Cases reported "Lymphoma, B-Cell"

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1/63. Epstein-Barr virus related hemophagocytic syndrome in a T-cell rich B-cell lymphoma.

    We report the case of a 30-year-old woman who presented with an EBV related hemophagocytic syndrome. After a few months she developed a T-cell rich B-cell non-Hodgkin's lymphoma with liver involvement. Serological data demonstrated a reactivation of the EBV infection. Tumor progression with liver involvement occurred during treatment with conventional chemotherapy. Tumor reduction and disappearance of all masses was seen after starting high-dose sequential chemotherapy, followed by an autologous peripheral blood progenitor transplantation LMP-1 could be amplified in the tumor material by PCR technology, but no LMP-1 expression could be found in the few malignant B-cells with Reed-Sternberg morphology. sequence analysis of the carboxy terminal of the LMP-1 region revealed the naturally occurring 30 bp deletion variant of the LMP-1 with multiple point mutations within the NF kb region. Since LMP-1 was not expressed in the malignant tumor cells, no evidence could be found, that EBV participated in the tumorigenesis of this case.
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2/63. adrenal insufficiency caused by primary aggressive non-Hodgkin's lymphoma of bilateral adrenal glands: report of a case and literature review.

    A 64-year-old woman was hospitalized because of poor general condition, gastrointestinal upset, unexplained fever, electrolyte imbalances, and an incidental finding of bilateral huge adrenal masses on computerized tomography (CT) of the abdomen. Non-Hodgkin's lymphoma (NHL) of B-cell origin was proven by ultrasound-guided aspiration biopsy of the left adrenal gland. Meanwhile, primary adrenal insufficiency was confirmed by her low serum cortisol level, high ACTH level, and inadequate adrenal response to the rapid ACTH stimulation test. The diagnosis of primary adrenal NHL was supported by detailed physical examinations, bone marrow examination, and such imaging studies as CT scan and sonography. She received three courses of chemotherapy with cyclophosphamide, vincristine, and prednisolone and there was an initial transient response, but she died of sepsis and progression of NHL three and a half months later.
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3/63. "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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4/63. Modulation of clinical drug resistance in a B cell lymphoma patient by the protein kinase inhibitor 7-hydroxystaurosporine: presentation of a novel therapeutic paradigm.

    Emerging evidence suggests that apoptosis is an important mechanism of tumor cell death from antineoplastic therapy. 7-hydroxystaurosporine (UCN-01) is a novel protein kinase inhibitor that increases chemotherapy-induced apoptosis in vitro and is in early phases of clinical development. In this report, we present a 68-year-old patient with chemotherapy-resistant lymphoma treated with UCN-01 and chemotherapy. He had a stage IV plasmacytoid lymphoma that failed to enter remission with high-dose EPOCH II (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) chemotherapy. Due to disease progression and transformation to large cell lymphoma in the liver and bone marrow, he received UCN-01. Four weeks later, he received "standard-dose" EPOCH because of progression, developed severe neutropenia for 9 days, and expired from candida sepsis on day 23. At autopsy, there was no histological evidence of residual lymphoma, although PCR for immunoglobulin gene rearrangement analysis revealed a faint clonal band in two of six nodes but none in the liver. Significantly, no B cells were detected by immunohistochemistry in lymph nodes, and a polyclonal ladder pattern associated with the presence of normal B cells was not seen in the immunoglobulin gene rearrangement PCR assay. Profound peripheral lymphopenia (50 cells/microliter) was also observed. pharmacokinetics showed UCN-01 salivary concentrations, a surrogate for free drug concentrations, to be within an effective range in vitro (45 nmol/L) as a modulator of dna-damaging agent cytotoxicity. in vitro, UCN-01 is synergistic with multiple cytotoxic agents and increases fludarabine-induced apoptosis in a human breast cell line. These results suggest that UCN-01 sensitized the lymphoma to the cytotoxic effects of EPOCH, possibly by modulating the "threshold" for apoptosis, and may illustrate a new paradigm for reversal of drug resistance.
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ranking = 38.32038178581
keywords = disease progression, progression
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5/63. B-cell-type malignant lymphoma with placental involvement.

    We present here a case of B-cell-type mediastinal malignant lymphoma during pregnancy complicated by placental involvement. In this case, some nodular high-echo patterns were recognized in the placenta by ultrasonography. A cesarean section and resection of the mediastinal tumor were performed at 33 weeks and 6 days of gestation due to the deterioration of the dyspnea. A female infant weighing 1,868 g was delivered and she is now a healthy 2-year-old. The mother, however, died of the disease 1 month after surgery, due to progression of the tumor. The placenta showed numerous white firm nodules varying from 3 mm to 3 cm in diameter. The pathologic findings of both the mediastinal tumor and the placenta indicated primary mediastinal (thymic) B-cell lymphoma.
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6/63. retreatment with chimeric CD 20 monoclonal antibody in a patient with nodal marginal zone B-cell lymphoma.

    A patient with advanced and chemotherapy-refractory nodal marginal zone B-cell lymphoma was given a course of chimeric CD 20 monoclonal antibody Rituximab. Partial response was observed without any major toxicities. retreatment with Rituximab for disease progression six months after the first course led to partial remission. Adjuvant radiotherapy was given for the residual disease and, currently, patient's disease remains stable eight months after the second course of Rituximab. This case demonstrates the therapeutic efficacy and feasibility of retreatment with Rituximab for relapsed or refractory low grade lymphoma.
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ranking = 37.32038178581
keywords = disease progression, progression
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7/63. borrelia burgdorferi-associated cutaneous marginal zone lymphoma: a clinicopathological study of two cases illustrating the temporal progression of B. burgdorferi-associated B-cell proliferation in the skin.

    AIMS: A relationship between borrelia burgdorferi and primary cutaneous B-cell lymphoma (PCBCL) has recently been confirmed following demonstration of the organism in lesional skin of patients with PCBCL. We report herein two cases of B. burgdorferi-associated PCBCL which strengthen this association by demonstrating the organism in cutaneous B-cell infiltrates present at sites in which PCBCL subsequently developed. methods AND RESULTS: All studies were performed on formalin-fixed paraffin-embedded tissues. These were examined by routine light microscopy and immunohistochemically by a standard streptavidin-biotin-complex technique. Genotypic studies were also undertaken using semi-nested polymerase chain reaction (PCR) for immunoglobulin heavy chain gene rearrangement, and nested PCR for B. burgdorferi flagellin gene. Both patients presented with erythematous skin lesions, biopsy of which showed dense perivascular infiltrates comprising small t-lymphocytes and collections of B-blasts. Primary cutaneous marginal zone lymphoma (MZL) developed subsequently in both cases at the same site. PCR for B. burgdorferi flagellin gene was positive in the perivascular lymphocytic infiltrates and the succeeding lymphomas in both patients. CONCLUSIONS: These results show that, at least in some instances, PCBCL arises from chronically stimulated lymphoid tissue acquired in the skin in response to B. burgdorferi infection. This may have significant therapeutic implications and warrant further studies on the extent of this association.
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8/63. Systemic B-cell lymphoma presenting as an isolated lesion on the ear.

    We report a case of systemic B-cell lymphoma that presented as an isolated cutaneous lesion on the ear, mimicking a primary cutaneous B-cell lymphoma. Although there was no clinical evidence of systemic disease, bone marrow involvement was found on further investigation and subsequent immunoglobulin gene analysis revealed an identical clone in the skin lesion and bone marrow aspirate. Evidence of a t(14 : 18) translocation was not identified. This case is unusual for several reasons. First, involvement of the pinna as a presenting feature of systemic lymphoma has not been reported previously. Second, the cutaneous lesion had been present for 3 years prior to diagnosis and there has been no clinical progression of systemic lymphoma during 2 years of follow-up. Third, the lymphoma does not correspond exactly to any of the entities in the REAL classification of systemic B-cell lymphoma. This case underlines the indolent nature of some systemic B-cell lymphomas and the need to investigate thoroughly patients with disease apparently confined to the skin.
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9/63. central nervous system involvement in pyothorax-associated lymphoma: ring enhancement on CT scan.

    A 71-year-old male was diagnosed as having pyothorax-associated lymphoma (PAL), a rare hematological malignancy associated with artificial pneumothorax for the treatment of pulmonary tuberculosis. Because of the low incidence, its clinical features have not been fully clarified. The PAL lesion in this patient disappeared after local radiotherapy and corticosteroid treatment. However, 8 months after the initial diagnosis, he developed a variety of neurological disorders and low-grade fever. Neither recurrence of the primary pulmonary lesion nor nodal enlargement was observed. Immediate magnetic resonance imaging of the brain revealed a ring-enhanced mass, suggesting a brain abscess. However, through a stereotactic biopsy of the brain lesion, infiltration of PAL cells was identified. The brain lesion rapidly enlarged despite intensive radiotherapy, and he finally died of lymphoma progression. This case demonstrates that PAL may progress as a type of extranodal lymphoma, and that brain lesions may produce a ring-enhanced pattern on computed tomography.
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10/63. Management of non Hodgkin's intraocular lymphoma with intravitreal methotrexate.

    Intraocular non Hodgkin's lymphoma has traditionally been treated with radiotherapy or systemic chemotherapeutic agents. Unfortunately, radiotherapy rarely leads to a long term remission, and can only be safely administered once. Systemic chemotherapy has limited intraocular penetration. Intravitreal administered methotrexate is highly effective at inducing remission and is well tolerated. A case report is presented of a recurrent intraocular lymphoma following radiotherapy and systemic chemotherapy which responded to a combination of intravitreal methotrexate and dexamethasone. A response was seen within three weeks of treatment initiation. Toxicity was limited to the progression of a pre-existing cataract. review of the literature shows that intravitreal methotrexate is well tolerated, with few acute complications. However, with monotherapy, local recurrences have been observed.
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