Cases reported "Splenomegaly"

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1/9. An unusual form of persistent polyclonal B lymphocytosis in an infant.

    We report a case of persistent polyclonal lymphocytosis in an infant. The circulating lymphocytes were of a small to medium size and a small proportion were larger and had lymphoplasmacytoid features. The presence of either an infectious or mutagenic agent was excluded. The polyclonal B-cell nature of the lymphocyte was demonstrated by immunological markers and confirmed by Southern blot analysis and by polymerase chain reaction targeting immunoglobulin genes. In contrast to the common form of polyclonal lymphocytosis, this case was not associated with HLA-DR7 and/or abnormalities of chromosome 3, p53 or Bcl2/IgH. Whether this lymphocytosis represents a premalignant or a benign condition remains uncertain, although there has been no progression to date.
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2/9. T-cell-rich B-cell lymphoma presenting in the spleen: a clinicopathologic analysis of 3 cases.

    We review the clinical, pathologic, and molecular genetic features of 3 splenic T-cell-rich B-cell lymphomas and discuss their differential diagnosis. All patients presented with symptomatic splenomegaly and underwent diagnostic/therapeutic splenectomy. Microscopically, the spleen in all cases showed a micronodular proliferation of lymphoid cells. A proportion of the nodules demonstrated central hyalinization or sclerosis. There was also an exuberant extramedullary hematopoiesis. On immunohistochemical stain, the nodules consisted predominantly of small T cells with scattered large atypical B cells. The clonal nature of the atypical B cells was confirmed by polymerase chain reaction assays for immunoglobulin heavy-chain gene rearrangement. In the H&E sections, the differential diagnoses included Hodgkin's lymphoma, follicular lymphoma, peripheral T-cell lymphoma, and nonneoplastic granulomatous process. The presence of exuberant extramedullary hematopoiesis also raised the possibility of a chronic myeloproliferative disorder. The combined morphologic, immunohistochemical, and molecular genetic data are essential for a correct diagnosis of splenic T-cell-rich B-cell lymphoma.
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3/9. Diffuse hemangiomatosis of the spleen: splenic hemangiomatosis presenting with giant splenomegaly, anemia, and thrombocytopenia.

    In an elderly patient with oligosymptomatic giant splenomegaly, clinical and laboratory data were nondiagnostic, while nonhomogeneous splenic enlargement was the only finding detected by imaging procedures. splenectomy was performed and diffuse hemangiomatosis of predominantly capillary-type found. The failure of imaging techniques to even hint at the nature of the underlying disorder is comprehensible in view of the organ being essentially replaced in toto by the abnormal vascular channels. Diffuse splenic hemangiomatosis, a rare condition, may cause hypersplenism, and its diagnosis may be elusive because of misleading patterns on imaging.
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4/9. splenic rupture and infectious mononucleosis.

    infectious mononucleosis is an acute viral illness associated with a high incidence of splenomegaly, although the incidence of splenic rupture is low. When rupture occurs, the mortality has been significant, presumably, because a history of trauma is not present. The spleen may be vulnerable to injury due to the histopathologic changes that occur as a result of this illness. Essentially all patients with spontaneous rupture related to infectious mononucleosis have epigastric or upper abdominal pain. The diagnosis of splenic rupture may be confirmed in a variety of ways. In those patients who are hemodynamically stable, CT scan, ultrasound, or radionuclide scan may aid in establishing the diagnosis. Selective splenic angiography is very accurate but has been largely abandoned because of the invasive nature of the study. peritoneal lavage is efficacious in establishing the diagnosis in hemodynamically unstable patients. The treatment of choice, at this time, is splenectomy. Current interest in splenic salvage has resulted in reports of nonoperative therapy in stable patients and splenorrhaphy in one instance. Due to the extent of the histologic changes in the spleen, caution is urged in electing the conservative approach to this clinical situation.
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5/9. Splenic immune deposits in bacterial endocarditis.

    Splenic and renal tissues from a 61-year-old man with subacute bacterial endocarditis and acute renal failure were studied. Immune complex deposits were found both within glomeruli and splenic venous sinus basement membranes, substantiating the systemic nature of the immune injury in this disorder. The splenic deposits may, in part, be responsible for the splenomegaly often present in endocarditis.
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6/9. Splenic compression of the left kidney simulating pathologic unilateral renal enlargement.

    A case in which pathologic enlargement of the left kidney was simulated by splenic compression in a 17-year-old boy with hemoglobin sc disease and recurrent left flank pain is presented. The true nature of the apparent enlargement was demonstrated by computed tomography.
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7/9. Symptomatic splenic hemartoma: a report of two cases and review of the literature.

    Two cases of large, multiple splenic hamartomas in children with pancytopenia, bone marrow hyperplasia, lymphadenopathy, hepatosplenomegaly, frequent infections, growth retardation, and fever are reported. These symptoms were relieved by splenectomy, and have not recurred during follow-up periods of one year and nine years. The sharply circumscribed lesions comprised large portions of the resected spleens and were composed of dilated vascular channels filled with mononuclear cells and iummunoblasts. The lesions lacked splenic cords or trabeculae, lymphoid follicles, reed-sternberg cells, and granulomas or other evidence of infection. Splenic hamartomas are usually single small lesions found incidentally at necropsy or laparotomy. Splenic hamartomas associated with symptoms and hypersplenism are large, and often confluent multiple tumors. Recognition of their benign nature is important in light of the current practice of laparotomy for staging and diagnosis of malignant conditions.
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8/9. Reversible functional asplenia. Autoimmune hemolytic anemia with thrombocytopenia in chronic lymphocytic leukemia.

    An enlarged spleen failed to accumulate Tc-99m-labeled phytate, but it showed normal perfusion and uptake of labeled and heat-denatured red blood cells. A blood cell count demonstrated moderately low hemoglobin and hematocrit levels and a markedly low platelet count, and results of direct Coombs' and antiplatelet-antibody (IgG) tests were positive. Steroid therapy resulted in normal splenic function. This case demonstrates reversible functional asplenia caused by reticuloendothelial dysfunction secondary to pure splenic chronic lymphocytic leukemia.
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9/9. Leukemic meningitis in a patient with splenic lymphoma with villous lymphocytes (SLVL). meningitis as a possible initial manifestation of SLVL.

    BACKGROUND. Splenic lymphoma with villous lymphocytes (SLVL) is a low grade, non-Hodgkin's lymphoma with a stable or slowly progressive clinical course. To the authors' knowledge, central nervous system involvement has not been described previously in patients with SLVL. methods. Morphologic, immunocytochemical, and immunohistochemical analyses were conducted to determine the nature of villous lymphocytes in the peripheral blood, spleen, and cerebrospinal fluid (CSF) of a patient with massive splenomegaly. RESULTS. A diagnosis of SLVL was made, based on tartrate-resistant acid phosphatase-negative peripheral villous lymphocytosis with CD19 , CD20 , HLA-DR phenotypes, and the involvement of spleen white pulp with these cells. Mononuclear cells in the CSF showed the same morphologic and immunocytochemical features seen in the villous lymphocytes in the peripheral blood and spleen. splenectomy and intrathecal chemotherapy were successful in clearing leukemic cells from the CSF. CONCLUSION. In this patient with SLVL in whom leukemic meningitis developed, meningitis was found to be a possible initial manifestation of SLVL.
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