Cutaneous T Cell Lymphoma

Characterization of primary cutaneous T‐cell lymphoma following solid organ transplantation

“…16 Worse prognosis is associated with age, more advanced stages of MF, folliculotropism, low white blood cell count, and malignant infiltration into lymph nodes, blood, and viscera. 14 Unfortunately, PT-CTCL may be more aggressive and refractory to standard intervention than lymphoproliferative disease in the general population. 7,17 Additionally, there is evidence that the incidence of PTLD, and CTCL specifically, is increasing.…”

“…In the general population, the annual incidence of CTCL is about 0.5 in 100,000, with greater frequency observed among blacks, adults aged 55–60 years old, and males 12,13 . Definitive evidence regarding its pathogenesis has yet to be described, although numerous hypotheses such as chronic antigen stimulation, infectious agents or environmental exposures, and ultraviolet (UV) irradiation have been proposed 14–16 . There are many subtypes of CTCL with the most common being mycosis fungoides (MF), which accounts for 44% of primary CTCL cases 17,18 .…”

“…For most cases of PTLD, reduction in immunosuppression is the first‐line treatment 20 with adjunctive measures including skin‐targeted photochemotherapy, topical nitrogen mustard, or radiotherapy as options for skin‐localized CTCL 21 . In the general population, the mortality rate of CTCL ranges from 10% to 15% up to 43%, 14 with an estimated 5‐year survival of 87% for MF reported in one study of 278 patients 16 . Worse prognosis is associated with age, more advanced stages of MF, folliculotropism, low white blood cell count, and malignant infiltration into lymph nodes, blood, and viscera 14 .…”

“…In the general population, the mortality rate of CTCL ranges from 10% to 15% up to 43%, 14 with an estimated 5‐year survival of 87% for MF reported in one study of 278 patients 16 . Worse prognosis is associated with age, more advanced stages of MF, folliculotropism, low white blood cell count, and malignant infiltration into lymph nodes, blood, and viscera 14 . Unfortunately, PT‐CTCL may be more aggressive and refractory to standard intervention than lymphoproliferative disease in the general population 7,17 .…”

“…12,13 Definitive evidence regarding its pathogenesis has yet to be described, although numerous hypotheses such as chronic antigen stimulation, infectious agents or environmental exposures, and ultraviolet (UV) irradiation have been proposed. [14][15][16] There are many subtypes of CTCL with the most common being mycosis fungoides (MF), which accounts for 44% of primary CTCL cases. 17,18 A rarer subtype of CTCL known as folliculotropic mycosis fungoides (FMF) accounts for only 5-10% of CTCL, 19 however, it may be more common in posttransplant patients compared with the general population.…”