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KMID : 1100820210110030183
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Laboratory Medicine Online
2021 Volume.11 No. 3 p.183 ~ p.190
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T-cell Large Granular Lymphocytic Leukemia Presenting as Post-transplant Lymphoproliferative Disorder: A Report of Two Cases and Literature Review
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Kim Si-Hwan
Cho Young-Uk
Jang Seong-Soo
Seo Eul-Ju
Park Chan-Jeoung
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Abstract
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T-cell large granular lymphocytic leukemia (T-LGLL) can present as a form of post-transplant lymphoproliferative disorder. Here, we described clinicopathological findings from 58 cases of post-transplant T-LGLL (our own 2 cases and 56 cases from literature), and compared clinical features between solid organ transplantation (SOT) and allogeneic hematopoietic stem cell transplantation (alloHSCT) groups. The first of our two cases involved a 39-year-old man diagnosed with T-LGLL and cytomegalovirus (CMV) colitis 81.7 months post liver transplantation. He had underlying primary immunodeficiency, and a probable germline IKZF1 mutation. The second case involved a 51-year-old man with ALL diagnosed with T-LGLL 1.8 months post alloHSCT. The patient tested positive for CMV DNA and the disease was of donor origin. Both patients were alive at the last follow-up, although they had persistent lymphocytosis. Overall, the median duration for T-LGLL onset after transplantation was 43.2 months, and the median number of large granular lymphocytes was 2.5×109/L. Nearly half of patients (46.0%) had CMV infection. Most patients (80.0%) of those whose clinical data were available showed good outcomes. The alloHSCT group showed significantly shorter latency (P<0.001) and a trend for higher frequency of CMV positivity (P=0.067) compared to the SOT group. Eleven patients (9 from the SOT and 2 from the alloHSCT group) showed autoimmune feature occurrence. This study reveals that CMV reactivation is a plausible driver for T-LGLL in the early phase after transplantation, and that the possibility of T-LGLL emergence should be considered, particularly for SOT patients with autoimmune features.
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KEYWORD
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T-cell large granular lymphocytic leukemia, Post-transplant lymphoproliferative disorder, Cytomegalovirus, Autoimmune disease
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