Cases reported "Splenic Neoplasms"

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1/228. Pathologic-spontaneous-rupture of the spleen as a presenting sign of splenic T-cell lymphoma--case report with review.

    A 39 year-old man presented for surgery with epigastric pain, tachycardia, hypotension and a progressive decrease of hemoglobin due to blood loss. Immediate abdominal ultrasonography followed by prompt paracentesis revealed massive intraperitoneal hemorrhage. During emergency laparotomy, a linear, actively bleeding rupture of an enlarged spleen was found and splenectomy was performed. The patient survived and the post-operative course was uneventful. Histopathology of the spleen as well as bone marrow biopsy confirmed the diagnosis of T-Cell lymphoma. Chemotherapy was initiated 3 weeks after surgery. To the best of our knowledge, this is the first reported case of previously undiagnosed T-Cell lymphoma presenting as pathologic rupture of the spleen.
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2/228. Splenic lymphoma with villous lymphocytes presenting as leucocytoclastic vasculitis.

    We describe a 69-year-old woman who presented with purpura on the legs. Examination of a blood film revealed a homogenous population of abnormal lymphoid cells with villous projections. The immunophenotype was consistent with a diagnosis of splenic lymphoma with villous lymphocytes (SLVL). A type II (IgM-IgG) cryoglobulin was detected in the serum. Renal biopsy demonstrated a membranoproliferative type I glomerulonephritis with intraluminal IgG and IgM deposits, and a skin biopsy showed a leucocytoclastic vasculitis with outlining of the vessels by IgG and IgM. vasculitis has been reported in association with a number of haematological malignancies, most frequently with hairy cell leukaemia. To our knowledge this is the first report of SLVL presenting with cryoglobulinaemic vasculitis.
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3/228. Primary splenic tuberculosis in a patient with nasal angiocentric lymphoma: mimicking metastatic tumor on abdominal CT.

    Tuberculosis may be difficult to diagnose when it presents in an uncommon extrapulmonary site. The authors report a case of splenic tuberculosis mimicking metastatic tumor on computed tomography in a 60-year-old woman who had been treated with combination chemotherapy for nasal angiocentric lymphoma. Diagnostic splenectomy revealed multiple necrotic masses in the spleen, which were consistent with caseating granulomas microscopically. diagnosis was confirmed by positive cultures in Lowenstein medium, which grew typical mycobacterium tuberculosis organisms. Following splenectomy, the patient was also treated with a triple-drug antituberculosis regimen with no recurrence of her symptoms.
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4/228. Consistency of isochromosome 7q and trisomy 8 in hepatosplenic gammadelta T-cell lymphoma: detection by fluorescence in situ hybridization of a splenic touch-preparation from a pediatric patient.

    Hepatosplenic gamma-delta (gammadelta) T-cell lymphoma is a rare but increasingly recognized lymphoid malignancy predominantly affecting young adult males. It is not well appreciated in the pediatric population. We report the third case of this aggressive lymphoma in a child as well as additional support for the consistency of the recently discovered cytogenetic abnormalities, isochromosome 7q and trisomy 8, which in this case were documented using fluorescence in situ hybridization (FISH) of a touch-preparation of the spleen.
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5/228. "Composite" lymphoma, lymphoplasmacytoid and diffuse large B-cell lymphoma of the spleen: molecular-genetic evidence of a common clonal origin.

    We describe here the first well-characterized case of "composite" lymphoma of the spleen in which the two components were a low-grade and a high-grade B-cell non-Hodgkin's lymphomas. The patient was an elderly man with prominent splenomegaly and multiple hypoechogenic lesions of the spleen. A splenectomy was performed, and the macroscopic and histological findings showed the simultaneous presence of a "low-grade" B-cell lymphoma, lymphoplasmacytoid (immunocytoma) and a "high-grade" B-cell lymphoma (immunoblastic), which were spatially separated. The two lesions expressed the same immunoglobulin light chain (lambda), but the Southern blot analysis showed different patterns of immunoglobulin heavy chain (IgH) clonal rearrangement. PCR analysis followed by direct sequencing of the IgH-amplified rearrangement products provided molecular-genetic evidence that the two components of the composite lymphoma had the same clonal origin. Since both EBV LMP-1 and p53 were negative by immunohistochemistry, it is unlikely that EBV and p53 were involved in the neoplastic progression in this case. PCR analysis and direct sequencing of IgH-amplified rearrangement products are useful tools to investigate clonality in cases in which Southern blot analysis cannot be performed or does not provide conclusive findings.
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6/228. Splenic involvement by blastic mantle cell lymphoma (large cell/anaplastic variant) mimicking splenic marginal zone lymphoma.

    The most cases of splenic marginal zone lymphoma (SMZL) seem to respond favorably to splenectomy. The diagnosis of this lymphoma is mainly based on the recognition of a micronodular pattern of splenic involvement with marginal zone differentiation. However, it is possible to find so-called "marginal zone differentiation" in splenic involvement by other small B-cell lymphomas, particularly mantle cell lymphoma (MCL) and follicular lymphoma. We report a case of blastic MCL, large cell/anaplastic variant with a high level of clinical aggressiveness, showing biphasic cytology and a micronodular pattern which resembles SMZL. A single biopsy corresponding to this case shows two phases of tumoral progression in a MCL, a rare finding in MCL. In conclusion, the differential diagnosis of SMZL must take the possibility of a blastic MCL with biphasic cytology into account, as the case here.
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7/228. The relationship between splenic marginal zone B-cell lymphoma and chronic liver disease associated with hepatitis c virus infection.

    An etiologically important role has been suggested for hepatitis c virus infection in the development of low-grade B-cell non-Hodgkin's lymphoma, such as splenic marginal zone B-cell lymphoma. We present a study of 3 patients with splenic marginal zone B-cell lymphoma and chronic hepatitis c, and describe clinical, histologic, and immunohistochemical features and the response to therapy in these cases. All 3 patients underwent splenectomy, polychemotherapy and alpha-interferon therapy. The first patient achieved complete remission; the second died of hepatic failure and anasarca 3 months after admission; as this writing, the third remains in complete remission 4.5 years after diagnosis. In the second patient, a long latency period of chronic hepatitis c virus infection was observed. Our data indicate that when early detection of the disease is possible, splenic marginal zone B-cell lymphoma has a relatively favorable prognosis. Our results could furthermore suggest an etiologic role for hepatitis C virus infection in the development of splenic B-cell lymphoma through multistep cooperating events. A fuller understanding of the virus-related mechanisms of lymphoproliferation could contribute significantly to the development of new therapeutic strategies.
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8/228. Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report.

    While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.
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9/228. Correlation of response to 1-beta-D-arabinofuranosyl cytosine and metabolism of drug by tumor.

    phosphorylation versus deamination of ara-C by tumor homogenates was measured in 10 patients prior to treatment with ara-C infusion. The ratio ranged from 0.24 to 1.2 in 5 malignant melanomas, 2.6 to 3.2 in 3 histiocytic lymphomas and 0.9 in a hemangiopericytoma. Treatment of the 9 patients with 2 courses of 5-day continuous ara-C infusion failed to produce objective evidence of tumor regression. The tenth patient had lymphosarcoma leukemia. The baseline ratio of ara-C phosphorylation over deamination activity of the tumor cells was 1.23. Treatment with 2 courses of continuous ara-C infusions produced a brief state of complete remission. The ratio of araC phosphorylation over deamination activity in the tumor cells after relapse was only 0.1 and retreatment with ara-C infusions failed to produce antitumor response. This study indicates that factors other than a high ratio of phosphorylation over deamination activity by the tumor seem to play an important role in the susceptibility of tumors to ara-C treatment.
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10/228. Hepatosplenic gammadelta T-cell lymphoma: relation to Epstein-Barr virus and activated cytotoxic molecules.

    AIMS: Hepatosplenic gammadelta T-cell lymphoma (TCL) is a rare, aggressive subset of peripheral TCL that presents with hepatosplenomegaly and cytopenia. Epstein-Barr virus (EBV) infection and activated cytotoxic molecules (granzyme and perforin) are uncommon in hepatosplenic gammadelta CTL. EBV infection and activated cytotoxic molecules are occasionally detected in non-hepatosplenic gammadelta TCL. We describe the clinicopathological features of three Japanese cases who were not immunodeficient. methods AND RESULTS: All cases showed gammadelta T-cell type (CD2 , CD3 , T-cell receptor (TCR)delta-1 , betaF1-). Two cases expressed natural killer (NK) cell-associated antigens (CD8-, CD16 , CD56 ; CD8-, CD16-, CD56 ), and one expressed CD8 (CD8 , CD16-, CD56-). All cases expressed cytotoxicity-associated molecules (perforin, granzyme B, TIA-1 and Fas ligand). However, perforin and Fas ligand were not detected in one case. In-situ hybridization analysis with EBER probes revealed strong nuclear positivity in all neoplastic cells. In addition, two cases showed clonal bands of the EBV terminal repeat (TR) gene. Cytologically, instead of the presence of monomorphic medium-sized cells, our three cases showed pleomorphic medium-sized and large cells. CONCLUSIONS: Our gammadelta TCL cases were clinicopathologically considered to be compatible with hepatosplenic gammadelta T-cell lymphoma. However, with regard to EBV association, activated cytotoxic profile and cytological features they resembled non-hepatosplenic gammadelta TCL. EBV may play a role in this disease by inducing cellular activation.
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