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Donor derived T-cell large granular lymphocyte leukemia after cord blood transplant for pediatric T-cell lymphoblastic leukemia

Bone Marrow Transplantation volume 53, pages 352–355 (2018)Cite this article

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Large granular lymphocyte (LGL) leukemia is characterized by a clonal expansion of either CD3(+) cytotoxic T or CD3(−) NK cells. LGL leukemia most commonly affects the elderly with a median age of 60, although cases in younger adults and very rarely pediatric patients have been reported [1,2,3,4]. In most cases T-LGL leukemia is associated with an indolent clinical course characterized by cytopenias and autoimmune phenomena, with infections due to neutropenia being among the most common presenting symptoms [4,5,6,7]. LGL leukemia mortality is mainly due to severe infections, although this occurs in <10% of patients and the overall survival at 10 years is approximately 70% [8, 9]. Donor-derived T-LGL leukemia has been reported after hematopoietic stem cell transplant (HSCT) with both bone marrow and peripheral blood donor sources [4, 6]. T-LGL leukemia is a rare post-HSCT neoplasm that is rarely diagnosed in the pediatric population. Here we present the case of a female who underwent an unrelated umbilical cord blood transplant for T-cell acute lymphoblastic leukemia (T-ALL) and was subsequently diagnosed with donor-derived T-LGL at 11 years of age.

At 6 years of age, the patient was diagnosed with T-ALL, and had refractory disease with 25% T lymphoblasts in the bone marrow following induction chemotherapy. She then underwent salvage chemotherapy and achieved complete remission without detection of minimal residual disease by flow cytometry. She subsequently received an unrelated, 4/6 matched cord blood transplant (CBT) with single mismatches at HLA-B and DRB1. The preparative transplant conditioning regimen contained fludarabine, cyclophosphamide and total body irradiation (13.2 Gy) with cranial boost. Graft versus host disease (GVHD) prophylaxis included cyclosporine and mycophenolate mofetil. Early post-transplant, the patient developed acute skin grade IIB GVHD which was treated with high-dose prednisone. Following completion of an initial prednisone taper, she developed delayed acute gastrointestinal GVHD grade IIA that was treated with lower dose prednisone, beclomethasone, and budesonide. The patient developed a mild, intermittent skin rash which was treated with topical steroid ointment as needed. She also developed transaminitis 9 months after transplant in the setting of her cyclosporine taper that was presumed liver GVHD. She was transitioned to sirolimus and low-dose prednisone was restarted with good response. She ultimately completed systemic immune suppression approximately 14 months after transplant and aside from mild skin flares treated with topical immune suppression only, had been without signs or symptoms of chronic GVHD since that time.

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Acknowledgements

This investigation was supported by the National Institutes of Health under Ruth L. Kirschstein National Research Service Award T32CA009351.

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Authors and Affiliations

  1. Seattle Children’s Hospital, Seattle, WA, USA

    Tyler G. Ketterl & Katherine Tarlock

  2. University of Washington, Seattle, WA, USA

    David Wu, Jonathan R. Fromm, Lorinda Soma & Brent L. Wood

  3. Fred Hutchinson Cancer Research Center, Seattle, WA, USA

    Ann E. Dahlberg

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  1. Tyler G. Ketterl
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Correspondence to Tyler G. Ketterl.

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Ketterl, T.G., Wu, D., Fromm, J.R. et al. Donor derived T-cell large granular lymphocyte leukemia after cord blood transplant for pediatric T-cell lymphoblastic leukemia. Bone Marrow Transplant 53, 352–355 (2018). https://doi.org/10.1038/s41409-017-0037-9

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