Abstract:OBJECTIVES:The aim of this retrospective study was to investigate the results of T-cell large granular lymphocytic leukemia treatment with fludarabine by assessing the complete hematologic response, the complete molecular response, progression-free survival, and overall survival.METHODS:We evaluated the records of six patients with T-cell large granular lymphocytic leukemia who were treated with fludarabine as a first-, second-, or third-line therapy, at a dose of 40 mg/m2, for three to five days per month and… Show more
“…There are varying methods of disease treatment, which includes chemotherapy, immunotherapy or hormonal therapy, and combinations of these therapies. Among patients requiring treatment, which is approximately 60%, the overall survival at five years is approximately 62% [7,8]. Among all patients diagnosed with LGL leukemia, the overall survival at 10 years is approximately 70% [1].…”
Large granular lymphocytic (LGL) leukemia is a rare, indolent disease that can cause destruction of neutrophils. We discuss the case of a previously healthy 63-year-old male who presented with severe, recurrent febrile neutropenia, in whom three bone marrow biopsies over 13 months failed to produce a diagnosis. He presented to our facility with persistent fevers and an absolute neutrophil count of 20 cells/mm 3 (reference range 1,700-7,000 cells/mm 3). A fourth bone marrow biopsy did not show clonal proliferation, but T-cell LGL leukemia was diagnosed based on the identification of T-cell rearrangements. We propose that LGL leukemia could be an underdiagnosed cause of severe neutropenia in patients with no overt malignancy or immunosuppressive therapy and that population-based database studies of patients with unexplained neutropenia may reveal more cases of this rare disease class.
“…There are varying methods of disease treatment, which includes chemotherapy, immunotherapy or hormonal therapy, and combinations of these therapies. Among patients requiring treatment, which is approximately 60%, the overall survival at five years is approximately 62% [7,8]. Among all patients diagnosed with LGL leukemia, the overall survival at 10 years is approximately 70% [1].…”
Large granular lymphocytic (LGL) leukemia is a rare, indolent disease that can cause destruction of neutrophils. We discuss the case of a previously healthy 63-year-old male who presented with severe, recurrent febrile neutropenia, in whom three bone marrow biopsies over 13 months failed to produce a diagnosis. He presented to our facility with persistent fevers and an absolute neutrophil count of 20 cells/mm 3 (reference range 1,700-7,000 cells/mm 3). A fourth bone marrow biopsy did not show clonal proliferation, but T-cell LGL leukemia was diagnosed based on the identification of T-cell rearrangements. We propose that LGL leukemia could be an underdiagnosed cause of severe neutropenia in patients with no overt malignancy or immunosuppressive therapy and that population-based database studies of patients with unexplained neutropenia may reveal more cases of this rare disease class.
“…alemtuzumab, anti-CD52, and the humanized MiK-beta1 monoclonal antibody), combination chemotherapy (CTX, doxorubicin, vincristine, prednisolone [CHOP]), and splenectomy have all been used in refractory/relapsed and treatment-naïve patients, but the response rates of these agents vary between studies. [19][20][21][22][23][24][25] Most physicians consider that high-dose combination chemotherapy regimens are relatively ineffective and are associated with more toxicity than benefits for this indolent disorder. Instead, chronic exposure to low-dose therapy seems more efficient than sequential high-dose combination chemotherapy, despite some patients being refractory to single oral MTX or CTX.…”
“…In the second patient, rituximab therapy was followed by immediate neutropenia recovery; then, one year later, the LGLL clone showed marked shrinkage [21]. Raposo et al and Verhoevan et al also described one and three cases, respectively, where rituximab therapy improved rheumatoid arthritis control and increased the neutrophil count, with sustained responses [20][21][22]. In the four cases of CD20+…”
Section: Discussionmentioning
confidence: 99%
“…Rituximab treatment was described in a few cases of T-LGLL, which were associated with rheumatoid arthritis, to control the autoimmune aspects of the disease [20][21][22]. In general, good responses were achieved with a monoclonal antibody against CD20.…”
T-cell large granular lymphocytic leukemia (T-cell LGLL) is a rare disorder characterized by the monoclonal expansion of CD3-positive cytotoxic T cells. Cell morphology and immunophenotyping are the main tools for diagnosis. Typical phenotypic findings include the expression of markers CD3+, CD5+dim, CD8+, CD16+, CD57+, and T-cell receptor (TCR) αβ (+). However, a few cases may present with the CD3+/CD4+ phenotype or the double expression of CD4 and CD8. Similarly, in 20% of cases, TCRγ/δ is positive. In this study, we aimed to describe a rare, unusual case of T-cell LGLL that exhibited expression of the B-cell antigen, CD20. Additionally, we performed a literature review to compare the clinical characteristics of our patient to those of other patients with CD20-positive T-cell LGLL and to seek new therapeutic possibilities.
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