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Indolent T-lymphoblastic proliferation with disseminated multinodal involvement and partial CD33 expression
Robert S Ohgami1, Anoop K Sendamarai, Susan K Atwater
1Departments of *Pathology §Medicine, Stanford University Medical Center, Stanford, CA †Department of Pathology, Boston Children's Hospital ‡Harvard Medical School, Boston, MA.
The American Journal of Surgical Pathology
|March 13, 2014
Summary
Indolent T-lymphoblastic proliferations (iT-LBP) are rare but do not require chemotherapy, unlike aggressive forms. This case highlights disseminated iT-LBP in a healthy woman, showing significant improvement without treatment.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Indolent T-lymphoblastic proliferations (iT-LBP) are rare extrathymic T-cell neoplasms.
- Distinguishing iT-LBP from aggressive T-lymphoblastic leukemia/lymphoma is crucial as treatment differs significantly.
Observation:
- A 49-year-old woman presented with disseminated multinodal iT-LBP.
- Multiple lymph node biopsies revealed persistent, diffuse involvement over several months.
- The patient remained healthy with significant lymphadenopathy improvement over 18 months without therapy.
Findings:
- Atypical T cells exhibited partial CD33 expression, detected via flow cytometry and immunohistochemistry.
- T-cell clonality and Human Androgen Receptor Assay (HUMARA) studies were negative, indicating a nonclonal proliferation.
- No bone marrow or peripheral blood involvement was observed.
Implications:
- This case is the first detailed description of disseminated, nonclonal iT-LBP with partial CD33 expression.
- It underscores the importance of considering iT-LBP in extrathymic T-cell proliferations.
- The findings suggest that some disseminated iT-LBP cases may follow an indolent course without chemotherapy.

