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T-cell large granular lymphocyte leukemia in solid organ transplant recipients: case series and review of the literature

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Abstract

T-cell large granular lymphocyte (T-LGL) leukemia is a rare clonal proliferation of cytotoxic lymphocytes rarely described in solid organ transplant (SOT). We reviewed records from 656 kidney transplant recipients in follow-up at our Center from January 1998 to July 2017. In addition, we researched, through PubMed, further reports of T-LGL leukemia in SOT from March 1981 to December 2017. We identified six cases of T-LGL leukemia in our cohort of patients and 10 in the literature. This lymphoproliferative disorder was detected in one combined liver–kidney, one liver and 14–kidney transplant recipients. Median age at presentation was 46.5 years (IQR 39.2–56.9). The disease developed after a median age of 10 years (IQR 4.9–12) from transplantation. Anemia was the most common presentation (62.5%) followed by lymphocytosis (43.7%) and thrombocytopenia (31.2%). Splenomegaly was reported in 43.7% of the patients. Eight patients (50%) who experienced severe symptoms were treated with non-specific immunosuppressive agents. Six of them (75%) had a good outcome, whereas two (25%) remained red blood cell transfusion dependent. No cases progressed to aggressive T-LGL leukemia or died of cancer at the end of follow-up. These results suggest that T-LGL leukemia is a rare but potentially disruptive hematological disorder in the post-transplant period.

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Abbreviations

AIHA:

Autoimmune hemolytic anemia

Anti-dsDNA:

Anti-double-stranded DNA

B19V:

Parvovirus B19

CMV:

Cytomegalovirus

EBV:

Epstein Barr virus

GFR:

Glomerular filtration rate

HTLV:

T cell lymphotropic virus

IQR:

Interquartile range

KT:

Kidney transplant

LGL:

Large granular lymphocyte

LGLs:

Large granular lymphocytes

MBL:

Monoclonal B cell lymphocytosis

MGUS:

Monoclonal gammopathy of undetermined significance

m-TORi:

Inhibitor of mammalian target of rapamycin

PRCA:

Pure red cell aplasia

PTLD:

Post-transplant lymphoproliferative disorder

SOT:

Solid organ transplant

TCR:

T-cell receptor

T-LGL:

T-cell large granular lymphocyte

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Authors and Affiliations

  1. Surgical, Medical and Dental Department of Morphological Sciences, Section of Nephrology, University of Modena and Reggio Emilia, Modena, Italy

    Gaetano Alfano, Caterina Cerami & Gianni Cappelli

  2. Nephrology Dialysis and Transplant Unit, University Hospital of Modena, Via del Pozzo, 71, 41124, Modena, Italy

    Gaetano Alfano, Francesco Fontana, Giacomo Mori & Gianni Cappelli

  3. Section of Hematology, Surgical, Medical and Dental Department of Morphological Sciences, University of Modena and Reggio Emilia, University Hospital of Modena, Modena, Italy

    Elisabetta Colaci, Andrea Messerotti, Leonardo Potenza & Mario Luppi

Authors
  1. Gaetano Alfano
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  2. Francesco Fontana
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  3. Elisabetta Colaci
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  4. Giacomo Mori
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  5. Caterina Cerami
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  7. Leonardo Potenza
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  8. Mario Luppi
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  9. Gianni Cappelli
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Contributions

All the Authors listed above met the following criteria recommended by International Committee of Medical Journal Editors. In particular: AG designed study. AG, FF and CE performed the study and wrote the paper. MA, MG, CC collected data. PL, LM and CG analyzed data and reviewed the paper. All Authors approved the final version of the manuscript to be published and were accountable for any part of the work.

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Correspondence to Gaetano Alfano.

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Alfano, G., Fontana, F., Colaci, E. et al. T-cell large granular lymphocyte leukemia in solid organ transplant recipients: case series and review of the literature. Int J Hematol 110, 313–321 (2019). https://doi.org/10.1007/s12185-019-02682-2

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