Cases reported "Splenomegaly"

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1/14. Recurrent infectious mononucleosis caused by Epstein-Barr virus with persistent splenomegaly.

    We present the clinical case of a 20-year-old male soldier who appeared in general good physical condition. He suffered from infectious mononucleosis caused by Epstein-Barr virus that had recurred 2 years after the first serologically documented episode. The detected splenomegaly persisted in the healthy young man, who otherwise showed no apparent immune deficiency. To our knowledge, this is an extremely rare condition.
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2/14. New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.

    Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis b. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.
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keywords = physical examination, physical
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3/14. Portal hypertension develops in a subset of children with standard risk acute lymphoblastic leukemia treated with oral 6-thioguanine during maintenance therapy.

    BACKGROUND: 6-thioguanine (TG) was recently studied to determine whether TG in maintenance therapy achieves better event free survival than 6-mercaptopurine (MP) for standard risk acute lymphoblastic leukemia (ALL) on the clinical trial, CCG-1952 (5/1996-1/2000). Veno-occlusive disease was previously recognized as a complication of TG on CCG-1952. We report a newly recognized pediatric complication of TG: splenomegaly and portal hypertension (PH) developing during maintenance or after completion of therapy. PROCEDURE: Twelve patients (3-10 years) had been randomized to receive a targeted dose of 50 mg/m(2)/day of TG during maintenance phases. Actual TG dose ranged from 25 to 77 mg/m(2)/day (median 34 mg/m(2)/day). RESULTS: The initial patient, a boy who had marked thrombocytopenia and intermittent splenomegaly during maintenance therapy, was evaluated for persistent pancytopenia and progressive splenomegaly 3 months after completion of therapy. Dilated splenic vein and collaterals consistent with PH were documented by MRI/MRA. Esophagogastroduodenoscopy found esophageal varices. Liver biopsy showed periportal fibrosis and marked dilatation of veins and venules. Of the other 12 patients, 9 patients studied had abnormal MRI/MRAs with evidence of varices in 4. Eight patients had splenomegaly on physical examination. Liver biopsies in a girl after 3.3 courses of TG and a boy after 4.6 courses of TG showed periportal fibrosis and dilatation of venules and sinusoids and minimal focal fatty changes. Subsequent MRI/MRAs have been stable or improved. CONCLUSIONS: The evaluations of these 12 patients suggest that treatment with TG causes injury to the liver leading to PH and that thrombocytopenia and splenomegaly are clinical hallmarks of this toxicity.
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4/14. Fibrolamellar carcinoma of the liver--a case report.

    A 36-year-old woman was admitted to our hospital because of general fatigue. The physical and laboratory findings on admission revealed splenomegaly, pancytopenia, hypocoagulopathy, liver hypofunction with a hepaplastin test of 55% and ICG Rmax of 0.6 mg/kg/min. diagnostic imaging showed a hypoechoic mass 1.5 in diameter a low density area on the CT scan and a faint tumor stain on the AAG in the posterior inferior area of the liver. On a diagnosis of hepatocellular carcinoma with liver cirrhosis and hypersplenism, partial hepatectomy and splenectomy were performed. The resected hepatic specimen revealed a small liver cancer of 1.9 x 1.5 x 1.3 cm with liver cirrhosis. The specimen consisted of a firm rubbery mass. Macroscopically, the tumor appeared oval and was lobulated with a thin capsule. A fibrous scar was observed in the central area. Microscopically, malignant hepatocytes showed various shapes, ranging from polygonal to spindle form, with eosinophilic granular cytoplasm and were surrounded by abundant fibrous stroma. Orcein stain, revealed that these malignant hepatocytes contained many black granules of copper-binding protein. Immunoperoxidase staining for alpha 1-antitrypsin was also positive in the malignant hepatocytes. However, within this lamellar fibrous regions, there were many cords of tumor cells in which nucleoli were absent and abortive biliary differentiation was suggested. Consequently this tumor was diagnosed as an atypical fibrolamellar hepatocellular carcinoma. We think that this case is the 3rd case reported in japan and the 2nd case in a Japanese person.
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5/14. Ectopic splenomegaly in Felty's syndrome.

    A 50-year-old woman with Felty's syndrome, who presented with "menopausal" symptoms, was found to have a large pelvic mass on physical exam. Computed tomography of the pelvis led to an incorrect diagnosis of malignancy, while radionuclide imaging using Tc-99m sulfur colloid confirmed the diagnosis of ectopic splenomegaly.
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6/14. Development of polycythaemia vera in a patient with myelofibrosis.

    In March 1981, a 53-year-old man presented with itching and was diagnosed as having myelofibrosis. There was gradual enlargement of the spleen over the following 5 yr. His spleen had to be removed in February 1986 because of physical discomfort. 3 months post-splenectomy he became polycythaemic. bone marrow examination was consistent with severe myelofibrosis. It was possible to demonstrate erythropoietin-independent BFU-E from peripheral blood, and ferrokinetic studies showed that erythropoiesis was localised to the liver with little bone marrow activity. Thus, despite severe marrow fibrosis, liver erythropoiesis was now polycythaemic, suggesting the coexistence of myelofibrosis and polycythaemia vera.
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7/14. Felty's syndrome without splenomegaly.

    A woman with chronic rheumatoid arthritis and severe granulocytopenia but without splenic enlargement by physical examination or radionuclide scanning was studied for granulocyte-bound immunoglobulin g (IgG) and serum antigranulocyte antibodies. Prior to splenectomy 73 to 110 X 10(-14) g/cell of IgG were detected on the patient's granulocytes, a value in the range (20 to 220 X 10(-14) g) found in 16 patients with classic Felty's syndrome. Granulocyte-bound IgG in 21 patients with rheumatoid arthritis without Felty's syndrome was less than 20 X 10(-14) g. Following splenectomy, the patient had a partial correction of her peripheral granulocyte count, and granulocyte bound IgG was repeated less than 20 X 10(-14) g/cell. When paraformaldehyde-fixed granulocytes, obtained either from normal donors or from the patient after splenectomy, were incubated in the patient's serum obtained before splenectomy, more IgG was bound than with control serums from patients with rheumatoid arthritis. Similar results were obtained when serums from patients with classic Felty's syndrome were incubated with paraformaldehyde-fixed granulocytes. Thus, patients with rheumatoid arthritis without overt splenic enlargement may have pathophysiologic Felty's syndrome, and in vitro studies such as these may be used to define this process.
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keywords = physical examination, physical
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8/14. Pseudo-splenomegaly as a result of subphrenic abscess.

    A case of left-sided subphrenic abscess, secondary to perforation of a carcinoma of stomach, is described. The patient presented with a palpable spleen which was shown to be normal in size but displaced by the subphrenic collection. The importance of correct interpretation of this physical sign is discussed.
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9/14. Eosinophilic fasciitis, reactive hepatitis, and splenomegaly.

    Eosinophilic fasciitis (EF) is an acute, idiopathic inflammatory disorder often manifested by tender swelling of the extremities after extreme physical exertion. It is usually without visceral complications. I treated a 25-year-old man with EF who had reactive hepatitis and splenomegaly. To my knowledge, the former has never been reported and the latter only once.
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10/14. Bone marrow imaging with 59Fe.

    Commerically available collimators are not suitable for the visual demonstration of the kinetics of 59Fe, because of its physical properties. A locally designed collimator, that provides integral visual information of the whole body distribution of erythropoietic marrow, is discussed. Wholebody scans of 4 individuals are also included to demonstrate the capabilities of the collimator.
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