Aggressive natural killer-like T-cell malignancy with leukemic presentation following solid organ transplantation

Am J Clin Pathol. 1999 May;111(5):663-71. doi: 10.1093/ajcp/111.5.663.

Abstract

NK-like T-cell malignancies are part of a spectrum of lymphoproliferative diseases that complicate immunosuppression associated with solid organ transplantation. We describe 2 patients with long-standing immunosuppression following solid organ transplantation. Both patients had systemic symptoms that included fever, myalgia, and weight loss. Organ involvement and lymphadenopathy were not initially observed. Unique to these 2 cases are the initial leukemic symptoms, which led to further characterization and identification of NK-like T-cell malignancies. Both patients exhibited an anomalous T/NK phenotype, CD56 positivity, and atypical blastic architecture of the large granular lymphocytes. Clonal rearrangement of T-cell receptor genes was detected in both patients. In 1 patient, a cytogenetic abnormality involving 8q24 was demonstrated. The disease course in both patients was aggressive, with involvement of multiple sites and rapid demise. This study emphasizes the importance of including NK-like T-cell malignancies in the differential diagnosis of lymphoproliferative disorders associated with immunosuppression and recognizing that an aggressive clinical course may follow leukemic presentation of disease.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blood Cells / pathology
  • Bone Marrow / pathology
  • Chromosome Mapping
  • Diagnosis, Differential
  • Female
  • Flow Cytometry
  • Heart-Lung Transplantation
  • Herpesvirus 4, Human / genetics
  • Humans
  • Immunohistochemistry
  • Immunosuppression Therapy / adverse effects*
  • Kidney Transplantation
  • Killer Cells, Natural / pathology*
  • Leukemia, T-Cell / etiology*
  • Leukemia, T-Cell / pathology*
  • Middle Aged
  • Organ Transplantation*
  • RNA, Messenger / metabolism
  • T-Lymphocytes / pathology*

Substances

  • RNA, Messenger