title: Aggressive natural killer-like T-cell malignancy with leukemic presentation following solid organ transplantation.
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.
authors.affiliation: Department of Pathology, Stanford University School of Medicine, CA, USA.
authors.forename: Y
authors.initials: Y
authors.lastname: Natkunam
authors.forename: R A
authors.initials: RA
authors.lastname: Warnke
authors.forename: J L
authors.initials: JL
authors.lastname: Zehnder
authors.forename: P J
authors.initials: PJ
authors.lastname: Cornbleet
mesh_terms: D000328:Adult; D001773:Blood Cells / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D002874:Chromosome Mapping; D003937:Diagnosis, Differential; D005260:Female; D005434:Flow Cytometry; D016041:Heart-Lung Transplantation; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D016030:Kidney Transplantation; D007694:Killer Cells, Natural / Q000473:pathology*; D015458:Leukemia, T-Cell / Q000209:etiology* / Q000473:pathology*; D008875:Middle Aged; D016377:Organ Transplantation; D012333:RNA, Messenger / Q000378:metabolism; D013601:T-Lymphocytes / Q000473:pathology*
chemical_list: D012333:RNA, Messenger