RESUMEN
Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are the 2 main subtypes of pityriasis lichenoides. They represent the acute and chronic forms of the disease; both may have clonal T cells. Several treatment modalities are used, but it has been difficult to determine efficacy because of the possibility of spontaneous remission. Cutaneous CD30+ lymphoproliferative disorders constitute many cutaneous T-cell lymphomas and comprise lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma (ALCL). Both have an excellent prognosis. Lymphomatoid papulosis often only requires observation or treatment of symptoms. First-line therapies for primary cutaneous ALCL are surgical excision or radiotherapy.
Asunto(s)
Linfoma Anaplásico Cutáneo Primario de Células Grandes/terapia , Papulosis Linfomatoide/terapia , Pitiriasis Liquenoide/terapia , Neoplasias Cutáneas/terapia , Corticoesteroides/uso terapéutico , Antibacterianos/uso terapéutico , Anticuerpos Monoclonales/uso terapéutico , Antineoplásicos/uso terapéutico , Procedimientos Quirúrgicos Dermatologicos , Humanos , Inmunosupresores/uso terapéutico , Queratolíticos/uso terapéutico , Antígeno Ki-1 , Linfoma Anaplásico Cutáneo Primario de Células Grandes/diagnóstico , Linfoma Anaplásico Cutáneo Primario de Células Grandes/patología , Papulosis Linfomatoide/diagnóstico , Papulosis Linfomatoide/patología , Fototerapia , Pitiriasis Liquenoide/diagnóstico , Pitiriasis Liquenoide/patología , Radioterapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patologíaRESUMEN
: Acquired expression of CD30 is frequently noted in histological transformation of mycosis fungoides (MF), but simultaneous gain of CD15 accompanied with loss of pan-T-cell antigens are extremely rare. We report an unusual case of transformed MF with such an immunophenotypic alteration resembling classical Hodgkin lymphoma. The patient was an 81-year-old male with MF, who was initially treated with topical steroids and phototherapy. Despite the initial response, the patient developed a tumor-like skin lesion that was confirmed to be CD30-positive large T-cell lymphoma and was subsequently found to have a regional lymph node involvement by pleomorphic large cell lymphoma. Besides CD30, pleomorphic large cells were positive for CD15 but negative for all B cell- and T cell-specific antigens. Epstein-Barr virus was negative. Polymerase chain reaction-based assays demonstrated a clonal rearrangement of T-cell receptor gamma gene but detected no B-cell clone. The mechanism and clinical significance of this phenotypic conversion remains to be elucidated.
Asunto(s)
Transformación Celular Neoplásica/patología , Enfermedad de Hodgkin/patología , Micosis Fungoide/patología , Enfermedades de la Piel/patología , Anciano de 80 o más Años , Biomarcadores de Tumor/análisis , Fucosiltransferasas/biosíntesis , Humanos , Inmunofenotipificación , Antígeno Ki-1/biosíntesis , Antígeno Lewis X/biosíntesis , Linfoma Anaplásico Cutáneo Primario de Células Grandes/patología , MasculinoRESUMEN
The 2005 EORTC/WHO classification includes three CD30+ lymphoproliferative disorders: 1) primary cutaneous anaplastic large cell lymphoma, 2) lymphomatoid papulosis and 3) borderline cases. These entities may present with many different clinical appearances. Therefore, a precise differentiation among them often is impossible. We present a 40-year-old female who initially presented with a neutrophil-rich, anaplastic CD30+ T cell lymphoma followed by lymphomatoid papulosis.
Asunto(s)
Neoplasias Faciales/diagnóstico , Linfoma Anaplásico Cutáneo Primario de Células Grandes/diagnóstico , Papulosis Linfomatoide/diagnóstico , Neoplasias Primarias Múltiples/diagnóstico , Neutrófilos/patología , Neoplasias Cutáneas/diagnóstico , Adulto , Antimetabolitos Antineoplásicos/uso terapéutico , Diagnóstico Diferencial , Eosinófilos/patología , Neoplasias Faciales/patología , Femenino , Humanos , Linfocitos/patología , Linfoma Anaplásico Cutáneo Primario de Células Grandes/tratamiento farmacológico , Linfoma Anaplásico Cutáneo Primario de Células Grandes/patología , Papulosis Linfomatoide/tratamiento farmacológico , Papulosis Linfomatoide/patología , Metotrexato/uso terapéutico , Neoplasias Primarias Múltiples/tratamiento farmacológico , Neoplasias Primarias Múltiples/patología , Terapia PUVA , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patologíaRESUMEN
OBJECTIVES: To identify prognostic factors in primary cutaneous anaplastic large cell lymphoma (pcALCL), focusing on extensive limb disease (ELD), defined as initial presentation or progression to multiple skin tumors in 1 limb or contiguous body regions, and to study gene expression profiles of patients with pcALCL. DESIGN: Retrospective cohort study. SETTING: The Stanford Comprehensive Cancer Center and dermatology ambulatory clinics. PATIENTS: A total of 48 patients with pcALCL evaluated from 1990 through 2005. MAIN OUTCOME MEASURES: Hazard ratios (HRs) for prognostic factors for overall survival (OS) and disease-specific survival (DSS) and risk factors for progression to extracutaneous disease were identified using Cox regression. Gene expression profiles of 9 typical pcALCL and 3 ELD samples were investigated using complementary DNA microarrays. RESULTS: Univariate analysis demonstrated age, ELD, and progression to extracutaneous disease as significant prognostic factors for OS, whereas ELD and progression to extracutaneous disease were significant for DSS. In multivariate analysis, age (HR, 1.83; 95% confidence interval [CI], 1.02-3.26) and progression to extracutaneous disease (HR, 6.42; 95% CI, 1.39-29.68) remained significant for OS, whereas ELD (HR, 29.31; 95% CI, 1.72-500.82) and progression to extracutaneous disease (HR, 13.12; 95% CI, 1.03-167.96) remained independent prognostic factors for DSS. Presentation with T3 disease was a risk factor for progression to extracutaneous disease (HR, 10.20; 95% CI, 1.84-56.72). Microarray data revealed that patients with ELD and typical pcALCL formed distinct clusters. CONCLUSIONS: Patients with ELD have a more aggressive course associated with a differential gene expression profile. More aggressive treatments may be indicated for patients with ELD and those whose disease progresses to extracutaneous disease because they have poorer outcomes.