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2018 ; 35
(1
): 35-41
Nephropedia Template TP
gab.com Text
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English Wikipedia
Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve
Prognoz
#MMPMID28533196
Vural S
; Akay BN
; Botsal? A
; Atilla E
; Parlak N
; Okçu Heper A
; ?anl? H
Turk J Haematol
2018[Mar]; 35
(1
): 35-41
PMID28533196
show ga
OBJECTIVE: Transformed mycosis fungoides (T-MF) is a rare variant of MF with an
aggressive course. In this study, we aimed to describe characteristics of
MF/Sezary syndrome (SS) patients with transformation. MATERIALS AND METHODS:
Patients diagnosed with T-MF among MF/SS patients between 2000 and 2014 in a
tertiary single center were evaluated retrospectively. Demographic data, clinical
data, laboratory data, immunophenotype features, response to treatment, survival,
and histopathologic features were analyzed. RESULTS: Among 254 MF patients, 25
patients with T-MF were identified (10.2%) and included in the study. The
male-to-female ratio was 2.6/1. The median time between MF diagnosis and
transformation was 32 months (range: 0-192). Nine (36%) patients were diagnosed
initially with T-MF. Advanced disease stage and high serum lactate dehydrogenase
(LDH) levels were indicators of poor prognosis and treatment response. Five of
the 18 patients with progressive disease had undergone allogeneic hematopoietic
stem cell transplantation (allo-HSCT). Allo-HSCT resulted in complete remission
in three (60%) patients. Ten (40%) patients died as a result of disease
progression. Mean survival time was 25.2±14.9 (2-56) months after transformation.
CONCLUSION: Advanced stage, high serum LDH levels, and loss of CD26 and CD7
expression in the peripheral blood are poor rognostic factors in T-MF.
Treatment-resistant tumors and nodules should be cautionary for T-MF. Patients
with T-MF have a shortened survival. Some patients may respond to first-line
treatments. However, the majority of patients who do not respond to first-line
therapies also are unresponsive to second or third-line therapies. Allo-HSCT may
be an alternative option in patients with T-MF.
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