1/5. Aggressive variant of morphologically typical T large granular lymphocyte leukemia/lymphoma lacking NK cell markers.A 46-year-old woman with a previous diagnosis of sarcoidosis presented with morphologically typical large granular lymphocyte (LGL) leukemia/lymphoma with an aggressive clinical course. Epstein-Barr virus dna was detected in peripheral blood mononuclear cells by PCR. The phenotype was typical of the T cell lineage (CD2 CD3 CD5 CD7 CD8 TCRalphabeta ) but with the absence of the CD16, CD56, CD57 NK cell markers. In addition, the LGLs expressed CD122 (p75) in the absence of CD25 which is characteristic of LGLs. These leukemic LGLs did not exhibit NK activity. The clonal nature of this proliferation was demonstrated by the rearrangement of the TCRgamma gene. This phenotypically unusual but morphologically typical LGL leukemia/lymphoma may represent the clonal expansion of a minor normal subset of T-LGLs which do not express any NK cell markers, probably corresponding to in vivo activated T cells.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/5. Treatment of T-prolymphocytic leukemia with nonmyeloablative allogeneic stem cell transplantation.AIM: T-prolymphocytic leukemia (T-PLL) is a rare disease of the elderly characterized by a high white blood cell count and organomegaly, and is currently incurable. Our aim was to elicit graft-versus-leukemia reactions in a patient with T-PLL. methods: A 52-yr-old woman with refractory T-PLL underwent a nonmyeloablative regimen followed by allogeneic peripheral blood stem cell transplantation (a "minitransplant") from her HLA-matched sibling. RESULTS: There was no treatment related toxicity other than neutropenia. Engraftment was successful. The patient experienced no graft-versus-host disease (GVHD) at any time but, on day 84 after transplantation, had a relapse in the central nervous system. Despite infusion of donor lymphocytes and intralumbar chemotherapy, she died on day 157 of systemic disease. CONCLUSION: The reasons why treatment may have failed are discussed (nature of disease, disease progression, treatment schedule).- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/5. Lessons from a case of T-cell large granular lymphocytic leukaemia suggesting that immunomodulatory therapy is more effective than intensive treatment.leukemia treatment strives to eradicate the malignant clone. With T-cell large granular lymphocytic (LGL) leukemia, the onus of treatment appears to be modification of the disease rather than eradication of the clone. We describe a case of T-cell LGL leukemia where aggressive, eradicative type therapy proved ineffective. The patient achieved hematological response to low dose oral methotrexate after failing to respond to and/or tolerate eight previous treatments including CAMPATH-1H and peripheral blood stem cell transplantation. We highlight the resistant nature of the LGL clone and discuss the relative merits of immunomodulatory type therapy in this disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/5. Poorly expressed CD2 antigen on the leukemic cells of adult T-cell leukemia and chronic lymphocytic leukemia of T-cell lineage.adult T-cell leukemia (ATL) and T-cell chronic lymphocytic leukemia (T-CLL) are hematologic neoplasms in differentiated stages of peripheral mature T cells. This is suggested by the presence of CD2 (E rosette receptor) and mature and/or pan-T cell membrane surface antigens on their leukemic cells. We recently encountered one patient with ATL and another with T-CLL; their leukemic cells poorly expressed CD2 antigens, but clinical presentation, morphology of the leukemic cells and other marker studies were characteristic of either ATL or CLL. The clinical significance of the poor expression of CD2 remains to be further studied. The two patients reported here died of severe complications 4 and 9 weeks after diagnosis. The poor expression of CD2 in the peripheral T cells in these neoplasms is likely to indicate an aggressive nature of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/5. clostridium septicum bacteremia in a patient with large granular lymphocyte leukemia.This is the first case report of clostridium septicum septicemia in a patient with large granular lymphocyte leukemia. C. septicum infection is highly associated with malignancy and causes a rapidly fatal enterocolitis among patients who are profoundly neutropenic. The need for early recognition and combination of early antibiotic therapy and necessary surgical intervention may help to alter the fulminating nature of C. septicum infection.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |