Atypical mycosis fungoides with cerebral involvement

Abstract

This report concerns a 17-year-old male patient with atypical mycosis fungoides (m.f.). Initial examination revealed generalized lymphoma and uncharacteristic livid skin efflorescence. The patient developed bone marrow involvement and meningeal leukaemia 6 months later. Diagnosis was confirmed by immunohistochemistry and electron microscopy. Aggressive chemotherapy yielded no response.

Mycosis fungoides in Patients under 20 Years of Age: Report of 7 Cases, Review of the Literature and Study of the Clinical Course

“…5 and one reported by Zackheim et al [20]). The immunophenotype of the lymphoid infiltrate [6, 7, 8, 9, 11, 12, 13, 14, 15, 16, 17, 18, 21]was CD2+CD3+CD4+CD5+CD7–CD8– in the majority of cases. A loss of T cell lineage markers was found in 4 cases [7, 11, 15], whereas in 3 patients half or more of the skin-infiltrating lymphocytes lacked CD4 expression [8, 11, 12].…”

“…MF onset in patients younger than 20 years of age is rare [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21]with a percentage varying from 0.5% [12]to 2.3% [20]of the total MF cases (2.7% in our series). However, the true incidence of early-onset MF may be higher than that reported, in as much as the histological diagnosis of MF is frequently delayed in this age group.…”

“…Forty-nine patients (30 males and 19 females; ratio: 1.58) were included in the early-onset group, of whom 42 were identified by a literature review [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21]. The median age of histological diagnosis was 14 years (range: 2–19).…”

“…Two groups of patients were compared: (1) MF patients diagnosed in our Clinic from January 1975 to August 1997, in whom the first histological diagnosis was performed after 20 years of age (252 cases; median age at diagnosis: 61 years, range: 24–91) and (2) the cases of early-onset MF (first histological diagnosis before 20 years of age) reported in the literature from January 1975 to August 1997 [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21]plus the 7 herein reported. SS cases reported in the literature [26, 27]with histological diagnosis before 20 years of age were not included in this study.…”

“…Clinical and pathological features of these cases have not yet been well defined. In fact, some authors suggest that early-onset MF/SS is more likely to be associated with extracutaneous involvement [7, 12, 21]as well as with the development of high-grade T cell lymphomas [12]or other non-T-cell lymphomas, such as Hodgkin’s disease [12]. On the other hand, other studies [15, 20]suggest that early-onset MF/SS is not more aggressive than those cases with a later onset.…”