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Sézary syndrome represents the disseminated, erythrodermic, leukemic form of mycosis fungoides, a primary cutaneous T-cell lymphoma; it is characterized by significant blood involvement and lymphadenopathy.
Tests generally appear in the order most useful for common clinical situations
| Test name: CBC with Platelet Count & Automated Differential |
| ARUP #: 0040003 |
| Methodology: Automated Cell Count with Flow Cell Differential |
| Use: Manual differential may detect Sézary cells |
| Test name: Manual Differential |
| ARUP #: 0040005 |
| Methodology: Microscopic |
| Use: Use to detect Sézary cells |
| Limitations: Requires relatively large numbers of neoplastic cells (>20% of lymphocytes) due to morphologic overlap with benign lymphocytes |
| Test name: Leukemia/Lymphoma Phenotyping (Comprehensive - Whole Blood) |
| ARUP #: 0096299 |
| Methodology: Flow Cytometry |
| Use: Screen for presence of abnormal T-cells Antigens included: T-cell: CD1, CD2, CD3, CD4, CD5, CD7, CD8, TCR alpha-beta, TCR gamma-delta, Cytoplasmic CD3 |
| Test name: T-Cell Clonality Screening Assay by PCR, Fluid |
| ARUP #: 0055567 |
| Methodology: Polymerase Chain Reaction/Fragment Analysis |
| Use: Characterize abnormal T-cells Confirm results from phenotyping testing |
| Limitations: “Not detected” PCR screening results should be terminally analyzed by restriction fragment Southern blot hybridization (RF-SBH) to definitively exclude T-cell monoclonality |
| Test name: Neoplastic Mature T-Cell Evaluation by Flow Cytometry |
| ARUP #: 0093000 |
| Methodology: Flow Cytometry |
| Use: Monitor treatment response and follow levels of neoplastic T-cells in patients previously diagnosed with Sézary syndrome Suggest ordering along with TCR V-Beta flow cytometry Cost-effective test for Sézary syndrome |
| Limitations: Should not be used alone for diagnosis of T-cell malignancy; results should always be correlated with clinicopathologic data Neoplastic T-cells must show definitive phenotypic aberrancies relative to normal T-cells |
| Test name: T-Cell Clonality by Flow Cytometry Analysis of TCR V-Beta |
| ARUP #: 0093199 |
| Methodology: Flow Cytometry |
| Use: Further characterize phenotypically abnormal T-cell populations identified by flow cytometry and identify evidence of monoclonality based on expression of T-cell antigen receptor beta chain variable regions (TCR V-Beta) Suggest ordering along with Neoplastic Mature T-Cell Evaluation flow cytometry |
| Test name: T-Cell Clonality Assessment by Restriction Fragment-Southern Blot Hybridization, Tissue |
| ARUP #: 0055594 |
| Methodology: Restriction Fragment Southern Blot Hybridization |
| Use: Aid in typing of T-cell lymphomas |
| Test name: T-Cell Clonality Assessment by Restriction Fragment-Southern Blot Hybridization, Fluid |
| ARUP #: 0055596 |
| Methodology: Restriction Fragment Southern Blot Hybridization |
| Use: Aid in typing of T-cell lymphomas |
| Test name: Sezary Cell Exam |
| ARUP #: 0049180 |
| Methodology: Stain |
| Use: Diagnose Sézary syndrome in patient with mycosis fungoides Not a first-line test in diagnosis |
| Limitations: Requires relatively large numbers of neoplastic cells (>20% of lymphocytes) due to morphologic overlap with benign lymphocytes |
| Test name: CD3 by Immunohistochemistry |
| ARUP #: 2003508 |
| Methodology: Immunohistochemistry |
| Use: Aid in histologic diagnosis of Sézary syndrome Stained and returned to client pathologist for interpretation; consultation available if needed |
| Test name: CD8 by Immunohistochemistry |
| ARUP #: 2003520 |
| Methodology: Immunohistochemistry |
| Use: Aid in histologic diagnosis of Sézary syndrome Stained and returned to client pathologist for interpretation; consultation available if needed |
| Test name: CD20, L26 by Immunohistochemistry |
| ARUP #: 2003532 |
| Methodology: Immunohistochemistry |
| Use: Aid in histologic diagnosis of Sézary syndrome Stained and returned to client pathologist for interpretation; consultation available if needed |
| Test name: CD4 by Immunohistochemistry |
| ARUP #: 2003511 |
| Methodology: Immunohistochemistry |
| Use: Aid in histologic diagnosis of Sézary syndrome Stained and returned to client pathologist for interpretation; consultation available if needed |
| Test name: Comprehensive Metabolic Panel |
| ARUP #: 0020408 |
| Methodology: Refer to individual components |
| Use: Assess visceral organ involvement |
| Test name: Lactate Dehydrogenase, Serum or Plasma |
| ARUP #: 0020006 |
| Methodology: Quantitative Enzymatic |
| Use: Determine bone involvement |
| Test name: Leukemia/Lymphoma Phenotyping (Comprehensive - Bone Marrow) |
| ARUP #: 0095244 |
| Methodology: Flow Cytometry |
| Comments: |
| Test name: Leukemia/Lymphoma Phenotyping (Comprehensive - Miscellaneous) |
| ARUP #: 0095243 |
| Methodology: Flow Cytometry |
| Comments: |
| Test name: CD2 by Immunohistochemistry |
| ARUP #: 2003505 |
| Methodology: Immunohistochemistry |
| Comments: |
| Test name: CD5 by Immunohistochemistry |
| ARUP #: 2003514 |
| Methodology: Immunohistochemistry |
| Comments: |
| Test name: CD7 by Immunohistochemistry |
| ARUP #: 2003517 |
| Methodology: Immunohistochemistry |
| Comments: |
| Test name: CD30 (Ki-1) by Immunohistochemistry |
| ARUP #: 2003547 |
| Methodology: Immunohistochemistry |
| Comments: |
| Test name: CD56 (NCAM) by Immunohistochemistry |
| ARUP #: 2003589 |
| Methodology: Immunohistochemistry |
| Comments: |