The median age at CTCL diagnosis was higher than previously reported in the UK (67 vs. In the current study, the male-to-female ratio in MF and SS patients (1.6:1) was similar to previous Swedish and international data. These findings have important implications for future CTCL research based on registry data. Patients with advanced-stage disease and poor OS were registered more often. As for the SLR validation, we show low CTCL patient completeness relative to medical records and long median time between diagnosis and registration. The median OS for MF and SS patients was relatively short, and the median age was high compared to international data.
CUTANEOUS TCEL LYMPHOMA REGISTRATION
In this study, we performed a comprehensive multi-center cohort analysis of OS among CTCL patients as well as the first investigation of completeness and timeliness of CTCL registration in Sweden. Two patients were treated at both hospitals but were only counted once. Patient data from the medical records were linked to the SLR by the unique Swedish personal identity number, and we noted if the patients were registered in the SLR or not. The diagnosis was verified in the chart, non-CTCL patients were excluded and relevant clinical information was retrieved following written informed consent from live patients ( Supplementary Figure 1). Since CTCL is a heterogeneous group of rare diseases where many subtypes lack specific ICD-10 codes or are frequently misdiagnosed, the additional codes C84.4 (Peripheral T-cell lymphoma, not classified), C84.5 (other mature NK-T cell lymphoma) and C84.9 (mature NK-T cell lymphoma, unspecified) were also included in the initial search. Index patients were identified through the medical record systems at the dermatology, hematology, and oncology clinics using the International Classification of Diseases 10th revision (ICD-10) codes C84.0 (MF), C84.1 (SS) and C84.8 (CTCL, unspecified). The study population consisted of patients diagnosed with CTCL between the years 20 at KUH and between the years 20 at UUH. In addition, we have performed a comprehensive evaluation of patient characteristics and OS in a large CTCL cohort. To investigate these questions, we have performed the first validation of the data quality of national registration of CTCLs in Sweden using medical records as the gold standard. We also hypothesized that patients with early-stage disease and hence longer survival time may have been registered to a lower extent. We hypothesized that CTCL patients may be underreported into the SLR, since they are often managed at dermatology clinics and not at oncology or hematology clinics where most other lymphoma patients are treated. However, the level of completeness for specific lymphoma subtypes, including CTCLs, has so far not been described. According to the yearly report of the SLR, the completeness of the registry regarding all lymphoma patients is >95%, when compared with cases registered in the SCR. To avoid selection bias in register studies, it is important to ensure that the levels of coverage and completeness of a register are sufficient. SLR thus records data on adult (>18 years) lymphoma patients including lymphoma subtype, stage, primary treatment, treatment response, and survival. The Swedish Lymphoma Register (SLR) was initiated in the year 2000 to complement SCR with more detailed lymphoma-specific data for follow-up of quality of care. The Swedish Cancer Register (SCR) is a nation-wide register with mandatory recording of all newly diagnosed cancer patients. Population-based registers are important and relatively non-biased data sources for both clinical and research purposes. Population-based data on the overall survival (OS) among CTCL patients are mostly limited to studies that are small, old, or with short follow-up considering the often indolent course of CTCL. Early-stage disease (stage IA–IIA) generally has a favorable prognosis with a 5-year disease-specific survival of 89%–98%, while advanced-stage MF (IIB-IV) has a worse prognosis with a 5-year disease-specific survival reported to range between 18 and 67%. Consequently, the prognosis of MF is often favorable, but may still vary considerably among patients and is dependent on the stage of the disease.
CUTANEOUS TCEL LYMPHOMA SKIN
However, for unknown reasons some patients develop a more aggressive form of the disease with skin tumors, erythroderma, and/or extracutaneous involvement.
MF often has an indolent clinical course characterized by superficial skin patches and/or elevated plaques. Mycosis fungoides (MFs) represent the most common primary CTCL accounting for nearly 60% of all cases with an incidence of 0.2–0.6 cases/100,000 person years in Europe and the USA. Primary cutaneous T-cell Lymphoma (CTCL) is a group of rare non-Hodgkin lymphomas characterized by malignant T cells that are initially only present in the skin.
0 kommentar(er)
