Cases reported "Burkitt Lymphoma"

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1/39. Burkitt's lymphoma presenting as lower lip paraesthesia in a 24 year old Nigerian. Case report.

    An unusual case of stage D Burkitt's lymphoma in a 24 year old Nigerian female undergraduate is reported. There was a four month history of left lower lip paraesthesia followed three months later by a slowly progressive 'pimple-sized' nodular mandibular swelling arising from the mental foramen region. A full-blown, rapidly developing abdominal mass manifested only three weeks after a biopsy of the mandibular swelling. Aspiration of the latter and a histologic report of the mandibular mass confirmed Burkitt's lymphoma. The patient responded very well to appropriate chemotherapy. Clinicians should not overlook insidious jaw swellings in any adult residing in the endemic zone of Burkitt's lymphoma, in view of the fact that successful therapy is dependent on early diagnosis. Mental nerve paraesthesia is very rarely seen in Burkitt's lymphoma.
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2/39. Anti-CD20 monoclonal antibody therapy in Epstein-Barr Virus-associated B cell lymphoma following lung transplantation.

    Post-transplant lymphoproliferative disease is a complication of bone marrow and solid organ transplantation, mostly associated with Epstein-Barr virus infection and chronic immunosuppression. Even if spontaneous resolution after cessation of immunosuppressive therapy can be observed, the prognosis of this disorder is usually poor with a low response to specific treatment. We describe a case of B-cell lymphoma of the nasopharynx occurring 6 months after double-lung transplantation. In spite of its monoclonal nature, anti-CD 20 monoclonal antibody given in the presence of reduced immunosuppression resulted in a complete response. The patient also received "consolidation" radiation therapy to prevent the recurrence. The treatment was well tolerated with minimal side effects. The patient was asymptomatic and had a well functioning graft more than 1 year after therapy.
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3/39. Development of a biologically distinct EBV-related lymphoproliferative disorder following autologous bone marrow transplantation for an EBV-negative post-renal allograft Burkitt's lymphoma.

    Post-transplant lymphoproliferative disorder (PTLD) is a known complication of both solid organ transplantation and allogeneic bone marrow transplantation (BMT) but is rarely seen following autologous BMT. We report the case of a 45 year-old female who developed Burkitt's lymphoma eight years after a renal allograft. This PTLD was found to have lambda light chain restriction, contained del(8)(q24) and add(14)(q32), and was negative for EBV on immunohistochemical and dna-based PCR analyses. Immunoglobulin heavy chain (IgH) PCR studies revealed a prominent clonal rearrangement. She responded to intravenous cyclophosphamide and proceeded to high-dose chemoradiotherapy and mafosfamide-purged autologous BMT. Thirty-nine days post-BMT she presented with cough and fever and developed hepatic dysfunction; abnormal lymphoplasmacytoid cells were noted in the peripheral blood. Investigations revealed kappa light chain restriction, an oligoclonal IgH rearrangement, a normal karyotype and PCR studies for EBV were positive, consistent with a clinically and biologically distinct PTLD. She initially improved following discontinuation of immunosuppression, but then deteriorated abruptly and died 58 days post-BMT. It is likely that the two separate episodes of PTLD in this patient, one of which was atypical, arose as a result of both the chronic use of cyclosporine and the impairment of cell-mediated immunity associated with autologous BMT. The sequence of events in this patient should contribute to a better understanding of late-onset, EBV-negative PTLD.
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4/39. Unusual gingival presentation of post-transplantation lymphoproliferative disorder: a case report and review of the literature.

    Post-transplantation lymphoproliferative disorder is a well-documented complication of solid organ or bone marrow transplantation. Histologically, it is characterized by an abnormal proliferation of lymphocytes, which can range from benign B-cell hyperplasia to malignant lymphoma. Non-Hodgkin's lymphoma (NHL) is associated with several risk factors, such as congenital or acquired immunodeficiency states, autoimmune disorders, and infectious agents (eg, Epstein-Barr virus). Primary sites of presentation in the head and neck are Waldeyer's ring, paranasal sinuses, salivary glands, the oral cavity, and the larynx. Clinical appearance of gingival NHL varies but is usually found to be an asymptomatic gingival enlargement or mass resembling a pyogenic granuloma. We present a patient with a gingival ulceration that was subsequently diagnosed as Epstein-Barr virus malignant lymphoma resulting from the immunosuppression needed to prevent graft-versus-host disease after bone marrow transplantation.
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5/39. Peripheral neuropathy associated with acquired immunodeficiency syndrome (AIDS)-related Burkitt's lymphoma.

    Peripheral neuropathy associated with acquired immunodeficiency syndrome (AIDS)-related Burkitt's lymphoma usually occurs as a toxic effect of chemotherapeutic agents. Whereas primary peripheral nerve involvement is an unusual complication, we report on a human immunodeficiency virus (hiv)-positive patient with Burkitt's lymphoma and sciatic neuropathy due to compression by a lymphomatous mass. Therapy with radiation and chemotherapy was followed by clinical and radiological improvement, but recurrent neurological deficits in a similar distribution occurred later from lymphomatous meningitis.
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6/39. Burkitt s lymphoma variant of post-transplant lymphoproliferative disease (PTLD).

    The occurrence of posttransplant lymphoproliferative disorder (PTLD) in solid organ allograft recipients can be quite varied in clinical presentation, histopathological characteristics and frequency. A variety of lymphomas can develop as a PTLD although some types appear infrequently and remain poorly understood in this clinical setting. In this report, we describe two cases of Burkitt s lymphoma presenting as a PTLD following liver transplantation. The recipients were 12 and 44 years of age and displayed gastrointestinal involvement by the tumors several years following transplant. The tumors displayed the typical histological features of Burkitt s lymphoma and were markedly positive for EBV. The tumors displayed similar immunophenotypic characteristics by flow cytometry and had rearrangements of the immunoglobulin J-H heavy chain. The tumors required aggressive chemotherapy and a cessation of immunosuppressive therapy. This report demonstrates that Burkitt s type lymphomas can develop in the posttransplant setting and that these tumors contain morphologic, cytofluorographic and molecular features identical to Burkitt s lymphomas that occur in non-transplant patients. Our experience is that these PTLD- Burkitt s lymphomas behave aggressively and require intensive chemotherapeutic intervention.
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7/39. Tailored chemotherapy for malignant lymphoma arising in the setting of posttransplant lymphoproliferative disorder after solid organ transplantation.

    Posttransplant lymphoproliferative disorder is a clinical challenge. Traditional chemotherapy results in tumor response, but toxicity and transplant rejection limit survival. The authors treated seven patients with malignant lymphoma after organ transplant with chemotherapy tailored to individual patient response. Chemotherapy consisted of vincristine, cyclophosphamide, and prednisone, with or without doxorubicin (Adriamycin; Pharmacia & Upjohn, Peapack, NJ, U.S.A.), or the ProMACE-CytaBOM regimen. Six of seven patients (86%) showed a complete response to treatment, with five of seven (71%) alive disease-free at 9 to 72 months (mean 38.2) after treatment. The results show that chemotherapy tailored to individual patient response is a safe and effective therapy for malignant lymphoma arising in patients with posttransplant lymphoproliferative disorder.
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8/39. burkitt lymphoma arising in organ transplant recipients: a clinicopathologic study of five cases.

    We report five cases of burkitt lymphoma arising in organ transplant recipients. There were four men and one woman with a mean age of 35 years. All were solid organ recipients with three renal, one liver, and one double lung transplantation. The time interval between organ transplantation and lymphoma averaged 4.5 years. patients typically presented with high-stage disease with generalized lymphadenopathy and bone marrow involvement. histology showed classic burkitt lymphoma or atypical variant/Burkitt-like morphology. C-MYC rearrangement, including three cases with immunoglobulin heavy chain and two cases with lambda light chain, and Epstein-Barr virus were detected in all the cases. Additional chromosomal abnormalities were present in two of three cases and p53 mutation was found in one of three cases. Aberrant genotype and phenotype were frequently encountered, including minor monoclonal or oligoclonal T-cell populations and undetectable surface immunoglobulin light chain expression. Four patients received antilymphoma regimens, with combination chemotherapy (three patients) and/or Rituximab (three patients), in addition to reduction of immunosuppression. All four patients achieved complete remission. We conclude that posttransplant burkitt lymphoma represents a characteristic clinicopathologic entity and occurs later after transplantation. Genotypic and phenotypic aberrations are often present. Rituximab may be an effective alternative to conventional combination chemotherapy in the treatment of a posttransplant burkitt lymphoma.
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9/39. Epstein-Barr virus-associated primary B-cell lymphoproliferative disorder of the cerebellum in an immune competent man.

    BACKGROUND. The role of the Epstein-Barr virus (EBV) in lymphoproliferative lesions has been widely accepted. Most of these lesions occur in patients who have deficiencies in their immune status. lymphomatoid granulomatosis (LG) is a lymphoproliferative disorder originally characterized as an angiocentric, necrotizing, pleomorphic infiltrate of mononuclear cells. The etiology of LG is unknown. It was originally hypothesized that LG may represent an unusual lymphoid response to an infective organism, possibly EBV. methods. tissues from a previously healthy 60-year-old, healthy white man with primary cerebellar lymphomatoid granulomatosis were examined for the presence of EBV by nucleic acid hybridization. RESULTS. The original LG lesion was a polyclonal B-cell proliferation that contained detectable amounts of EBV. Peripheral blood leukocytes were negative for EBV by the same assay. After an 18-month remission, a tumor reappeared near the site of the primary lesion, which had the histologic appearance of a lymphoma. The cells showed restricted clonality and contained a similar amount of EBV-related dna as the original lesion. Peripheral blood leukocytes at the time of recurrence were negative for EBV. The patient died approximately 2 months after the recurrent tumor was detected. CONCLUSIONS. This case demonstrated the development of a primary cerebellar B-cell lymphoproliferative disorder, histologically identical to lymphomatoid granulomatosis, that transformed into a lymphoma. The original tumor and the subsequent lymphoma contained, on average, several copies of EBV-related dna per cell. Despite an extensive survey of the patient, no immune deficit was detected. Interpretation of the literature with the results of this case suggest that this instance of primary cerebellar LG arose as a consequence of an unusual EBV-associated B-cell lymphoproliferation. It is suggested that EBV may be a significant factor in the initiation of the abnormal proliferations of T-cells or B-cells reported in this disorder.
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10/39. Multiple cranial neuropathies: presenting signs of systemic lymphoma.

    A 58-year-old man four years after orthototic liver transplantation presented with a mental neuropathy, multiple ocular motor nerve palsies, and an upper extremity motor neuropathy. Serial neuroimaging, cerebrospinal fluid analysis, total body computerized tomography and paraesophageal needle biopsy failed to reveal the underlying etiology. sural nerve biopsy showed endoneurial perivascular infiltration with lymphoid cells, and bone marrow biopsy confirmed the diagnosis of a Burkitt's-type lymphoma. Systemic lymphoma with neural infiltration should be suspected in the immunosuppressed patient who presents with cranial or peripheral neuropathies.
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