Lab Med Online.  2021 Jul;11(3):183-190. 10.47429/lmo.2021.11.3.183.

T-cell Large Granular Lymphocytic Leukemia Presenting as Post-transplant Lymphoproliferative Disorder: A Report of Two Cases and Literature Review

• Kim S
• Cho YU
• Jang S
• Seo EJ
• Park CJ

• KMID: 2526066
• DOI: http://doi.org/10.47429/lmo.2021.11.3.183

Abstract

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.

Keyword

T-cell large granular lymphocytic leukemia; Post-transplant lymphoproliferative disorder; Cytomegalovirus; Autoimmune disease

Figure

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  Fig. 1 Representative images of case 21 of Table 1. (A) A peripheral blood smear shows typical large granular lymphocytes with moderate cytoplasm and fine azurophilic granules. (B) Flow cytometric analysis shows a predominant population of CD4-/CD8+ lymphocytes. (C) Capillary electrophoresis assay for clonal TCRG rearrangements shows a prominent positive peak within the valid size range, which is consistent with the presence of a clonal cell population.

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