Diagnosis of Central Nervous System (CNS) Involvement of T Cell Lymphoproliferative Diseases from Cerebrospinal Fluid (CSF) with Combined Cytology, Flow Cytometry and Lymphoid Antigen-Cell Receptor Antigen Rearrangement Studies.
Yaxia Zhang, Parvin Ganjei-Azar, Phillip Ruiz. Univeraity of Miami-Jackson Memorial Hospital, FL; University of Miami, FL
Background: Leptomeningeal involvement of the CNS by a T cell lymphoproliferative disorder tends to be allied with a worse prognosis. The diagnosis of intrathecal and CNS involvement is often defined by the clinical presentation and/or the presence of neoplastic cells in the CSF cytology specimen. However, cytological recognition of neoplastic lymphoreticular cells in CSF is often difficult and the sensitivity can be rather low. Ancillary studies of CSF such as flow cytometry and PCR evaluation of lymphoid cell antigen-receptor gene rearrangement are often helpful. The aim of this study was to investigate whether cytofluorographic and molecular studies increase the sensitivity and specificity of diagnosis of lymphoproliferative disorders involving the CNS.
Design: Cases with positive T-cell antigen-receptor rearrangement from CSF over the last 7 years were reviewed. The clinical histology, cytology results, flow cytometry and PCR gene rearrangement studies were reviewed and compared.
Results: Twenty-five (25) cases with 38 samples of CSF showed T-cell receptor antigen rearrangement. Among them, 20 (75%) patients (32 out of 38 samples, 84.2%) presented with clinical signs such as vomiting, lethargy, with morphology of a lymphoproliferative disorder either T cell (14 cases) or B cell (6 cases). The cytology specimen showed blast or atypical lymphocytic process in 6 out of 25 cases (24%), 15 out of 38 samples (39.5%). Flow cytometry revealed abnormality in 9 out of 25 cases (36%), 18 out of 38 samples (47.4%). 5 cases patients do not have any lymphoproliferative disorders. (1 patient has squamous cell carcinoma, 2 HIV infection, 1 sarcoidosis, 1 optic neuritis).
Conclusions: Using ancillary studies such flow cytometry and PCR for T cell receptor antigen rearrangement increases the sensitivity of diagnosis of CNS involvement of T lymphoproliferative disorders over standard cytology. However, when analyzing the results, pathologists have to be aware of the potential for false positivity of the molecular study. Thus, clinicians and pathologists have to put together the entire picture including the clinical symptoms, cytology, flow cytometry and molecular studies when making a diagnosis of a T lymphoproliferative disorder involving the CNS.
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 70, Wednesday Afternoon