Buenas noches Doctor Juan Macaluso.
Le escribe la Dra Yuranis Tirado residente de
Oncología Pediátrica, envío imágenes de paciente de 15 años con cuadro clínico
de 2 años de evolución manejada con múltiples medidas convencionales por
servicio de dermatología e infectología; posterior a un tiempo se envía muestra
a patología quienes reportan diagnóstico de Micosis Fungoide, que aunque la literatura expresa
ser unos de los tipos de linfomas cutaneos de células T más frecuentes, en 2
años ha sido el primero que observo y quise compartirles.
Actualmente paciente en espera de estudio
inmunohistoquímico.
Dra. Yuranis Tirado.
Maracaibo. Venezuela.
Opinión: Muy interesante el caso Yuranis por varios motivos. Siempre tuve el concepto de que micosis fungoide (MF), era una enfermedad poco frecuente y de adultos. Cuando leí tu caso me fui a actualizar información en UpToDate y encontré que la incidencia de MF en Europa y los Estados Unidos es de aproximadamente seis casos por millón por año, lo que representa alrededor del 4 por ciento de todos los casos de linfoma no Hodgkin. Por otro lado, la edad pico de presentación es de 55 a 60 años, con una proporción hombre:mujer de 2:1. La enfermedad es más común en poblaciones negras. Aunque la MF es una enfermedad principalmente de pacientes mayores, se puede observar en pacientes menores de 35 años con hallazgos clínicos y evolución similares (39-41).
Referencias
39) Crowley JJ, Nikko A, Varghese A, et al. Mycosis
fungoides in young patients: clinical characteristics and outcome. J Am Acad
Dermatol 1998; 38:696.
40) Quaglino P, Zaccagna A, Verrone A, et al. Mycosis
fungoides in patients under 20 years of age: report of 7 cases, review of the
literature and study of the clinical course. Dermatology 1999; 199:8.
41) Pope E, Weitzman S, Ngan B, et al. Mycosis
fungoides in the pediatric population: report from an international Childhood
Registry of Cutaneous Lymphoma. J Cutan Med Surg 2010; 14:1.
ABSTRACTS
Abstracts for References 39-41 of 'Clinical
manifestations, pathologic features, and diagnosis of mycosis fungoides'
39
PubMed
TI
Mycosis fungoides in young patients: clinical
characteristics and outcome.
AU
Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH
SO
J Am Acad Dermatol. 1998;38(5 Pt 1):696.
BACKGROUNDMycosis fungoides (MF) can begin as early as
the first decade of life. Few studies have reviewed MF in younger patients and
none has been large enough to assess prognosis and outcome.
OBJECTIVEWe reviewed the clinical characteristics,
prognosis, factors related to disease progression, and therapy in patients with
MF younger than 35 years of age.
METHODSFifty-eight patients were entered into this
retrospective cohort analysis.
RESULTS Significantly fewer patients with MF who are
younger than 35 years presented with erythroderma (T4) and more with limited
patch/plaque (T1) disease than older patients. Duration of skin disease before
diagnosis of MF did not vary between the two groups. The long-term survival of
younger patients with MF is significantly decreased when compared with a race-,
age-, and sex-matched control population (p<0.001). Disease-specific
survivals (DSS) of younger and older patients are similar, but young patients
show a slight but significantly better overall DSS (p<0.02). However, DSS
comparison of generalized patch/plaque (T2) and tumor stage (T3) patients with
MF showed no significant difference between young and old patients (p=0.47, p=0.59).
Patient age was not a significant predictor of survival when controlled for
T-stage. Sixteen of 58 young patients with MF have died, 13 because of MF
(22%), compared with 138 of 500 older patients (28%) who died as a result of
MF. All younger patients with MF who progressed had at least T2 disease at
presentation. Fifty of 56 young patients with MF and T1-T3 disease were treated
initially with total skin electron beam or topical nitrogen mustard. The
response to therapy was similar in younger and older patients with MF.
CONCLUSIONT1 disease is more common and T4 disease is
unusual in young patients with MF compared with an older population of patients
with MF. Young patients with T1 disease, all of whom were treated with either
topical nitrogen mustard or total skin electron beam therapy, or both
therapies, showed no disease progression. Overall, young patients with MF
showed slightly better DSS, but this was because of differences in stage
distribution.
AD
Department of Dermatology, Stanford University School
of Medicine, California, USA.
PMID
9591813
40
PubMed
TI
Mycosis fungoides in patients under 20 years of age:
report of 7 cases, review of the literature and study of the clinical course.
AU
Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo
MG
SO
Dermatology. 1999;199(1):8.
BACKGROUNDMycosis fungoides (MF) is rare in young
patients. Its clinical behavior is still uncertain, as some reports have
suggested that it has a more aggressive course than does the adult-onset type.
AIMTo ascertain if early-onset MF represents a
heterogeneous group of cutaneous T cell lymphomas.
MATERIALS AND METHODSClinical, immunohistopathological
and follow-up data of early-onset (<20 years of age) MF cases reported in
the literature (n = 42) plus 7 described herein were compared with those of a
cohort of adult-onset MF patients (n = 252) diagnosed at our institution since
1975.
RESULTS The majority of the 49 early-onset MF patients
had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The
predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage
progression, 6 with extracutaneous involvement. Five- and 10-year survival
rates were 93 and 74%, respectively.
CONCLUSIONSNo statistically significant differences
were found in the disease course between early- and adult-onset MF.
AD
Section of Dermatology, Department of Medical and
Surgical Specialties, University of Turin, Italy.
PMID
10449950
41
PubMed
TI
Mycosis fungoides in the pediatric population: report
from an international Childhood Registry of Cutaneous Lymphoma.
AU
Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams
J, Stein S, Garzon M, Knobler E, Lieber C, Turchan K, Wargon O, Tsuchiya A
SO
J Cutan Med Surg. 2010 Jan;14(1):1-6.
BACKGROUND/OBJECTIVESThere are limited data on the
clinical presentation and progression of pediatric cutaneous lymphoma. This
study focuses on the clinical characteristics of pediatric patients with
mycosis fungoides (MF).
MATERIALS AND METHODS This descriptive study presents
clinical characteristics of 22 pediatric patients with MF, enrolled in the
international Childhood Registry for Cutaneous Lymphomas (CRCL).
RESULTS The mean ages at onset and at diagnosis were
7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common
MF presentation was patch stage (68%), followed by hypopigmentation (59%) and
plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the
most common pathologic features, found in 89% and 85%, respectively.
Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in
16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and
clonality was detected in 21% (3 of 14) of patients. All patients presented
with early-stage disease and received skin-directed therapy (topical steroids,
73%; light therapy, 54%; or combination therapy, 35%).
CONCLUSIONS Pediatric patients with MF present in the
first decade of life, with early-stage disease and unusual forms such as
hypopigmented variant. Further patient enrollment will provide information
regarding natural history, treatment response, and overall prognosis of
pediatric cutaneous T-cell lymphoma (CTCL).
AD
Section of Dermatology, The Hospital for Sick
Children, Toronto, ON, Canada. Elena.pope@sickkids.ca
PMID
20128983
