Evaluation and staging |
Complete physical examination, including: |
Determination of type(s) of skin lesions |
Estimate percentage of BSA involved by patches, plaques, and tumor lesions and note any ulceration or folliculocentricity of lesions. The patient's palm (including all fingers) is approximately 1 percent of BSA.* |
Identification of any palpable lymph node, especially those ≥1.5 cm in largest diameter or firm, irregular, clustered, or fixed |
Identification of any organomegaly |
Skin biopsy |
Essential: At least two skin biopsies should be obtained of different anatomic areas and morphology for H&E evaluation. If only one biopsy can be obtained, then the lesion that has the most scaling and induration should be chosen. |
If needed: Immunophenotyping to include at least the following markers: CD2, CD3, CD4, CD5, CD7, and CD8, beta-F1 (to confirm alpha-beta TCR), and a B cell marker, such as CD20; CD30 may also be indicated in cases where lymphomatoid papulosis, anaplastic lymphoma, or large-cell transformation is considered. |
If needed: Evaluation for clonality of TCR gene rearrangement. |
Blood tests |
CBC with manual differential (assess for Sézary cells), comprehensive chemistries, LDH |
Analysis for abnormal lymphocytes by either Sézary cell count with determination of the absolute number of Sézary cells and/or flow cytometry (including CD4+ / CD7- or CD4+ / CD26-) |
If needed: TCR gene rearrangement and relatedness to any clone in skin |
Radiologic tests |
Radiologic tests are not necessary in patients with T1N0B0 or T2aN0B0 stage disease who are otherwise healthy and without complaints directed to a specific organ system. |
In all other patients, contrast-enhanced CT scan of chest, abdomen, and pelvis (neck included if clinically indicated) or whole body integrated PET-CT is recommended to further evaluate any potential lymphadenopathy, visceral involvement, or abnormal laboratory tests; in patients unable to safely undergo CT scans, MRI may be substituted. |
Lymph node biopsy |
Excisional biopsy is indicated in those patients with a node that is at least 1.5 cm (confirmed by imaging) in diameter; and/or is firm, irregular, clustered, or fixed; and/or significantly PET avid. |
Site of biopsy: Preference is given to the largest lymph node draining an involved area of the skin or if FDG-PET scan data are available, the node with highest standardized uptake value. |
Analysis: Pathologic assessment by light microscopy, flow cytometry, and TCR gene rearrangement. |
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