1/149. Characterization of Epstein-Barr virus (EBV)-infected natural killer (NK) cell proliferation in patients with severe mosquito allergy; establishment of an IL-2-dependent NK-like cell line.The clinical evidence of a relationship between severe hypersensitivity to mosquito bite (HMB) and clonal expansion of EBV-infected NK cells has been accumulated. In order to clarify the mechanism of EBV-induced NK cell proliferation and its relationship with high incidence of leukaemias or lymphomas in HMB patients, we studied clonally expanded NK cells from three HMB patients and succeeded in establishing an EBV-infected NK-like cell line designated KAI3. immunoblotting and reverse transcriptase-polymerase chain reaction (RT-PCR) analyses revealed that KAI3 cells as well as infected NK cells exhibited an EBV latent infection type II, where EBV gene expression was limited to EBNA 1 and LMP1. As KAI3 was established by culture with IL-2, IL-2 responsiveness of peripheral blood NK cells from patients was examined. The results represented markedly augmented IL-2-induced IL-2R alpha expression in NK cells. This characteristic property may contribute to the persistent expansion of infected NK cells. However, KAI3 cells as well as the NK cells from patients were not protected from apoptosis induced by either an anti-Fas antibody or NK-sensitive k562 cells. Preserved sensitivity to apoptosis might explain the relatively regulated NK cell numbers in the peripheral blood of the patients. To our knowledge, KAI3 is the first reported NK-like cell line established from patients of severe chronic active EBV infection (SCAEBV) before the onset of leukaemias or lymphomas. KAI3 cells will contribute to the study of EBV persistency in the NK cell environment and its relationship with high incidence of leukaemias or lymphomas in HMB patients.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/149. Blastic mantle cell leukemia: a previously undescribed form.The leukemic phase of mantle cell lymphoma (mantle cell leukemia) is defined as an absolute lymphocyte count of greater than 4,000/microliter and characterized by the presence of relatively small, slightly irregular lymphocytes in the peripheral blood. Although a variant of mantle cell lymphoma with blastic morphsology exists and has been previously well described, a blastic morphologic variant of mantle cell leukemia has not been described. We report such a case in a 74-year-old male who presented with splenic rupture and an elevated white blood cell (WBC) count. The diagnosis was based on flow cytometric immunophenotyping and the cytomorphology of the peripheral blood.- - - - - - - - - - ranking = 0.66666666666667keywords = lymphoma (Clic here for more details about this article) |
3/149. Acute leukemia following prolonged cytotoxic agent therapy.1. Nine patients in whom acute non-lymphoblastic leukemia (ANLL) developed following prolonged alkylating agent therapy are described. Five of the patients received no radiotherapy. The conditions treated were: Hodgkin's disease (four patients), primary amyloidosis, primary macroglobulinemia, malignant lymphoma, multiple myeloma, and carcinoma of the tonsil. 2. Prior to the advent of chemotherapy, this complication was not observed in large series of patients with lymphoproliferative disorders and multiple myeloma. However, the medical literature now contains at least 125 other detailed reports of ANLL developing after prolonged cytotoxic agent therapy. 3. multiple myeloma and Hodgkin's disease, both of which commonly have good responses to chemotherapy, predominate as the underlying diseases. However, 35% of the case reports involve patients with other illnesses, including 12 patients who did not have neoplasms. 4. More than half of the patients developing ANLL have received chemotherapy alone without radiotherapy. 5. At least half of the patients developing ANLL experienced long periods of significant cytopenia during therapy, often with documentation of bone marrow dysplasia. 6. The wide variety of drugs associated with this complication suggests that any cytotoxic agent may be leukemogenic. However, alkylating agents overwhelmingly predominate as the class of compounds which are most often associated with terminal ANLL. 7. The vast majority of patients reported in the literature with ANLL complicating underlying malignancies have received cytotoxic drugs for prolonged periods (median 3 1/2 years) and leukemia developed most commonly 3 to 5 years after the diagnosis of the underlying disease. Most of these patients benefited from therapy and survived longer (median 5 years) than historical control of untreated patients. 8. The leukemogenic potential in man of prolonged cytotoxic agents therapy, especially with alkylating agents, seems to be well established. This evidence admonishes against the prolonged use of these drugs in non-fatal disorders. 9. More accurate assessment of risk: benefit ratios awaits the results of prospective controlled studies. The results of these studies could also lead to significant modifications in recommendations for long-term maintenance therapy with cytotoxic agents.- - - - - - - - - - ranking = 0.33333333333333keywords = lymphoma (Clic here for more details about this article) |
4/149. Anaplastic large-cell lymphoma with rapid evolution to leukemic phase.Anaplastic large-cell lymphoma (ALCL) is a lymphoproliferative disorder that frequently presents with disseminated disease and extranodal involvement. Rare atypical cells have been detected in the peripheral blood in occasional cases. However, the presence of a prominent leukemic phase is extremely rare in these patients. We describe a patient with a small-cell variant of ALCL of T-cell phenotype, ALK-1 positive, who developed a rapid leukemic phase in association with the progression of the disease. Similar to the nodal biopsy, the predominant cells in bone marrow and peripheral blood were small atypical lymphoid cells. The large tumor cells expressed ALK immunoreactivity with a cytoplasmic and nuclear pattern, whereas some of the small cells showed only a nuclear-restricted pattern of staining. An RT-PCR study detected the NPM-ALK chimeric product in the nodal biopsy and in a peripheral blood sample in the early phase of the disease, but it became negative in a peripheral blood sample obtained after completion of the chemotherapy treatment, suggesting that this assay may be useful in the follow-up of these patients. This case indicates that a prominent leukemic phase may develop in ALCL as a manifestation of tumor dissemination and that it may be composed of a predominant small-cell atypical component.- - - - - - - - - - ranking = 1.6666666666667keywords = lymphoma (Clic here for more details about this article) |
5/149. Meningeal leukemia in the blastic phase of chronic granulocytic leukemia.One hundred one patients were treated for Ph' positive chronic granulocytic leukemia (CGL) in the blastic phase. In seven of these (6.9 per cent), meningeal leukemia developed. Of the 99 patients who died of their disease, a complete remission was achieved in 12 with a median survival of 12 months (three to 28 months). Incomplete responders had a median survival of only 2.5 months (one to 14 months). In five of the 12 complete responders (42 per cent), but in only two of the incomplete responders (2.3 per cent), meningeal leukemia developed. The principal neurologic signs were cranial nerve palsies and papilledema. All patients had pleocytosis with myeloblasts in the cerebrospinal fluid. As in patients with acute leukemia and diffuse histiocytic lymphoma, increased survival of patients in whom hematologic remission from the blastic phase of CGL is achieved may allow sufficient time for the development of meningeal leukemia. Intrathecal methotrexate is extremely successful in treating this complication. cerebrospinal fluid pleocytosis was eradicated in all seven of our patients, and neurologic symptoms and signs were completely eliminated in five patients. No evidence of meningeal leukemia was found in three of the five patients in whom an autopsy was performed.- - - - - - - - - - ranking = 0.33333333333333keywords = lymphoma (Clic here for more details about this article) |
6/149. splenic rupture in children with hematologic malignancies.BACKGROUND: splenic rupture is an uncommon but life-threatening complication of leukemias and lymphomas, and is reported mostly in adults. The authors investigated the frequency with which splenic rupture is diagnosed in pediatric patients with hematologic malignancies and reviewed its clinical profile and outcome. methods: The data base of St. Jude Children's research Hospital was searched for cases coded as splenic laceration or rupture, splenic infarction, or splenectomy in patients diagnosed with lymphoma or leukemia between January 1962 and December 1997. The medical records of patients with histopathologic or radiologic evidence of splenic rupture were reviewed. The time spanned by the study was divided into early (1962-1990) and recent (1991-1997) eras to reflect the availability of modern diagnostic imaging techniques. RESULTS: Seven children experienced splenic rupture. They were between ages 5-17 years. There were four males and three females. Primary diagnoses included acute myeloid leukemia (four patients), acute lymphoblastic leukemia (two patients), and Hodgkin lymphoma (one patient). Five patients were diagnosed in the recent era and two in the early era. Four patients had radiologic or bacteriologic evidence of fungal infection concomitant with the splenic event. Of five deaths, only two were related causally to splenic rupture; these occurred in the early era. All seven acute episodes of splenic rupture were managed conservatively without surgery. CONCLUSIONS: The overall frequency with which splenic rupture was detected in children with hematologic malignancy at the study institution was 0.18%. In the recent era, the frequency of detection was 9-fold higher (0.55%) than that of the early era (0.06%). Improved imaging techniques and increased utilization of imaging studies may account for the increased incidental detection of "preclinical" splenic rupture. adolescent age group, acute myeloid leukemia (especially acute promyelocytic leukemia), a high leukocyte count, thrombocytopenia, and coagulopathy may predispose children with leukemia to pathologic splenic rupture. Fungal infection frequently was associated with splenic rupture and may play a role in its pathogenesis.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
7/149. 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.- - - - - - - - - - ranking = 0.33333333333333keywords = lymphoma (Clic here for more details about this article) |
8/149. Coexistence of two distinct cell populations (CD56( )TcRgammadelta( ) and CD56( )TcRgammadelta(-)) in a case of aggressive CD56( ) lymphoma/leukemia.BACKGROUND AND OBJECTIVE: Large granular lymphocytes derive from two major lineages: one expressing the CD3 surface antigen (t-lymphocytes), and the other lacking this marker (NK-cells). Although developmental overlaps between natural killer cells and T-cells have been described, malignancies derived from these two cell types are considered as distinct lymphoid disorders. DESIGN AND methods: We report the case of a 30-year old man affected by a lymphoma/leukemia syndrome presenting with hepatosplenic lymphoma which rapidly transformed into aggressive NK-leukemia. Extensive flow cytometry studies and molecular analysis were repeated during the course of the disease, and showed an unexpected changing pattern. RESULTS: At diagnosis, flow cytometry analysis showed the co-existence of two cell populations, one CD56( ), CD3( ), TcRgd( ), and the other CD56( ), CD3(-) and TcRgd(-). Molecular analysis showed that the TcR genes had the same clonally rearranged pattern involving b, g and d genes in both populations. At disease relapse and during the terminal refractory phase, only CD3(-) cells were present. INTERPRETATION AND CONCLUSIONS: This is an unusual case of CD56( ) aggressive lymphoma/leukemia characterized by the clonal expansion of two phenotypically different cell populations, variably balanced during the course of the disease. The presence of the same TcR genomic rearrangement suggests the origin from a common progenitor able to differentiate along both T- and NK-pathways.- - - - - - - - - - ranking = 2.3333333333333keywords = lymphoma (Clic here for more details about this article) |
9/149. A case of lymphoma with T-cell characteristics.This case report concerns a woman of 54 who died from a rapidly progressive lymphosarcoma-cell leukaemia with the terminal complication of toxoplasmosis. The grossly atypical lymphosarcoma cells were shown to form rosettes with sheep erythrocytes but were incapable of responding to stimulation with phytohaemagglutinin. They thus had the functional characteristics of abnormal T-cells. The proportion of immunoglobulin-bearing lymphocytes was greatly reduced.- - - - - - - - - - ranking = 1.3333333333333keywords = lymphoma (Clic here for more details about this article) |
10/149. Relapse of leukemia and lymphoma after marrow transplant: a review of cases with extramedullary relapse.We review our cases of leukemia and lymphoma relapse after allogeneic marrow transplant and describe here a series of 10 patients with extramedullary (EM) relapse. In the 13 relapses in acute myeloid leukemia, 5 cases had EM involvement. There were 3 EM involvement out of 13 acute lymphoblastic leukemia relapses, one EM disease in 11 chronic myeloid leukemia relapses and one case of lymphoma with EM relapse. A common observation is that in some of these cases, EM relapse occurred in the presence of continuous marrow remission, In those cases with both marrow and EM involvement marrow remission could often be achieved and maintained temporarily while EM disease progressed despite chemotherapy or immunotherapeutic measures such as immunosuppressant withdrawal and donor lymphocyte infusion. survival in partial remission after relapse could be prolonged in some cases but eventual death from progressive disease was often the case.- - - - - - - - - - ranking = 2keywords = lymphoma (Clic here for more details about this article) |
| | Next -> |