Cases reported "Lymphoma, Non-Hodgkin"

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1/259. High-dose chemotherapy with peripheral blood stem cell rescue in blastoid natural killer cell lymphoma.

    A 25-year-old man was referred because of skin rash, lymphadenopathy and anemia. Laboratory examinations revealed severe anemia (Hb, 4.8 g/dl) and elevated levels of GOT, GPT, LDH and soluble interleukin-2 receptor. work-up studies disclosed the involvement of lymphoma cells in lymph nodes, skin, bilateral kidneys and bone marrow. Lymph node biopsy revealed diffuse proliferation of medium- to large-sized lymphoblastic cells. Bone marrow aspiration showed massive infiltration of large blastic cells with no cytoplasmic granules. The lymphoma cells in bone marrow and lymph node showed surface CD3-, cytoplasmic CD3epsilon , CD4 , CD8-, CD56 , CD57-, CD16- and CD43 (MT-1) phenotype. Analyses of T cell receptor beta and gamma genes showed germ line configurations. EBER-1 was not detectable in the lymphoma cells. He was diagnosed as having blastoid natural killer (NK) cell lymphoma. In spite of several courses of combination chemotherapy, the lymphoma was progressive. He was then treated with high-dose chemotherapy and peripheral blood stem cell rescue, achieving remission which has now lasted for more than 12 months. We consider that blastoid NK cell lymphoma is an extremely aggressive subtype of CD56-positive lymphomas, and high-dose chemotherapy with peripheral blood stem cell rescue should be included for the choice of the treatment.
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2/259. Cutaneous waldenstrom macroglobulinemia in transformation.

    waldenstrom macroglobulinemia is a low-grade B-cell lymphoproliferative disorder of the elderly with characteristic monoclonal IgM-producing neoplastic infiltrates of the bone marrow, lymph node, and spleen. Cutaneous manifestations are usually nonspecific such as purpura, ulcers, and urticarial lesions. These lesions are caused by hyperviscosity of the blood, immune complex-mediated vascular damage, paraprotein deposition, and amyloid deposition. Specific skin lesions occur rarely and generally consist of translucent, flesh-colored papules composed of monoclonal IgM deposits. Rarely, there may be violaceous lesions composed of low-grade lymphoplasmacytic infiltrates characteristic of waldenstrom macroglobulinemia. Both cutaneous manifestations of the disease, as well as disease transformation to high-grade, large cell lymphoma are rare. We report two very unusual cases of waldenstrom macroglobulinemia with documented skin disease that demonstrated transformation to high-grade lymphoma. Both patients were elderly men with long-standing waldenstrom macroglobulinemia involving the bone marrow, who subsequently developed skin involvement by the disease. waldenstrom macroglobulinemia can rarely manifest as cutaneous disease, sometimes as a high-grade transformation of low-grade waldenstrom macroglobulinemia elsewhere. Distinction of cases of transformed waldenstrom macroglobulinemia from de novo cutaneous large cell lymphoma may be important, because the two entities are likely biologically different.
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3/259. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.

    Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.
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4/259. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.

    Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.
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5/259. Primary lymphosarcoma of the larynx in a child.

    A 4 7/12-year-old Caucasian female with a history of "croup-like symptoms" and persistent airway obstruction, was found to have a primary lymphosarcoma by biopsy at the time of laryngoscopy and bronchoscopy. No metastatic disease was found. After an induction course of vincristine, prednisone and local irradiation, she received CNS prophylaxis with intrathecal methotrexate and cranial irradiation. maintenance therapy, administered over a 2 3/4 year period, consisted of cyclophosphamide, methotrexate, and 6-mercaptopurine. Excluding the diagnostic evaluation, she was hospitalized only once for the management of suspected sepsis, gastrointestinal ulceration and severe bone marrow depression. Since discontinuing treatment 27 months ago, she has remained free of disease.
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6/259. Primary multifocal lymphoma of bone presenting as hypercalcemic crisis: report of a rare manifestation of extranodal lymphoma.

    Primary non-Hodgkin's lymphoma of bone is uncommon and usually manifests clinically as localized bone pain. Here we report a woman who presented with hypercalcemic crisis and extensive investigation revealed a primary multifocal lymphoma of bone. The course of the disease was very aggressive and despite intensive supportive care and urgent chemotherapy the patient died within 1 month. Since her blood PTH and calcitriol levels were suppressed and her parathyroid-hormone-related peptide (PTHrp) was mildly elevated, we believe that release of cytokines combined with PTHrp, as well as extensive osteolytic lesions, were the causes of the hypercalcemia. This is an unusual presenting symptom of lymphomas and to the best of our knowledge severe symptomatic hypercalcemia and crisis has never been reported in primary lymphoma of bone before.
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7/259. Lymphosarcoma of the mandible associated with macroglobulinemia of Waldenstrom.

    waldenstrom macroglobulinemia is a rare progressive immunoproliferative disorder involving lymphocytes and occasionally plasma cells. Unlike the multiple myeloma, this disease has minimal osseous lesions. Lesions in the jaws are very rare. This paper presents a case of lymphosarcoma in the mandible in a 68-year-old woman, who was diagnosed as suffering from waldenstrom macroglobulinemia. The possibility of immunosuppression by Leukeran as an aetiologic factor causing the bone lesions is suggested.
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8/259. Isolation of beta-lactamase positive vancomycin resistant enterococcus faecalis; first case in australia.

    An increasing number of clinical isolates of vancomycin resistant enterococci (VRE) have been reported in the literature since 1988. Only a few cases of beta-lactamase producing VRE have been described worldwide. This article reports the first case of beta-lactamase positive VRE in australia. This strain of enterococcus faecalis was isolated from a patient with non-Hodgkin's lymphoma who subsequently underwent a bone marrow transplant. Screening of all ward contacts did not detect any further case of beta-lactamase producing VRE. With the development of multiple antibiotic resistance in enterococci, additional infection surveillance protocols have been implemented in the hospital. These include routine screening of 'at risk' patients, instigating relevant infection control measures for management of VRE positive patients and controlling the usage of vancomycin in order to limit the development of resistant isolates.
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9/259. Non-Hodgkin's lymphoma: present status and future prospects.

    Both management and prognosis vary with the many different varieties of non-Hodgkin's lymphoma. In large-cell disease, age and other risk factors influence prognosis. Relapses may respond to chemotherapy plus autologous bone marrow transplantation. Therapy with antibodies to tumor cells has made its debut, but its role remains unclear.
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10/259. Ph-negative non-Hodgkin's lymphoma occurring in chronic phase of Ph-positive chronic myelogenous leukemia is defined as a genetically different neoplasm from extramedullary localized blast crisis: report of two cases and review of the literature.

    This report describes two cases of philadelphia chromosome-negative (Ph(-)) non-Hodgkin's lymphomas (NHLs) recognized in patients with chronic phase Ph-positive (Ph( )) chronic myelogenous leukemia (CML). Lymph node biopsy of patient 1 was initially diagnosed as diffuse large B cell non-Hodgkin's lymphoma (NHL, T cell rich variant), but at relapse showed immunoblastic features with a marked decrease of admixed lymphocyte components. Patient 2 presented with thickened parietal pleura which revealed a CD30-positive anaplastic large cell lymphoma showing null cell phenotype and genotype with abundant admixed neutrophils and lymphocytes. At the time of lymphoma diagnosis, the patients had CML for 33 and 10 months, respectively. dna obtained from bone marrow cells at the time of lymphoma diagnosis showed BCR/ABL gene rearrangements by both Southern blot analysis and reverse transcription polymerase chain reaction (RT-PCR), but lacked both immunoglobulin and T cell receptor gene rearrangements. BCR gene rearrangement and BCR/ABL fusion gene were also identified in lymph node and pleural biopsies by Southern blot and RT-PCR analysis, respectively. However, both biopsy specimens also contained reactive lymphocytes and neutrophils, and no fusion signals between BCR and ABL genes were identified in the hyperdiploid lymphoma cells of either case by fluorescence in situ hybridization (FISH). These data suggest the lymphoma cells in both cases were not genetically associated with BCR/ABL. Therefore, these cases were not diagnosed as an extramedullary localized blast crisis in CML, but as Ph(-) NHLs. This represents the first definitive demonstration of peripheral B cell lymphoma occurring by a separate genetic pathway, lacking BCR/ABL, in patients with Ph( ) CML. A review of the literature identified two different subtypes of malignant lymphomas arising in patients with an antecedent or concurrent diagnosis of CML. The most common are T cell lymphomas displaying an immature thymic phenotype, while peripheral B cell lymphomas are more rare. Our study shows, however, that 'Ph( ) NHL' occurring in CML or acute lymphocytic leukemia (ALL) may represent an unrelated neoplasm, even if standard cytogenetic analysis reveals a Ph( ) chromosome, and that FISH is required to confirm whether a localized lymphoid neoplasm is either a true extramedullary localized blast crisis or genetically distinct neoplasm. leukemia(2000) 14, 169-182.
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