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Two cases of refractory large granular lymphocytic leukemia treated by porcine anti-human thymocyte immunoglobulin.

Zeng Q, Wang H, Chang H - Chin. Med. J. (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China.

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To the Editor: Large granular lymphocytic leukemia (LGLL) is a rare chronic clonal lymphoproliferative disease characterized by a persistent increase in large granular lymphocytes, which are large lymphocytes with a low nucleus to cytoplasm ratio and an abundant cytoplasm with azurophilic granules... Then, FCM of marrow suggested that just a few clonal T-cell might be presented. 7 months later, FCM of her peripheral blood showed nucleated cells with 38% lymphocytes, 68% of which were clonal cytotoxic T-cell... FCM showed nucleated cells of 82% lymphocytes with an immunophenotype of CD2, CD3, CD7, CD8, CD57, CD11c, CD4, CD5, CD16, CD56, and restricted expression of TCR-γδ 7.1, suggesting large granular lymphoproliferative disorder... The first-line therapy of LGLL includes CsA, steroid, MTX and cyclophosphamide... But some patients who do not respond well to these drugs are refractory... For these patients, Lamy and Loughran suggested that purine analogs, such as Fludarabine or Campath, could be used or that investigational drugs should be considered... Although ATG can bind to T-cell and relieve inhibition of red linage, few refractory patients received the treatment... We searched OVID databases and found that patients with refractory LGLL were treated with horse ATG in 1980s; no patients have been treated by porcine ATG before... Our two refractory patients received porcine ATG therapy and achieved remission... One of them even achieved molecular remission... However, one patient relapsed afterwards, so long-term follow-up is also necessary to monitor the recurrence.

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Trend of changes in hemoglobin (Hb) and lymphocyte ratio (L) in Case 1 (a) and Case 2 (b). The arrow indicated the initiation of immunosuppressive therapy with antihuman thymocyte immunoglobulin. Both patients’ Hb and L% initially increased following the immunosuppressive therapy, but later decreased in Case 1 and maintained at safe level in Case 2.
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Figure 1: Trend of changes in hemoglobin (Hb) and lymphocyte ratio (L) in Case 1 (a) and Case 2 (b). The arrow indicated the initiation of immunosuppressive therapy with antihuman thymocyte immunoglobulin. Both patients’ Hb and L% initially increased following the immunosuppressive therapy, but later decreased in Case 1 and maintained at safe level in Case 2.

Mentions: A 44-year-old woman was admitted to the outpatient department with complaint of recurrent fatigue. The initial laboratory tests showed: Hemoglobin (Hb), 6.8 g/dl, reticulocytes (RET), 7.1 × 109/L. Bone marrow smears showed mature lymphocytes of 29.5%, granulocytic cells 68%, erythroid cells 4% and myeloid/erythroid (M/E) ratio 27.2:1. Flow cytometry (FCM) showed CD3+, CD8+, CD57±, CD56−, and restricted expression of T-cell receptor (TCR)-γδ3. She was diagnosed as LGLL. The patient then received the treatment of cyclosporin A (CsA), steroid, methotrexate (MTX) and cyclophosphamide, all of which were failed. However, after porcine ATG therapy, her Hb count increased to normal value [Figure 1a]. Then, FCM of marrow suggested that just a few clonal T-cell might be presented. 7 months later, FCM of her peripheral blood showed nucleated cells with 38% lymphocytes, 68% of which were clonal cytotoxic T-cell. She was identified to relapse.


Two cases of refractory large granular lymphocytic leukemia treated by porcine anti-human thymocyte immunoglobulin.

Zeng Q, Wang H, Chang H - Chin. Med. J. (2015)

Trend of changes in hemoglobin (Hb) and lymphocyte ratio (L) in Case 1 (a) and Case 2 (b). The arrow indicated the initiation of immunosuppressive therapy with antihuman thymocyte immunoglobulin. Both patients’ Hb and L% initially increased following the immunosuppressive therapy, but later decreased in Case 1 and maintained at safe level in Case 2.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4834792&req=5

Figure 1: Trend of changes in hemoglobin (Hb) and lymphocyte ratio (L) in Case 1 (a) and Case 2 (b). The arrow indicated the initiation of immunosuppressive therapy with antihuman thymocyte immunoglobulin. Both patients’ Hb and L% initially increased following the immunosuppressive therapy, but later decreased in Case 1 and maintained at safe level in Case 2.
Mentions: A 44-year-old woman was admitted to the outpatient department with complaint of recurrent fatigue. The initial laboratory tests showed: Hemoglobin (Hb), 6.8 g/dl, reticulocytes (RET), 7.1 × 109/L. Bone marrow smears showed mature lymphocytes of 29.5%, granulocytic cells 68%, erythroid cells 4% and myeloid/erythroid (M/E) ratio 27.2:1. Flow cytometry (FCM) showed CD3+, CD8+, CD57±, CD56−, and restricted expression of T-cell receptor (TCR)-γδ3. She was diagnosed as LGLL. The patient then received the treatment of cyclosporin A (CsA), steroid, methotrexate (MTX) and cyclophosphamide, all of which were failed. However, after porcine ATG therapy, her Hb count increased to normal value [Figure 1a]. Then, FCM of marrow suggested that just a few clonal T-cell might be presented. 7 months later, FCM of her peripheral blood showed nucleated cells with 38% lymphocytes, 68% of which were clonal cytotoxic T-cell. She was identified to relapse.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

To the Editor: Large granular lymphocytic leukemia (LGLL) is a rare chronic clonal lymphoproliferative disease characterized by a persistent increase in large granular lymphocytes, which are large lymphocytes with a low nucleus to cytoplasm ratio and an abundant cytoplasm with azurophilic granules... Then, FCM of marrow suggested that just a few clonal T-cell might be presented. 7 months later, FCM of her peripheral blood showed nucleated cells with 38% lymphocytes, 68% of which were clonal cytotoxic T-cell... FCM showed nucleated cells of 82% lymphocytes with an immunophenotype of CD2, CD3, CD7, CD8, CD57, CD11c, CD4, CD5, CD16, CD56, and restricted expression of TCR-γδ 7.1, suggesting large granular lymphoproliferative disorder... The first-line therapy of LGLL includes CsA, steroid, MTX and cyclophosphamide... But some patients who do not respond well to these drugs are refractory... For these patients, Lamy and Loughran suggested that purine analogs, such as Fludarabine or Campath, could be used or that investigational drugs should be considered... Although ATG can bind to T-cell and relieve inhibition of red linage, few refractory patients received the treatment... We searched OVID databases and found that patients with refractory LGLL were treated with horse ATG in 1980s; no patients have been treated by porcine ATG before... Our two refractory patients received porcine ATG therapy and achieved remission... One of them even achieved molecular remission... However, one patient relapsed afterwards, so long-term follow-up is also necessary to monitor the recurrence.

Show MeSH