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[CD56-positive peripheral T-cell lymphoma primarily presenting with tonsillar swelling]
Takayuki Tsujioka1, Hideho Wada, Shinichiro Suemori
1Division of Hematology, Department of Medicine, Kawasaki Medical School.
[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|November 24, 2004
Summary
This case report details a rare instance of CD56-positive T-cell lymphoma initially presenting in the tonsils, a location rarely associated with this aggressive lymphoma. Despite initial remission, the patient relapsed, highlighting the challenges in treating this specific lymphoma subtype.
Area of Science:
- Hematology and Oncology
- Immunology
- Pathology
Background:
- CD56-positive T-cell lymphoma is an aggressive hematologic malignancy with diverse clinical presentations.
- Primary involvement of the tonsils by CD56-positive T-cell lymphoma is exceptionally rare.
- Enteropathy-type T-cell lymphoma (ETL) typically involves the gastrointestinal tract, but extranodal presentations can occur.
Observation:
- A 51-year-old woman presented with tonsillar swelling, leading to a diagnosis of CD56-positive T-cell lymphoma.
- Immunohistochemistry and flow cytometry confirmed the malignant lymphocytes expressed CD3, CD8, CD56, TIA-1, and granzyme B.
- Southern blot analysis indicated T-cell receptor gamma chain gene rearrangement, confirming a T-cell origin.
Findings:
- The patient achieved complete remission (CR) after MCEC therapy and autologous peripheral blood stem cell transplantation (ASCT).
- Relapse occurred two months post-CR, manifesting as peritonitis due to ileal tumor perforation, leading to sepsis and death.
- Small bowel ulcers were identified during chemotherapy, suggesting a possible enteropathy-type T-cell lymphoma (ETL) with tonsillar origin.
Implications:
- This case underscores the importance of considering rare presentations of CD56-positive T-cell lymphoma, even in extranodal sites like the tonsils.
- The development of gastrointestinal symptoms and ulcers suggests a potential link to ETL, necessitating careful evaluation of the GI tract in similar cases.
- Further research is needed to elucidate the pathogenesis and optimize treatment strategies for CD56-positive T-cell lymphoma, particularly its rare extranodal and enteropathic forms.