1/135. Fas gene mutation in the progression of adult T cell leukemia.Fas antigen (Apo-1/CD95) is an apoptosis-signaling cell surface receptor belonging to the tumor necrosis factor receptor superfamily. adult T cell leukemia (ATL) cells express Fas antigen and show apoptosis after treatment with an anti-Fas monoclonal antibody. We established the ATL cell line KOB, which showed resistance to Fas-mediated apoptosis, and found that KOB expressed two forms of Fas mRNA, the normal form and a truncated form. The truncated transcript lacked 20 base pairs at exon 9, resulting in a frame shift and the generation of a premature stop codon at amino acid 239. The same mutation was detected in primary ascitic cells and peripheral blood cells. The mutation was not detected in lymph node cells, however, although all of the primary ATL cells were of the same clonal origin. A retroviral-mediated gene transfer of the truncated Fas to jurkat cells rendered the cells resistant to Fas-mediated apoptosis, suggesting a dominant negative interference mechanism. These results indicate that an ATL subclone acquires a Fas mutation in the lymph nodes, enabling the subclone to escape from apoptosis mediated by the Fas/Fas ligand system and proliferate in the body. mutation of the Fas gene may be one of the mechanisms underlying the progression of ATL.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/135. pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia.We describe the case of a 53-year-old Philadelphia-chromosome-positive woman with chronic myelogenous leukemia, who developed pulmonary alveolar proteinosis (PAP). The possible mechanism involved in the pathogenesis of PAP are discussed based on the clinical and laboratory data for this patient as well as on experimental and clinical data reported in the literature.- - - - - - - - - - ranking = 1.2223076356264keywords = leukemia, myelogenous, myelogenous leukemia (Clic here for more details about this article) |
3/135. Translocation (4;15)(p16;q24): a novel reciprocal translocation in a patient with BCR/ABL negative myeloproliferative syndrome progressing to blastic phase.A patient with BCR/ABL negative myeloproliferative syndrome with a 46,XY,del(3)(q21), t(4;15)(p16;q24) karyotype is described. fluorescence in situ hybridization performed with chromosomes 4 and 15 painting probes confirmed a novel reciprocal (4;15) translocation. The absence of crkl tyrosine phosphorylation, no activation of the abl kinase as measured by autophosphorylation, and a normal-size abl transcript suggest an alternative mechanism for leukemogenesis to that operative in Ph positive BCR/ABL positive chronic myeloid leukemia. A number of genes potentially relevant to tumorigenesis, some involving the ras signaling pathway, map to the 4p16 and 15q24 chromosome regions.- - - - - - - - - - ranking = 0.21578691647257keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
4/135. p190 BCR-ABL rearrangement in chronic myeloid leukemia and acute lymphoblastic leukemia.A minority of chronic myeloid leukemia (CML) cases have breakpoint in the minor cluster region (m-bcr) of the BCR-ABL fusion gene. We report a patient with Ph-positive acute lymphoblastic leukemia and m-bcr breakpoint at diagnosis. The patient was treated with chemotherapy followed by an autologous peripheral blood stem cell transplantation, achieving a clinical and hematological complete remission but with persistence of the philadelphia chromosome. One year later, she developed leukocytosis with a blood picture consistent with CML. She was treated with hydroxyurea and interferon alpha with no response. This is the second case of m-bcr CML reported presenting with features of lymphoid blast crisis or acute lymphoblastic leukemia.- - - - - - - - - - ranking = 2.2789345823628keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
5/135. Myelodysplastic syndrome that progressed to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormality: a case report.Myelodysplastic syndrome is a closely related group of acquired bone marrow disorders characterized by ineffective and dysplastic hematopoiesis. These clonal disorders frequently progress to acute leukemia. Acute myelomonocytic leukemia with eosinophilia is characterized by an increase in abnormal eosinophils in the bone marrow, relatively good clinical course and inv (16) chromosomal abnormality. We experienced one case of refractory anemia with excess blasts which progressed to refractory anemia with excess blasts in transformation and finally to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormalities of der (1;7).- - - - - - - - - - ranking = 1.4keywords = leukemia (Clic here for more details about this article) |
6/135. Smoldering acute myelogenous leukemia in the elderly.Out of 75 consecutive elderly AML patients who did not receive anti-leukemic treatment (52 pts) or failed to respond to differentiating agent (23 pts), 6 patients had survivals of 13.2 to 98 months with treatment restricted to supportive care. This cut-point is far longer than the median survival of the 235 elderly patients (3.5 mo.), either untreated (med. survival: 1 mo.) or treated (with treatment ranging from conventional induction to palliative chemotherapy) (4 mo.), admitted to our department within the same period of time. These cases of smoldering AML (4 women, 2 men) were all of AML2 FAB subtype (4 de novo, 2 post MDS) and presented with a significantly better performance status, lower WBC and circulating blast counts, higher platelet counts and with lower bone marrow infiltration than AML cases with more rapid progression. Cytogenetical analysis when available (3 pts) showed normal karyotypes and clonogenic assay performed in 3 of these patients showed a lack of (2 pts) or reduced in vitro leukemic cell growth (1 pt). The identification of specific characteristics of smoldering leukemia in the elderly might be an important development in the understanding of the physiopathology of acute leukemia and a tool for helping decision-making when selecting the time and intensity of cytotoxic treatment in these older patients.- - - - - - - - - - ranking = 1.3778461085011keywords = leukemia, myelogenous, myelogenous leukemia (Clic here for more details about this article) |
7/135. cladribine treatment of a patient with hairy cell leukemia and concomitant multiple sclerosis.This is the first report on a patient suffering from both multiple sclerosis (MS) and hairy cell leukemia. The patient was first treated with interferon-alpha. Due to disease progression two courses of cladribine were given resulting in an improvement of the clinical course of both diseases. Interestingly, it was possible to arrest and even ameliorate the progression of MS by administering as little as 30% of the dosage recently recommended for the treatment of this disease.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
8/135. Blastic/blastoid transformation of follicular lymphoma: immunohistologic and molecular analyses of five cases.Progression of follicular lymphoma to a higher-grade malignancy frequently heralds a poor prognosis. Clinical transformation is variably accompanied by a spectrum of histologic changes characterized by alteration in growth and cytology. Although several cytogenetic events and potential oncogenes have been documented in this progression, the underlying molecular mechanisms are largely unknown. We present five patients with an unusual histologic transformation of follicular lymphoma manifested by blastic/blastoid morphology. This transformation is histologically distinct from other types of transformation of follicular lymphoma. All five cases exhibited the t(14;18) translocation and expressed the BCL-2 protein. In addition, two of the five patients showed increased levels of the p53 protein within neoplastic cells implicating a possible role for this oncogene in blastic/blastoid transformation. The lack of BCL-1 and myeloid antigens by immunohistochemistry and flow cytometry studies served to distinguish blastic/blastoid transformation of follicular lymphoma from its morphologic mimics. This distinction is clinically important because lymphoblastic and myeloid leukemias require significantly different therapeutic modalities and show better prognosis. Moreover, the lack of Epstein-Barr virus-specific mRNA suggests that this virus is unlikely to participate in blastic/blastoid transformation of follicular lymphoma.- - - - - - - - - - ranking = 0.21578691647257keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
9/135. Acute myelogenous leukaemia and myelomonocytic blast crisis following polycythemia vera in hiv positive patients: report of cases and review of the literature.BACKGROUND: Acute myelogenous leukaemia (AML) and myeloproliferative diseases are rare in hiv-infected individuals and optimal treatment has not been defined. patients AND methods: We report on the cases of two hiv-infected men, one with AML and one with myeloid blast crisis after polycythaemia vera (PV). A comprehensive review of the available literature will be presented. RESULTS: Patient 1, a 57-year-old bisexual man known to be hiv seropositive for more than four years (CDC-category A1), presented with a pulmonary infiltrate. On admission WBC showed leukocytes 5.6 x 10(9)/l and the differential revealed 80% blasts. A diagnosis of AML FAB M0 was made. pneumonia resolved under antibiotic treatment and the patient received induction chemotherapy. However, he once more developed multiple pulmonary infiltrates and died of respiratory failure despite broad spectrum antibiotic and antimycotic therapy. autopsy revealed pulmonary aspergillosis. Patient 2 was a 63-year old hiv-positive hemophiliac (CDC A3) with a 10-year history of PV. On admission his white cell count showed leukocytes 256.6 x 10(9)/l with 82% blasts. Cytochemistry revealed myelomonocytic differentiation. The patient died of tumor lysis syndrome with renal and cardio-pulmonary failure two days later. CONCLUSIONS: This is the first report of an hiv-infected individual with AML M0. The literature describes the cases of 39 hiv patients with AML and only one further case with PV. The association of both, myeloproliferative disease and AML with hiv infection is coincidental. However, the proportion of FAB type M4/5 appears to be higher than in the general population. Despite a high risk of treatment associated mortality durable remissions can be achieved in a small proportion of hiv-infected patients with AML.- - - - - - - - - - ranking = 0.16560669942111keywords = myelogenous (Clic here for more details about this article) |
10/135. 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 = 1.4keywords = leukemia (Clic here for more details about this article) |
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