No abstract available.
Fibrosis* ; Hypersplenism*

No abstract available.
Estrogens, Conjugated (USP)* ; Hypersplenism*

BACKGROUND AND OBJECTIVES: Niemann Pick A disease causes a progressive accumulation of sphyngomyelin in several organs and the survival of the patients is usually limited to three years. We describe the outcome of a patient suffering from Niemann Pick A disease, who first underwent an haploidentical bone marrow transplantation, and then intrathecal and I.V injections of mesenchymal cells. METHODS AND RESULTS: While the outcome of bone marrow transplantation was a complete failure, one month after the treatment with the mesenchymal cells the patient improved from the psychomotor and the parenchymal storage perspective. When hypersplenism was solved platelets rose quickly from 20,000 to 120,000/microliter. CONCLUSIONS: Therefore cellular therapy should be considered as a possible choice of treatment of NPA disease.
Bone Marrow Transplantation ; Humans ; Hypersplenism ; Niemann-Pick Diseases* ; Stem Cells*

Leukemic reticuloendotheliosis (L.R.E.) was first described as a clinical and pathological entity by Ewald in 1923. LRE is a rare neoplastic disease of the hematopoietic system that is characterized cliniclly by chronic course with an insidious onset. marked splenomegaly with absence of substantial lymphadenopathy and predominant in male and characterized histologically by the presence of circulating abnormal mononuclear cells with many cytoplasmic projections, which have been refered to ??airy cells frequently. Splenectomy appeared to be the most beneficial treatment at present for those patients with massive splenomegaly and hypersplenism and chemotherapy is of little benefit in the treatment in LRE. Recently, the authors had the opportunity to observe a two year and eight old male child at this hospital whose clinical course and pathologid features were consistent with LRE. Splenectomy is performed and discharged with relatively good conditions.
Child ; Cytoplasm ; Drug Therapy ; Hematopoietic System ; Humans ; Hypersplenism ; Leukemia, Hairy Cell* ; Lymphatic Diseases ; Male ; Splenectomy ; Splenomegaly

Hamartoma of the spleen is a rare benign lesion and the pathogenesis of this is not completely understood. It is usually incidentally discovered during an operation or autopsy. Since the first report in 1861 by Rokitansky, less than 150 cases of splenic hamartoma have been documented in the literature to date. The majority of the patients with this tumor are asymptomatic. However, a few of these lesions are associated with clinical features such as hypersplenism, cirrhosis of the liver and spontaneous splenic rupture. Because there are no specific symptoms, tumor markers or radiologic results, it is usually difficult to make a diagnosis before surgery and the specimen pathology. We report here on three cases of splenic hamartoma (a 33-year-old man associated with thrombocytopenia, a 29-year-old woman with a 6-month history of left upper quadrant abdominal pain and a 43-year-old man with synchronous hepatocellular carcinoma) and we review the relevant literature.
Abdominal Pain ; Adult ; Autopsy ; Female ; Fibrosis ; Hamartoma ; Humans ; Hypersplenism ; Liver ; Spleen ; Splenic Rupture ; Thrombocytopenia ; Biomarkers, Tumor

Idiopathic portal hypertension (IPH) is a rare disorder which is clinically characterized by portal hypertension, splenomegaly, hypersplenism and the absence of liver cirrhosis. Patients with IPH have massive splenomegaly leading to increased portal venous flow and subsequent portal hypertension. In selected IPH patients with splenomegaly and hypersplenism, splenectomy can be regarded as an effective treatment protocol for decreasing portal hypertension. We report a case of a 44-year-old woman who was diagnosed with IPH accompanied by splenomegaly and hypersplenism. She underwent laparoscopic splenectomy and clinical symptoms and hypersplenism resolved. Our study shows that laparoscopic splenectomy can be considered as a procedure for treating patients with splenomegaly and hypersplenism due to IPH.
Adult ; Clinical Protocols ; Female ; Humans ; Hypersplenism* ; Hypertension, Portal* ; Liver Cirrhosis ; Splenectomy* ; Splenomegaly*

Malaria is a protozoan disease transmitted by the bite of infected mosquitoes. During acute and chronic malarial infection, altered splenic structure and function produce asymptomatic enlargement or com plications such as hematoma formation, rupture, hypersplenism, or cyst formation. Spontaneous splenic rupture occurs during acute infection, usually during the primary attack. However, rupture occurring in chronic malarial splenomegaly is seldom spontaneous; obvious trauma is almost always the precipitating event. Recently, conservative therapy has been applied in all cases of malarial splenic rupture. Surgical therapy should be reserved for patients with severe rupture or for those with continued or recurrent bleeding. We report the case of a recently treated 25-year-old male who had acute malaria complicated by fever and by severe abdominal pain, tenderness, and distension, a condition that may result in splenic rupture. At operation, the spleen was slightly enlarged and multiple tearing was found in the lateral surface of the capsule. A splenectomy was preformed.
Abdominal Pain ; Adult ; Culicidae ; Fever ; Hematoma ; Hemorrhage ; Humans ; Hypersplenism ; Malaria ; Male ; Rupture ; Rupture, Spontaneous* ; Spleen* ; Splenectomy ; Splenic Rupture ; Splenomegaly

Malaria is a protozoan disease transmitted by the bite of infected mosquitoes. During acute and chronic malarial infection, altered splenic structure and function produce asymptomatic enlargement or com plications such as hematoma formation, rupture, hypersplenism, or cyst formation. Spontaneous splenic rupture occurs during acute infection, usually during the primary attack. However, rupture occurring in chronic malarial splenomegaly is seldom spontaneous; obvious trauma is almost always the precipitating event. Recently, conservative therapy has been applied in all cases of malarial splenic rupture. Surgical therapy should be reserved for patients with severe rupture or for those with continued or recurrent bleeding. We report the case of a recently treated 25-year-old male who had acute malaria complicated by fever and by severe abdominal pain, tenderness, and distension, a condition that may result in splenic rupture. At operation, the spleen was slightly enlarged and multiple tearing was found in the lateral surface of the capsule. A splenectomy was preformed.
Abdominal Pain ; Adult ; Culicidae ; Fever ; Hematoma ; Hemorrhage ; Humans ; Hypersplenism ; Malaria ; Male ; Rupture ; Rupture, Spontaneous* ; Spleen* ; Splenectomy ; Splenic Rupture ; Splenomegaly

BACKGROUND: Liver cirrhosis causes secondary hypersplenism and thrombocytopenia is clinically troublesome. Splenectomy (SPL) was thought to be the curative management for correcting thrombocytopenia. However, decompensated liver function prevents any surgical approach due to high morbidity and mortality. Hence, partial splenic embolization (PSE) has been introduced, which is a less invasive procedure. The purpose of this study was to assess the long-term therapeutic effects of PSE and to compare them with those of SPL. METHODS: This study was performed retrospectively in patients who underwent PSE or SPL from Jan. 1999 to Dec. 2003. The patients either had symptoms of bleeding or they needed to correct their thrombocytopenia for further treatment of associated diseases. The therapeutic effects were evaluated, and the complications were assessed. RESULTS: Forty and 35 patients were enrolled in the PSE and SPL groups, respectively. WBC, platelet and hemoglobin counts were all significantly increased at the 2 year follow-up in both groups. Child-Pugh score significantly decreased in the PSE group from 6.5 before treatment to 5.5 after treatment (p=0.004). Minor complications were easily controlled with supportive care, and major complications very rarely occurred in both groups. CONCLUSIONS: PSE and SPL both proved to be effective measures with few serious complications for treating pancytopenia in patients with liver cirrhosis. Considering the improved liver function (the prothrombin time) and the Child-Pugh score after PSE, it may be more reasonable to initially recommend PSE for the patients with liver cirrhosis and secondary hypersplenism.
Blood Platelets ; Embolization, Therapeutic ; Follow-Up Studies ; Hemorrhage ; Humans ; Hypersplenism* ; Liver ; Liver Cirrhosis ; Mortality ; Pancytopenia ; Prothrombin ; Retrospective Studies ; Splenectomy ; Thrombocytopenia

Gaucher's disease is an autosomal recessive genetic disorder of lipid metabolism. A deficiency of beta-glucocerebrosidase causes an accumulation of glucocerebroside in the reticulo-endothelial system and bone marrow. Total or partial splenectomy has been used in case of massive splenomegaly with hypersplenism and/or mechanical pressure symtoms. Partial splenectomy is preferred to prevent susceptibility to overwhelming postsplenectomy sepsis and to delay the massive deposition of glucocerebroside in the liver and bones. We report the case of a 20-year-old woman with Gaucher's disease and who had a splenic embolization 4 years ago. The spleen cross the midline of the abdomen reached to the true pelvis and elevated the left diaphragm. Angiotensin-converting enzyme, acid phophatase and ESR were increased but beta-glucocerebrosidase was normal. Osteosclerotic changes of the distal femur was observed. Hepatomegaly and splenomegaly with mutiple accessory spleens were seen on abdominal CT. On isotope scan for liver and spleen, multiple accessory spleens had isotope uptake, but spleen did not. We noted severe adhesion of spleen to neighboring structure and no viable splenic tissue for preservation. Total splenectomy with preservation of four accessory spleens was performed. We needed multiple transfusion during dissection and bleeding was continuous for 3 days postoperatively. The patient was discharged without problems on the postoperative 15 th day.
Abdomen ; Bone Marrow ; Diaphragm ; Female ; Femur ; Gaucher Disease* ; Glucosylceramidase ; Hemorrhage ; Hepatomegaly ; Humans ; Hypersplenism ; Lesser Pelvis ; Lipid Metabolism ; Liver ; Sepsis ; Spleen* ; Splenectomy* ; Splenomegaly ; Tomography, X-Ray Computed ; Young Adult