrasonography and vessel Doppler studies showed an occlusive thrombus throughout the portal and splenic veins. The patient underwent intravenous heparin anticoagulation therapy. Her symptoms resolved completely over the next 2 days. The patient is currently receiving warfarin and anagrelide as an outpatient (international normalized ratio, 2). There were no long-term complications caused by portal vein thrombosis. This is the first reported case of portal vein thrombosis after laparoscopic splenectomy in the pediatric population.
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2/10. Percutaneous splenic embolization of the splenic artery in the treatment of hypersplenism.

    Percutaneous splenectomy was performed in 6 patients with hypersplenism. Peripheral blood cell counts improved in 5 of the patients. The authors review the indications, technique and complications of percutaneous splenic embolization. It is concluded that medical splenectomy is an effective method particularly to alleviate symptoms of hypersplenism.
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keywords = blood cell
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3/10. T-cell-rich B-cell lymphoma of the spleen presenting with severe hypersplenism.

    We report a 19-year-old woman who was presented with B-symptoms, massive splenomegaly, hepatomegaly and hypersplenism. She underwent diagnostic/therapeutic splenectomy. Microscopically, the spleen showed a vaguely micronodular and diffuse proliferation of lymphoid cells in the white pulp that also involved the red pulp. On immunohistochemical staining, this proliferation consisted predominantly of CD3( ), CD7( ) small T cells with the presence of a minor population of CD15(-),CD30(-), CD20( ) large atypical B cells. A liver biopsy also showed a similar morphology to that seen in the spleen. After splenectomy, only the pancytopenia improved. A combined immunochemotherapy regimen (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) was utilized, which resulted in a complete remission.
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keywords = white
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4/10. Skeletal manifestations in beta-thalassaemia intermedia.

    Thalassaemia intermedia with distressing complications is reported from india. An 11 year old boy presented with recurrent fractures since 6 years of age. Investigations revealed that he had multiple healed fractures of long bones, hypersplenism, iron overload and a red blood cell half life (t1/2) of 17 1/2 days. splenectomy was planned.
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keywords = blood cell
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5/10. spleen alterations in hairy cell leukemia: a scanning electron microscopic study.

    In Hairy Cell leukemia (HCL) peripheral blood and bone marrow cells show under the scanning electron microscope (SEM) a characteristic surface with numerous ruffles and microvilli. The spleen of a patient affected by HCL was studied by SEM after fresh sectioning and routine preparation. Cells with the typical "hairy" surface were observed infiltrating the red pulp, altering the normal reticular meshwork and causing red blood cell distortion. In the sinuses, hairy cells adhered to the endothelial cells causing sinus dilatation and destruction. Aggregates of hairy cells delimiting pooled erythrocytes were also observed and may represent the "pseudosinuses" described in previous light and transmission electron microscopic studies. These preliminary findings may explain the condition of hypersplenism which characterizes HCL. In addition, SEM is proposed as a rapid and simple method to identify HCL spleen involvement.
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keywords = blood cell
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6/10. Splenic pseudocyst associated with hypersplenism.

    A patient with hypersplenism, who was found to have a splenic pseudocyst containing an organized hematoma, is described. There are only two patients with splenic pseudocyst and hypersplenism and an additional two patients with splenic cysts and hypersplenism reported in the world literature. The hypersplenism associated with splenic cysts and pseudocysts is explained on the basis of an expansion of the plasma volume and the total blood volume, an increased destruction of red blood cells and a pooling of blood in the enlarged spleen. The combined use of ultrasonography and computerized tomography has increased the accuracy of noninvasive diagnosis and made more invasive examinations unnecessary. When the ultrasound is technically unsuccessful or when it shows a mixed echo pattern, one should resort to computerized tomography with which it is possible, almost invariably, to differentiate between cysts and neoplasms.
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keywords = blood cell
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7/10. Disseminated BCG infection in severe combined immunodeficiency presenting with severe anaemia and associated with gross hypersplenism after bone marrow transplantation.

    An infant with severe combined immunodeficiency (SCID) is described, who presented with severe anaemia and hepatosplenomegaly due to disseminated bacillus Calmette-Guerin (BCG) infection involving the bone marrow, liver and spleen. After BMT, huge splenic enlargement occurred, presumably due to proliferation of engrafted donor lymphocytes, leading to severe hypersplenism. Peripheral blood cell consumption was resolved by splenectomy, but gradual loss of the marrow graft followed.
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keywords = blood cell
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8/10. Laparoscopic splenectomy in a Jehovah's Witness with profound anemia.

    Open surgery in a severely anemic patient may be complicated by a substantial blood loss from a large incision and subsequent poor wound healing secondary to the anemia. We report our success in performing a splenectomy laparoscopically in a profoundly anemic patient. A 50-year-old white male Jehovah's Witness who was hiv positive was referred for splenectomy after he developed profound, worsening anemia secondary to hypersplenism that was refractory to medical management. His preoperative hemoglobin and hematocrit levels were 2.7 g/dl and 8.8%, respectively, but his religious beliefs precluded transfusion. A laparoscopic splenectomy by the posterior gastric approach was performed. The patient tolerated the surgery well and experienced no additional morbidity. On postoperative day 7, his hemoglobin and hematocrit were 6.8 g/dl and 22%, respectively. We conclude that laparoscopic splenectomy is an attractive procedure in a severely anemic patient who requires splenectomy and refuses blood transfusion.
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9/10. Persistent hypersplenism early after liver transplant: the role of splenectomy.

    BACKGROUND: Transient thrombocytopenia is common after liver transplantation, but persisting thrombocytopenia worsens the prognosis after transplant. methods: Two patients underwent splenectomy for persistent thrombocytopenia early after liver transplantation. The first patient had a platelet count of 17,000/mm3 on postoperative day (POD) 6; her hemoglobin and white blood cell counts were normal. work-ups including bone marrow aspiration, coombs test, and antiplatelet antibody test were negative. On POD 9, she had abdominal bleeding with a platelet count of 17,000/mm3 despite repeated platelet transfusions, and splenectomy was done. The second patient had a platelet count of 3000/mm3 on POD 14, white blood cell was 1600/mm3, and hemoglobin was 7.7 g/dl. Bone marrow biopsy revealed hypercellular marrow. Because his platelet count remained at 2000/mm3 despite empiric treatment with intravenous immune globulin and methylprednisolone, splenectomy was performed. RESULTS: The first patient's platelet count rose to 155,000/mm3 by POD 8. The second patient's platelet count reached 210,000/mm3 on POD 5. Neither patient has had an episode of thrombocytopenia at 36 and 32 months after splenectomy. CONCLUSIONS: splenectomy can be used after liver transplantation for severe, persistent thrombocytopenic states that cannot be attributed to sepsis, intravascular coagulation, immunological causes, or drug effects.
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keywords = blood cell, white
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10/10. Juvenile rheumatoid arthritis and common variable hypogammaglobulinemia.

    We describe a 9-year-old white boy with systemic juvenile rheumatoid arthritis (JRA) who developed pancytopenia and hypersplenism at the age of 13 years. He underwent splenectomy and 3 years later he developed Coombs' positive hemolytic anemia, alopecia, juvenile warts, and multiple bacterial infections. At that time, investigations were compatible with severe hypogammaglobulinemia associated with common variable immunodeficiency. Concomitantly with this condition he experienced complete remission of his inflammatory arthritis. Immunologic studies of B and T lymphocyte function showed that the number of circulating T and B lymphocytes were normal, while T cell function was depressed, as evidenced by markedly reduced proliferative responses to mitogens and antigens, and ability to mediate B cell help. In addition, his circulating B cells were unable to secrete IgM or IgG. He also exhibited anergy to intradermal challenge with a battery of common antigens. The literature dealing with this clinical association is reviewed, and possible immunologic mechanisms involved are discussed.
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keywords = white
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