Recommended evaluation/initial staging of the patient with mycosis fungoides/Sézary syndrome
| Complete physical examination including |
| Determination of type(s) of skin lesions |
| If only patch/plaque disease or erythroderma, then estimate percentage of body surface area involved and note any ulceration of lesions |
| If tumors are present, determine total number of lesions, aggregate volume, largest size lesion, and regions of the body involved |
| 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 |
| Most indurated area if only one biopsy |
| Immunophenotyping to include at least the following markers: CD2, CD3, CD4, CD5, CD7, CD8, 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. |
| Evaluation for clonality of TCR gene rearrangement |
| Blood tests |
| CBC with manual differential, liver function tests, LDH, comprehensive chemistries |
| TCR gene rearrangement and relatedness to any clone in skin |
| Analysis for abnormal lymphocytes by either Sézary cell count with determination absolute number of Sézary cells and/or flow cytometry (including CD4+/CD7− or CD4+/CD26−) |
| Radiologic tests |
| In patients with T1N0B0 stage disease who are otherwise healthy and without complaints directed to a specific organ system, and in selected patients with T2N0B0 disease with limited skin involvement, radiologic studies may be limited to a chest X-ray or ultrasound of the peripheral nodal groups to corroborate absence of adenopathy |
| In all patients with other than presumed stage IA disease, or selected patients with limited T2 disease and the absence of adenopathy or blood involvement, CT scans of chest, abdomen, and pelvis alone ± FDG-PET scan are 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 either ≥ 1.5 cm in diameter and/or is firm, irregular, clustered, or fixed |
| 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 (SUV). |
| If there is no additional imaging information and multiple nodes are enlarged and otherwise equal in size or consistency, the order of preference is cervical, axillary, and inguinal areas. |
| Analysis: pathologic assessment by light microscopy, flow cytometry, and TCR gene rearrangement. |
| Complete physical examination including |
| Determination of type(s) of skin lesions |
| If only patch/plaque disease or erythroderma, then estimate percentage of body surface area involved and note any ulceration of lesions |
| If tumors are present, determine total number of lesions, aggregate volume, largest size lesion, and regions of the body involved |
| 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 |
| Most indurated area if only one biopsy |
| Immunophenotyping to include at least the following markers: CD2, CD3, CD4, CD5, CD7, CD8, 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. |
| Evaluation for clonality of TCR gene rearrangement |
| Blood tests |
| CBC with manual differential, liver function tests, LDH, comprehensive chemistries |
| TCR gene rearrangement and relatedness to any clone in skin |
| Analysis for abnormal lymphocytes by either Sézary cell count with determination absolute number of Sézary cells and/or flow cytometry (including CD4+/CD7− or CD4+/CD26−) |
| Radiologic tests |
| In patients with T1N0B0 stage disease who are otherwise healthy and without complaints directed to a specific organ system, and in selected patients with T2N0B0 disease with limited skin involvement, radiologic studies may be limited to a chest X-ray or ultrasound of the peripheral nodal groups to corroborate absence of adenopathy |
| In all patients with other than presumed stage IA disease, or selected patients with limited T2 disease and the absence of adenopathy or blood involvement, CT scans of chest, abdomen, and pelvis alone ± FDG-PET scan are 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 either ≥ 1.5 cm in diameter and/or is firm, irregular, clustered, or fixed |
| 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 (SUV). |
| If there is no additional imaging information and multiple nodes are enlarged and otherwise equal in size or consistency, the order of preference is cervical, axillary, and inguinal areas. |
| Analysis: pathologic assessment by light microscopy, flow cytometry, and TCR gene rearrangement. |