Spontaneously relapsing clonal, mucosal cytotoxic T-cell lymphoproliferative disorder: case report and review of the literature.
Primary T-cell lymphoma of the gastrointestinal tract is a rare and usually aggressive disorder that may be associated with celiac disease. The authors describe a unique case of a clonal proliferation of CD8+ T cells involving the oral mucosa, ileum, and colon of a 35-year-old man that has regressed spontaneously and recurred numerous times over a 9-year period without treatment. The patient's symptoms were limited to occasional rectal bleeding and recurring painful oral ulcers. Within the intestine, these collections of small T cells induced minimal architectural distortions and did not show extensive epitheliotrophism. Polymerase chain reaction and sequencing analyses revealed that the identical T-cell clone has been present for more than 9 years and in different mucosal locations in this patient. This may represent a unique T-cell lymphoproliferative process akin to a mucosal counterpart of lymphomatoid papulosis of the skin.
Ranheim
E A
EA
Department of Pathology, Stanford University Medical Center, CA 94305, USA.
Jones
C
C
Zehnder
J L
JL
Warnke
R
R
Yuen
A
A
D000328:Adult; D001483:Base Sequence; D014408:Biomarkers, Tumor / Q000032:analysis; D002999:Clone Cells; D017931:DNA Primers / Q000737:chemistry; D004273:DNA, Neoplasm / Q000032:analysis; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D006801:Humans; D007124:Immunoenzyme Techniques; D007413:Intestinal Mucosa / Q000473:pathology*; D007414:Intestinal Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D008297:Male; D008969:Molecular Sequence Data; D009061:Mouth Mucosa / Q000473:pathology*; D009062:Mouth Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D009365:Neoplasm Regression, Spontaneous / Q000235:genetics / Q000473:pathology*; D016133:Polymerase Chain Reaction; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics; D013602:T-Lymphocytes, Cytotoxic / Q000473:pathology*
D014408:Biomarkers, Tumor; D017931:DNA Primers; D004273:DNA, Neoplasm; D016693:Receptors, Antigen, T-Cell, alpha-beta