Diagnóstico y tratamiento de los linfomas cutáneos de células T primarios

doi:10.1016/S0001-7310(04)76864-7

Actas Dermo-Sifiliográficas

Volume 95, Issue 8, 2004, Pages 473-490

https://doi.org/10.1016/S0001-7310(04)76864-7 Get rights and content

Resumen

—Los linfomas cutáneos primarios de células T constituyen un grupo heterogéneo de procesos caracterizados por la infiltración y proliferación de una población linfoide maligna de células T en la piel, sin evidencia de afectación extracutánea. Constituyen entidades diferenciadas tanto desde el punto de vista biológico como evolutivo de sus contrapartidas extracutáneas (nodales). Para un buen manejo de los distintos tipos de linfomas cutáneos primarios de células T resulta necesario un conocimiento preciso de cada uno de los distintos subtipos. Se dispone de una gran variedad de posibilidades terapéuticas en los linfomas cutáneos de células T, algunas de ellas con un efecto cutáneo directo, y otras con actividad sistémica.

Se revisan las características nosológicas, clinicopatológicas, diagnósticas, evolutivas y terapéuticas de las distintas entidades incluidas dentro del grupo de los linfomas cutáneos primarios de células T.

Abstract

—Primary cutaneous T-cell lymphomas make up a heterogeneous group of processes characterized by infiltration and proliferation of a malignant lymphoid population of T-cells in the skin, with no evidence of extracutaneous involvement. They are entities that are both biologically and evolutionally different from their extracutaneous counterparts (nodal lymphomas). To properly manage the different types of primary cutaneous T-cell lymphomas, accurate knowledge of each of the different subtypes is necessary. We have a great variety of therapeutic possibilities in cutaneous T-cell lymphomas, some of which have a direct cutaneous effect, while others have systemic activity.

We review the nosological, clinical-pathological, diagnostic, evolutional and therapeutic characteristics of the different entities included in the group of primary cutaneous T-cell lymphomas.

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Treatment of cutaneous lymphomas: An Update
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Primary cutaneous lymphomas (PCLs) are a heterogeneous group of lymphoid tumors that originate primarily in the skin. Most PCLs (75%) are T-cell lymphomas and only 20% to 25% involve B cells. It is important to differentiate between cutaneous lymphomas and lymph node tumors given the differences in their molecular biology and clinical, histopathologic, and immunophenotypic features. Moreover, PCLs generally follow a more indolent course and require different treatments.
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Los linfomas cutáneos primarios (LCP) constituyen un grupo heterogéneo de neoplasias linfoides que se originan primariamente en la piel. La mayoría (75%) son linfomas de células T y solo un 20-25% se originan a partir de los linfocitos B. Es importante diferenciar los LCP de sus equivalentes ganglionares, dado que presentan características clínicas, histopatológicas, inmunofenotípicas y de biología molecular diferentes con un pronóstico en la mayoría de los casos más indolente y tratamientos diferentes.
Existen múltiples opciones terapéuticas en el manejo de los LCP. La elección del tratamiento debe basarse principalmente en el estadio clínico del paciente, pero deben considerarse también otros factores, como la accesibilidad a los tratamientos, la edad y el estado general del paciente o el coste-beneficio. Además es importante el abordaje multidisciplinar de estos pacientes, formando un equipo experto entre dermatólogos, hematooncólogos y radioterapeutas que conozcan bien esta infrecuente patología. En los últimos años asistimos a la aparición de múltiples terapias nuevas, especialmente para el tratamiento de los estadios avanzados o de pacientes refractarios a tratamientos previos. El motivo de este artículo es revisar todas las alternativas terapéuticas a nuestro alcance.
Folliculotropic mycosis fungoides: A case-based review
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La micosis fungoide folicular es una variante poco frecuente de micosis fungoide, caracterizada por infiltrados de linfocitos atípicos alrededor y en el interior del epitelio folicular. La forma de presentación es muy diversa e incluye máculas, placas o nódulos, como los de la micosis fungoide clásica, pero en ocasiones también o únicamente lesiones tipo acné, como comedones, pápulas inflamatorias y quistes o pápulas foliculares e hiperqueratósicas, y placas de alopecia. Esta variedad clínica hace que pueda resultar muy difícil el diagnóstico precoz. Cursa con un prurito intenso acompañante. La cabeza, las áreas del cuello y de la región superior del tronco son las localizaciones de preferencia. Desde el punto de vista histológico, el hallazgo más característico es la localización profunda, folicular y perifolicular de los infiltrados neoplásicos, con o sin mucinosis folicular asociada, que hace que esta entidad sea poco sensible a las terapias dirigidas a la piel. Revisamos las características clínicas e histológicas, así como el diagnóstico diferencial, el pronóstico y el tratamiento más idóneo para esta entidad, basándonos en la descripción de cuatro de nuestros pacientes.
Folliculotropic mycosis fungoides is a relatively uncommon variant of mycosis fungoides characterized by atypical lymphocytes around and within the epithelium of the hair follicles. This entity can present with a wide variety of clinical pictures including acneiform lesions, comedones, cysts, alopecia with or without scarring, and nodular prurigo-like lesions. The head, neck region and upper trunk, are most commonly involved. Pruritus is especially common. From a biologic point of view, the most relevant feature in cases with and without associated follicular mucinosis is the deep, follicular, and perifollicular localization of the neoplastic infiltrates, which makes them less accessible to skin-targeted therapies. This case-based clinicopathologic review provides clinical-pathological, differential diagnostic, prognosis and therapeutic characteristics of this subtype of micosis fungoides.
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Repasamos la historia de los linfomas cutáneos a través de los artículos referidos al tema en la revista Actas Dermo-Sifiliográficas. Aproximadamente 100 años después de que Alibert describiera el primer paciente con micosis fungoide en 1806, comienzan a aparecer casos sobre el tema en Actas. Veremos cómo a lo largo del siglo que lleva publicándose la Revista ha ido cambiando el concepto, la clasificación y el tratamiento de esta enfermedad, que aún hoy en día sigue siendo un reto para el dermatólogo.
This article reviews the history of cutaneous lymphomas through the articles published on this subject in the journal Actas Dermo-Sifiliográficas. Approximately 100 years after Alibert published the first description of a patient with mycosis fungoides in 1806, reports of cutaneous lymphoma cases began to appear in Actas. These articles reflect how the definition, classification, and treatment of this disease, which continues to pose a challenge for dermatology, have evolved over the 100-year lifespan of the journal.
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CD30⁺ lymphoproliferative disorders are the most common group of cutaneous T-cell lymphomas after mycosis fungoides and its subtypes. This group includes lymphomatoid papulosis and CD30⁺ anaplastic large-cell lymphoma; these 2 entities are the extremes of a spectrum with numerous intermediate varieties in which it is not possible to establish a clear diagnosis based on clinical and histopathologic criteria. CD30⁺ lymphoproliferative disorders must be differentiated from other lymphoproliferative diseases with CD30⁺ cells in the tumor infiltrates, such as mycosis fungoides or Hodgkin disease, and also from other inflammatory conditions or nonhematological neoplasms that can include this cell type, such as pityriasis lichenoides et varioliformis acuta or certain mesenchymal tumors (CD30⁺ pseudolymphomas). In contrast to their systemic homologues, which arise in the lymph nodes, CD30⁺ lymphoproliferative disorders generally have a good prognosis. It is very important to exclude the presence of a lymphoma of systemic origin with extralymphatic spread, as the prognosis and treatment are different.
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Diagnóstico y tratamiento de los linfomas cutáneos de células T primariosDiagnosis and treatment of primary cutaneous t-cell lymphomas

Resumen

Abstract

Blood

J Am Acad Dermatol

Lancet

J Am Acad Dermatol

J Am Acad Dermatol

Am J Pathol

J Am Acad Dermatol

J Invest Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

Blood

J Am Acad Dermatol

Blood

Blood

J Am Acad Dermatol

Blood

J Am Acad Dermatol

Blood

Blood

J Am Acad Dermatol

J Invest Dermatol

Blood

Blood

Am J Pathol

The Whorld Health Organization classification of tumours. Pathology & genetics. Tumours of haematopoietic and lymphoid tissues

The new World Health Organizaton classification of haematopoietic and lymphoid tumours: a dermatopathological perspective

Br J Dermatol

EORTC Classification for Primary Cutaneous Lymphoma. The best guide to good clinical management

Am J Dermatopathol

Micosis fungoide y síndrome de Sézary

Actas Dermosifiliogr

Practical evaluation and management of cutaneous lymphoma

J Am Acad Dermatol

A case of pagetoid reticulosis bearing the T cytotoxic suppressor surface marker on the lymphoid infiltrate: further evidence that pagetoid reticulosis is not a variant of mycosis fungoides

Br J Dermatol

Pityriasis lichenoideslike mycosis fungoides in children

Br J Dermatol

Variants of mycosis fungoides and related cutaneous T-cell lymphomas

Semin Diagn Pathol

Accuracy, concordance, and reproducibility of histologic diagnosis in cutaneous T-cell lymphoma: an EORTC cutaneous lymphoma project group study

Arch Dermatol

Transformación de la micosis fungoide/síndrome de Sézary a linfoma de células grandes

Actas Dermosifiliogr

CD7 paraffin reactive antibody: loss of expression in benign and malignant cutaneous lymphocytic infiltrates [abstract]

J Cutan Pathol

Detection of TCR-gene rearrangements in early mycosis fungoides by non-radioactive PCR-SSCP

J Cutan Pathol

Joint British Association of Dermatologists and UK Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas

Br J Dermatol