Cases reported "Lymphoma, Mantle-Cell"

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1/126. De novo CD5 burkitt lymphoma/leukemia.

    CD5 is a T-cell marker aberrantly expressed in B-cell chronic lymphocytic leukemia and mantle cell lymphoma. Other B-cell neoplasms, including burkitt lymphoma, are usually CD5-. We report 4 cases of de novo CD5 burkitt lymphoma/leukemia in elderly patients, all of whom were in a leukemic phase and had variable lymph node and splenic involvement. The blasts were typically medium sized, with folded nuclei, distinct but not prominent nucleoli, and moderate amounts of somewhat vacuolated basophilic cytoplasm; they were terminal deoxynucleotidyl transferase--negative and surface immunoglobulin--positive. All 4 cases demonstrated c-myc rearrangement, but none had t(14;18), t(11;14), or cyclin d1 overexpression or rearrangement. Only 1 patient achieved complete remission after hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) therapy. One patient responded poorly to hyper-CVAD, and 2 patients died during induction chemotherapy. These rare cases of aggressive lymphoid malignancy with CD5 positivity and molecular features associated with burkitt lymphoma/leukemia are best classified as Burkitt leukemia. However, the morphologic and immunophenotypic similarity to the blastoid variant of mantle cell lymphoma are diagnostically challenging. The diseases can be distinguished at the genetic level, since burkitt lymphoma involves the rearrangement of c-myc, and mantle cell lymphoma usually the overexpression or rearrangement of cyclin d1.
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keywords = lymphoma, l
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2/126. 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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ranking = 1.1818186959006
keywords = lymphoma, l
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3/126. Long-term remission in an elderly patient with mantle cell leukemia treated with low-dose cyclophosphamide.

    We present an elderly patient with mantle cell leukemia who was successfully treated with low-dose cyclophosphamide (CY). A 76-year-old female was diagnosed as mantle cell leukemia based on abnormal lymphocytosis and splenomegaly without lymphadenopathy. She was orally treated with 50 mg of CY daily and had continuous remission over 4 years. Rearrangements of BCL1 and immunoglobulin heavy chain genes in the peripheral blood lymphocytes were detected at diagnosis, but not 1 or 4 years later. Further studies are required to confirm the role of low-dose CY therapy for patients with mantle cell leukemia and lymphoma.
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ranking = 0.090926312670589
keywords = lymphoma, l
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4/126. Expression of cell cycle regulating proteins in an unusual transformation of mantle cell lymphoma.

    We describe here two patients with mantle cell lymphoma (MCL) who after a few years, developed to the diffuse large cell lymphoma (DLCL) (anaplastic centrocytic lymphoma) growing in a diffuse sheets without the classical MCL component. In both the initial and second biopsy specimens, in each case, tumor cells were positive for cyclin d1, sIgM, sIgD, and CD5, but were negative for CD10 and CD23. In a study of immunoglobulin heavy chain (IgH) gene rearrangement, using the polymerase chain reaction (PCR) method, the products obtained from each paired biopsy tissue sample were the same size, and in one case had an identical sequence to the non-mutated VH gene. immunohistochemistry was used to examine the expression of p53, p27Kip1 and cyclin e. Interestingly, there was clear overexpression of p53 protein in case 1 but not in case 2, compared with other typical MCL cases. The expression of p27Kip1 in the second biopsies of each case was decreased compared with those in the initial biopsies. In case 2, however, p27Kip1 was clearly expressed in the first and second biopsies, in contrast to other typical MCL cases. Thus these 2 cases demonstrate not only that the variant form of MCL may arise de novo, but also that MCL may transform to DLCL at the time of relapse. Although the mechanism of tumor progression/transformation is still poorly understood, the overexpression of p53 or p27Kip1 may be linked to a cellular mechanism involved in the development of the variant form of MCL.
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ranking = 0.63636995957756
keywords = lymphoma, l
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5/126. Intracranial malignant B-cell lymphoma of the dura.

    OBJECTIVE: Malignant B-cell lymphomas of the dura mater are very rare. A case of primary centroblastic/centrocytic lymphoma of the dura mimicking a bilateral convexity meningioma is presented. CLINICAL PRESENTATION: A 50-year-old woman was referred to our institution with a 6-month history of headache and two Jacksonian seizures. Computed tomography revealed a parafalcine and bilateral convexity lesion. cerebral angiography and magnetic resonance imaging were performed prior to surgery. At surgery the tumor was removed subtotally. The patient was treated postoperatively by combined chemo- and radiotherapy. CONCLUSION: Laboratory studies and follow-up examinations revealed no evidence of systemic lymphoma nor of any immunocompromised state. According to the presented case combined surgery and chemoradiotherapy seems to be an effective treatment for this rare lesion.
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ranking = 0.63635978074539
keywords = lymphoma, l
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6/126. An adult patient with hypersensitivity to mosquito bites developing mantle cell lymphoma.

    hypersensitivity to mosquito bites (HMB) has been known to occur exclusively in the first 2 decades of life and is frequently associated with Epstein-Barr virus (EBV) infection and lymphoproliferative diseases. We report here the first adult patient with HMB, a 61-year-old Japanese man who developed mantle cell lymphoma. EBV was detected in the lymph node by polymerase chain reaction and by in situ hybridization. serum levels of interleukin (IL)-4, IL-6, and IL-10 were markedly increased, and the T-helper cell (Th)1/Th2 balance determined by intracellular cytokine levels was polarized to Th2. These findings suggest that the Th1/Th2 imbalance could partly be involved in the pathogenesis of HMB.
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ranking = 0.45455013269559
keywords = lymphoma, l
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7/126. Acute radiation-induced hepatic injury: evaluation by triphasic contrast enhanced helical CT.

    We report a case of radiation-induced hepatic injury as a complication of localized abdominal radiotherapy for epidural spread of non-Hodgkin's lymphoma. The liver was evaluated by triphasic contrast enhanced helical CT scan. Hepatic biopsy demonstrated changes typical of veno-occlusive disease. The pattern of hepatic enhancement resulting from the radiation-induced veno-occlusive process is discussed.
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ranking = 0.090914154621054
keywords = lymphoma, l
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8/126. Successful allogeneic stem-cell transplantation with prophylactic stepwise G-CSF primed-DLIs for relapse after autologous transplantation in mantle cell lymphoma: a case report and literature review on the evidence of GVL effects in MCL.

    Mantle cell lymphoma (MCL) is a distinctive clinicopathologic entity and represents 2-8% of all non-Hodgkin's lymphomas. The median survival of patients with MCL is only 3 years, and none of the available conventional chemotherapy regimens appears curative. Encouraging results have been reported with high-dose chemotherapy with autologous stem-cell transplantation (autoSCT). However, a plateau in disease-free survival was not observed in relapsed MCL on the autoSCT trials. Promisingly, alloSCT appears to induce durable remissions via a graft-versus-lymphoma (GVL) effect. Donor lymphocyte infusions (DLIs), by virtue of a GVL effect, have been shown to induce durable remissions in a few cases with refractory MCL that recur after alloSCT. In this article, we review the literature on the evidence of the GVL effects in MCL and describe a patient with relapsed MCL shortly after high-dose chemotherapy with autoSCT. The patient was then successfully treated with Bu/Cy/VP-16 for an alloSCT followed by DLIs in a stepwise fashion. MNCs > 10 x 10(8)/kg were collected by two large-volume leukaphereses from the donor. Harvested stem cells from the 2(nd) day were cryopreserved for the future use as prophylactic DLIs to be given in a stepwise fashion. Cyclosporin and methotrexate were used for GVHD prophylaxis. He had achieved only a partial response by D 64 post transplant. G-CSF-primed cryopreserved DLIs were then infused on D 64 and D 92 to enhance the GVL effect. Grade 3 intestinal GVHD developed 20 days after the 2(nd) DLI and was partially controlled with the combination of cyclosporin, prednisone, and mycophenolate mofetil. Clinical complete remission was observed at D 112, and maintained until the last follow-up day (D 615). Our findings suggest that alloSCT followed by prophylactic DLIs may offer a curative approach to refractory MCL.
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ranking = 0.63638607606183
keywords = lymphoma, l
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9/126. patients with mantle-cell lymphoma relapsing after autologous stem cell transplantation may be rescued by allogeneic transplantation.

    Two patients with disseminated mantle-cell lymphoma relapsed 24 and 13 months, respectively, after high-dose chemotherapy and autologous stem cell transplantation (autoSCT). Both patients had an HLA-identical sibling and received an allogeneic stem cell transplant (alloSCT) 32 and 18 months after autologous transplant, after conditioning with fractionated 12 Gy total body irradiation plus cyclophosphamide 120 mg/kg. They are both alive and in complete remission 24 months after transplant. Both patients have developed chronic graft-versus-host disease and their karnofsky performance status is 90%. AlloSCT may offer a useful approach in a subgroup of patients with mantle-cell lymphoma who have relapsed after autologous transplantation.
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ranking = 0.54546513555266
keywords = lymphoma, l
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10/126. Primary spinal epidural mantle cell lymphoma: case report.

    OBJECTIVE AND IMPORTANCE: Mantle cell lymphoma is a distinct clinicopathological type of non-Hodgkin's lymphoma that often presents at an advanced stage, with systemic spread. Spinal involvement is uncommon and generally occurs as part of advanced disease or generalized relapses. Primary spinal epidural lymphoma is a rare initial manifestation of non-Hodgkin's lymphoma, and mantle cell lymphoma with initial presentation in the spinal epidural space is extremely rare, having been previously reported in only two cases. CLINICAL PRESENTATION: We report a case of a 71-year-old man who presented with increasing weakness and numbness of the legs. magnetic resonance imaging revealed a spinal epidural mass in the lumbosacral region. INTERVENTION: The patient underwent a partial L4 and L5-S1 laminectomy, with incomplete resection of the mass for spinal decompression and tissue diagnosis. Mantle cell lymphoma was diagnosed in the pathological examination. CONCLUSION: After radiotherapy, the disease recurred with a soft-tissue mass in the anterior maxillary area of the face. The patient underwent restaging and was treated with chemotherapy, with only a partial response. Mantle cell lymphoma with primary spinal epidural presentation is rare. This diagnosis can be established and other causes of spinal cord compression can be ruled out by obtaining tissue for proper histopathological examinations. Because of its aggressive behavior and poor prognosis, mantle cell lymphoma should be treated using a combined-modality approach.
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ranking = 1.0909073687329
keywords = lymphoma, l
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