1/15. Splenic vascular malformations and portal hypertension in hereditary hemorrhagic telangiectasia: sonographic findings.Hereditary hemorrhagic telangiectasia, or Osler-Rendu-Weber disease, is an autosomal dominant disorder in which a variety of vascular dysplasias occur throughout the organ systems. We report the gray-scale and color Doppler sonographic findings in a case of hereditary hemorrhagic telangiectasia. Gray-scale sonographic examination revealed massive splenomegaly, multiple dilated intrasplenic vascular structures (some with adjacent punctate calcifications), an aneurysmal dilatation of the splenic vein, dilated intrahepatic portal branches, and marked atrophy of the right hepatic lobe. color Doppler sonography showed dilatation of the truncus coeliacus and high-velocity flow in the splenic artery. There were significant aliasing in the splenic hilum and an abnormal, arterialized flow in intrasplenic branches of the splenic vein. The splenic vein was massively enlarged with increased flow velocity and contained an isolated aneurysmal dilatation in the hilum. There were multiple serpiginous retroperitoneal collateral vessels, and the left gastric vein was dilated with hepatofugal flow. The patient had portal hypertension that developed secondary to the increased portal flow.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/15. Littoral cell angioma as a rare cause of splenomegaly.A 58-year-old, otherwise healthy man presented with a sudden onset of watery diarrhea. A pseudomembranous colitis due to antibiotics was identified as the cause of the diarrhea. Enlargement of the spleen was detected during the evaluation. The enlarged, plump spleen (20 cm long, 7.1 cm wide) had multiple nodules that differed in size from 1 to 8 cm. Neither clinical nor other symptoms of an underlying malignant disease could be detected. Because the signs were of little diagnostic value we arranged a splenectomy, which showed a littoral cell angioma (LCA) to be the cause of splenomegaly. In addition to the case report, we have reviewed the literature, clinical manifestations, differential diagnosis, special gross and microscopic pathological findings, and the location of this benign vessel tumor in the pathology of the spleen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/15. Marked splenomegaly in fetal cytomegalovirus infection: detection supported by three-dimensional power Doppler ultrasound.An enlarged fetal spleen can be associated with fetal infection, anemia and different syndromes but its prenatal diagnosis is rare. We report on a diagnosis of splenomegaly at 32 weeks' gestation in a fetus which was found to be affected by cytomegalovirus infection. An enlarged spleen was suspected when the stomach was found to be displaced anteriorly and medially and the diagnosis was supported on visualization of the splenic vessels by color and three-dimensional power Doppler ultrasound. The patient had been referred because of fetal growth restriction and intracerebral anomalies and the additional finding of splenomegaly was highly suspicious for cytomegalovirus infection. This was confirmed by positive maternal serology and by neonatal virus excretion in urine. Retrospectively, examination of stored blood samples from 9 and 23 weeks' gestation revealed an early cytomegalovirus infection. Antenatal and neonatal magnetic resonance imaging examinations showed microcephaly, lissencephaly and the presence of microcalcifications. At the age of 9 months, the child suffers from severe neurological impairment and blindness due to severe optical atrophy. This case emphasizes that color Doppler and three-dimensional power Doppler ultrasound can facilitate the antenatal diagnosis of splenomegaly and can help to delineate the spleen from the similar-looking neighboring liver.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/15. Subtotal splenectomy for treatment of retarted growth and sexual development associated with splenomegaly.This communication presents a new alternative for the treatment of retarded growth and sexual development associated with spleno- megaly: subtotal splenectomy, preserving the upper splenic pole supplied only by the splenogastric vessels, to avoid adverse effects of total splenectomy. We performed this procedure associated with central splenorenal shunt or portal-variceal disconnection in 3 teenagers with portal hypertension due to Schistosomia-sis Mansoni, complicated by variceal bleedings. All of them presented retarded growth and sexual development. All patients had uneventful postoperative follow-up, and normal growth and sexual development after the surgery. Subtotal splenectomy should be considered for treatment dwarfism associated with splenomegaly.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/15. Isolated diffuse hemangiomatosis of the spleen: case report and review of literature.Small localized hemangiomas are common neoplasms of the spleen. Isolated diffuse splenic hemangiomatosis, however, is very rare. This lesion can be accompanied by severe hypersplenism and other complications. We report on a case with significant splenomegaly caused by diffuse hemangiomatosis, which was an incidental finding without any clinical disorders. After splenectomy, the normal parenchyma was found to be widely replaced by multiple spongy nodules. Histologically, cavernous vessels were distributed throughout the whole organ, with endothelial cells expressing vimentin, factor viii and CD 31, but not CD8. Splenic sinus lining cells exhibited a strongly positive reaction with CD8, which became faint and disrupted in highly dilated sinuses in the vicinity of cavernous vessels. In some areas, there seemed to be a gradual transition from cystically dilated splenic sinuses to cavernous vessels. The differential diagnosis must consider other splenic vascular tumors, such as littoral cell angioma, lymphangioma, peliosis of the spleen, and hamartoma. The pathogenesis of diffuse splenic hemangiomatosis is controversial, and a malformative or neoplastic origin is under debate. A derivation from splenic sinusoidal cells was suggested by some authors, but was rejected by others. Our findings cannot exclude a neoplastic origin from splenic sinuses but, finally, the etiology and pathogenesis of this vascular lesion remain uncertain.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
6/15. Usefulness of bone marrow aspiration for definite diagnosis of Asian variant of intravascular lymphoma: four autopsied cases.The Asian variant of intravascular lymphoma (AIVL) is characterized by hemophagocytic syndrome, pancytopenia and hepatosplenomegaly but usually lacks any neurological abnormality and skin lesions, which are typical features of classical intravascular lymphoma (IVL). An ante-mortem diagnosis of AIVL is difficult due to the absence of visible lymphoma lesions and unspecific clinical manifestations. A definite diagnosis relies on the presence of neoplastic B cells in the lumina of small vessels. paraffin block samples of aspirated bone marrow clots were obtained from 4 patients with clinically suspected IVL and subjected to immunohistopathological analysis. All samples exhibited CD 20 or CD 79a lymphoma cells proliferating intravascularly as well as erythrocytic hemophagocytosis. The distribution of neoplastic cells in the structure of the bone marrow allowed IVL to be distinguished from bone marrow invasions due to other types of lymphoma. We demonstrated the successful establishment of a definite ante-mortem diagnosis of AIVL in 3 of 4 patients by the rapid and simple method of using aspirated bone marrow samples.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/15. Ninety-five percent splenectomy for massive splenomegaly: a new surgical approach.Subtotal splenectomy for children with Gaucher's disease has been a major contribution. When spleens of massive size are mobilized for partial resection, it may be technically difficult to remove more than 80% to 85% and still maintain hilar blood supply. The short gastric vessels are enlarged in patients who have marked splenomegaly and provide sufficient vascularity to support the 5% of remaining spleen that is desired in these patients. When the upper pole of the spleen is retained, as in the present case, the cut surface is relatively small and hemostasis is technically easier to achieve than when resection is performed closer to the center of the spleen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/15. immunoblastic lymphadenopathy. A report of two cases.Two patients with immunoblastic lymphadenopathy had lymphadenopathy, hepatosplenomegaly, rash, dysproteinemia, constitutional symptoms, and typical morphologic findings or lymph node obliteration by immunoblasts and plasma cells, proliferation of aborizing vessels, and infiltration with amorphous, eosinophilic material. One patient had massive pulmonary infiltrates that responded to steroid therapy early in the course of the disease, but recurred and were found, at autopsy, to represent immunoblastic invasion of the lung. In both cases, a severe peripheral neuropathy developed during the course of the disease. One patient was addicted to dextroamphetamine for 20 years. Response to corticosteroids and cytotoxic therapy was initially rapid and complete, but refractoriness developed as seen in postmortem findings of extensive visceral involvement.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/15. Systemic angioendotheliomatosis presenting with hemolytic anemia.Two patients with systemic angioendotheliomatosis had prominent constitutional symptoms such as fever, loss of weight, and general weakness, and had multiple organ dysfunctions, including bizarre neurologic findings and dementia. Severe anemia that required frequent blood transfusions also was present. One patient developed severe hemolysis and hypersplenism that required splenectomy for relief; the other patient had intravascular hemolysis and autoimmune hemolytic anemia, which were treated unsuccessfully with conservative measures. In both cases, postmortem examination showed many large, noncohesive malignant cells within the lumen of the blood vessels in many of the organs. There was no infiltration or replacement of the normal tissues by the tumor cells. Histochemical studies showed that the tumor cells were pyroninophilic but did not have cytoplasmic immunoglobulins or activity of chloroacetate esterase and muramidase. The cells showed factor viii antigen in their cytoplasm. Systemic angioendotheliomatosis may represent a true neoplastic process of the vascular endothelial cells.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/15. vasculitis in cytomegalovirus infection.A 7-year-old girl had fever, arthralgia, and a mild cutaneous vaculitis with papules, nodules, and livedo. A biopsy specimen of a papule showed lymphocytic small-vessel vasculitis, with some atypical lymphocytic nuclei. splenomegaly developed, and results of subsequent studies supported a diagnosis of cytomegalovirus (CMV) infection, with atypical peripheral blood lymphocytes and a characteristic pattern of complement-fixing antibodies to CMV antigen. The CMV mononucleosis syndrome is rarely reported in children, and the cutaneous manifestations are usually rubelliform. Distinctive cutaneous pathologic characteristics have been described previously only in neonates ("blueberry muffin" syndrome) and in immunosuppressed patients (viral inclusion bodies in endothelial cells).- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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