Diagnosis of CTCL is based on the combination of clinical, histopathological, immunopathologic, and molecular biological findings.18
As CTCL can mimic other disease states, delays in diagnosis are not uncommon. One long-term study found that the median time to diagnosis is 4.2 years.11
Complete skin examination and biopsy with immunohistochemical studies of suspicious sites are necessary to confirm a diagnosis. In the absence of a definitive skin diagnosis, suspicious lymph node biopsy and peripheral blood assessment for Sézary cells are recommended.18
Patients with early stage disease and without evidence of lymphadenopathy, peripheral blood involvement, or unfavorable features need only a chest X-ray. Other patients should undergo either a CT or a PET/CT scan of the neck/chest/abdomen/pelvis.18
Bone marrow biopsy may be helpful in patients with an unexplained hematological abnormality or suspected bone marrow involvement.18
If Sézary syndrome is suspected, T-cell receptor gene rearrangement analysis of peripheral blood lymphocytes is recommended.18
A revised CTCL staging system has been developed by the International Society of Cutaneous Lymphomas (ISCL) and the European Organization of Research and Treatment of Cancer (EORTC).
In this system, all staged patients should have a definitive diagnosis of mycosis fungoides (MF) or Sézary Syndrome (SS).14
*For skin, patch indicates any size skin lesion without significant elevation or induration. Presence/absence of hypo- or hyperpigmentation, scale, crusting, and/or poikiloderma should be noted.
†For skin, plaque indicates any size skin lesion that is elevated or indurated. Presence or absence of scale, crusting, and/or poikiloderma should be noted. Histologic features such as folliculotropism or large-cell transformation (>25% large cells), CD30+ or CD30-, and clinical features such as ulceration are important to document.
‡For skin, tumor indicates at least one 1-cm diameter solid or nodular lesion with evidence of depth and/or vertical growth. Note total number of lesions, total volume of lesions, largest size lesion, and region of body involved. Also note if histologic evidence of large-cell transformation has occurred. Phenotyping for CD30 is encouraged.
§For node, abnormal peripheral lymph node(s) indicates any palpable peripheral node that on physical examination is firm, irregular, clustered, fixed, or 1.5 cm or larger in diameter. Node groups examined on physical examination include cervical, supraclavicular, epitrochlear, axillary, and inguinal. Central nodes, which are not generally amenable to pathologic assessment, are not currently considered in the nodal classification unless used to establish N3 histopathologically.
¶For viscera, spleen and liver may be diagnosed by imaging criteria.
║For blood, Sézary cells are defined as lymphocytes with hyperconvoluted cerebriform nuclei. If Sézary cells are not able to be used to determine tumor burden for B2, then one of the following modified ISCL criteria along with a positive clonal rearrangement of the TCR may be used instead: (1) expanded CD4+ or CD3+ cells with CD4/CD8 ratio of 10 or more, (2) expanded CD4+ cells with abnormal immunophenotype including loss of CD7 or CD26.
#A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor gene.
Approximately 1,500 people are newly diagnosed with CTCL in the United States annually.2
The average age at diagnosis is 55 years.2
Men are twice as likely as women to get the disease.2
Important prognostic factors include the patient’s age at initial presentation, type and extent of skin involvement, overall stage, peripheral blood involvement, and presence of extracutaneous disease.18
People who have early stage (Stage IA) CTCL have excellent prognoses, with life expectancies similar to gender- and age-matched controls.17 However, people with more advanced CTCL have been shown to have a poorer prognosis.11
In a long-term study of 525 patients by Kim and colleagues, the median survival by stage was:11
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Methoxsalen Sterile Solution is indicated for extracorporeal administration with the THERAKOS™ UVAR XTS® or THERAKOS™ CELLEX® Photopheresis System in the palliative treatment of the skin manifestations of cutaneous T-cell lymphoma (CTCL) that is unresponsive to other forms of treatment.
Methoxsalen should be used only by physicians who have special training in the THERAKOS™ UVAR XTS® or THERAKOS™ CELLEX® Photopheresis Systems. Methoxsalen is contraindicated in patients exhibiting idiosyncratic reactions to psoralen compounds, patients with a specific history of a light sensitive disease, or patients with aphakia.
THERAKOS™ Photopheresis is not appropriate for patients who cannot tolerate extracorporeal volume loss or shifts, or patients with coagulation disorders. See Important Safety Information for additional details.
