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1.
Dermatol. argent ; 27(1): [3-11], ene.-mar. 2021. il, tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1359875

ABSTRACT

El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8 +, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.


The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.


Subject(s)
Humans , Skin Neoplasms , Lymphoma, Primary Cutaneous Anaplastic Large Cell/diagnosis , Carcinoma, Squamous Cell , Drug Therapy, Combination , Lymphoma, Primary Cutaneous Anaplastic Large Cell/therapy
2.
Korean Journal of Head and Neck Oncology ; (2): 77-80, 2019.
Article in Korean | WPRIM | ID: wpr-787525

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is rare among skin malignancies. C-ALCL usually manifests as reddish or violet nodules. Surgical excision or radiation therapy is generally considered as first-line therapy, but a clinically aggressive disease may require multiagent chemotherapy. Establishing a proper diagnosis of C-ALCL is challenging but should be made to avoid inappropriate treatment and its consequences. The authors report a case of medically resolved C-ALCL in an 81-year-old man presented with well-defined nodular lesions on the forehead.


Subject(s)
Aged, 80 and over , Humans , Diagnosis , Drug Therapy , Forehead , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell , Skin , Viola
3.
Archives of Craniofacial Surgery ; : 207-211, 2019.
Article in English | WPRIM | ID: wpr-762761

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.


Subject(s)
Aged , Humans , Ki-1 Antigen , Diagnosis , Follow-Up Studies , Lip , Lymph Nodes , Lymphoma , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Lymphomatoid Papulosis , Lymphoproliferative Disorders , Methotrexate , Prognosis , Recurrence , Skin , Ulcer
4.
In. Hajjar, Ludhmila Abrahão; Kalil Filho, Roberto; Hoff, Paulo Marcelo Gehm. Manual de condutas em cardio-oncologia / Manual of conducts in cardiology and oncology. Rio de janeiro, Atheneu, 1ª; 2018. p.95-110.
Monography in Portuguese | LILACS | ID: biblio-875221
5.
Medicina (Ribeiräo Preto) ; 50(4): 261-264, jul.-ago 2017.
Article in English | LILACS | ID: biblio-877424

ABSTRACT

Lymphomatoid papulosis (LyP) is defined as a chronic, recurrent, self-healing papulonecrotic or papulonodular skin disease with histologic features suggestive of a (CD30-positive) malignant lymphoma. In up to 20% of patients, LyP are preceded by, associated with, or followed by another type of cutaneous or systemic lymphoma, generally mycosis fungoides (MF), primary cutaneous anaplastic large cell lymphoma (C-ALCL). In this case, we describe a case of MF that preceded and continued to coexist with LyP type C.(AU)


A papulose linfomatóide (LyP) é definida como uma doença cutânea papulonecrótica ou papulonodular crônica, recorrente, com características histológicas sugestivas de linfoma maligno (CD30-positivo). Em até 20% dos pacientes, o LyP é precedido por, associado ou seguido por outro tipo de linfoma cutâneo ou sistêmico, geralmente micose fungóide (MF), linfoma cutâneo primário de células grandes anaplásicas (C-ALCL). Neste caso, descrevemos um caso de MF que precedeu e continuou a coexistir com LyP tipo C. (AU)


Subject(s)
Humans , Female , Adult , Lymphoma , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphomatoid Papulosis , Mycosis Fungoides , T-Lymphocytes
6.
An. bras. dermatol ; 92(1): 86-91, Jan.-Feb. 2017. graf
Article in English | LILACS | ID: biblio-838014

ABSTRACT

Abstract: The classification of cutaneous lymphomas is multidisciplinary and requires the correlation between clinical, histopathological, immunohistochemical, and molecular diagnostic elements. In this article, we present four different cases of CD30-positive T-cell lymphoma with cutaneous manifestations. We compare cases with definitive diagnosis of papulosis lymphomatoid type C, primary cutaneous anaplastic large T-cell lymphoma, systemic anaplastic large T-cell lymphoma with secondary skin involvement, and mycosis fungoides with large cell transformation, highlighting the importance of clinicopathological correlation to classify these cases.


Subject(s)
Humans , Male , Female , Adolescent , Middle Aged , Aged, 80 and over , Skin Neoplasms/pathology , Lymphoma, Primary Cutaneous Anaplastic Large Cell/pathology , Immunohistochemistry
7.
Korean Journal of Dermatology ; : 610-614, 2017.
Article in Korean | WPRIM | ID: wpr-112169

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare primary cutaneous lymphoma that is predominantly composed of large lymphoid cells that express the CD30 antigen. The skin lesion of PCALCL is usually single, ulcerative, and located on the trunk or extremities and rarely the palm. A 25-year-old woman presented with a plaque on the left palm for 20 days. The plaque was walnut-sized and purple to gray colored with erosion in the center. Histopathologic examination showed infiltration of large atypical cells in the dermis. The large tumor cells showed positivity for CD3, CD4, and CD30 and negativity for CD8, CD20, epithelial membrane antigen, and anaplastic lymphoma kinase. PET-CT showed no other hypermetabolic lesion except that on the left palm, and we finally arrived at a diagnosis of PCALCL. The patient was treated with an intralesional injection of methotrexate (25 mg/mL, 0.45 cc). After 3 months of treatment, the walnut-sized plaque had disappeared and a peripheral hyperpigmented patch remained.


Subject(s)
Adult , Female , Humans , Ki-1 Antigen , Dermis , Diagnosis , Extremities , Injections, Intralesional , Lymphocytes , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Methotrexate , Mucin-1 , Phosphotransferases , Skin , Ulcer
8.
Annals of Dermatology ; : 491-494, 2016.
Article in English | WPRIM | ID: wpr-171603

ABSTRACT

CD30+ lymphoproliferative disorders (LPD) represent a spectrum of T-cell lymphoma including lymphomatoid papulosis and anaplastic large cell lymphoma (ALCL). Epidermis overlying cutaneous CD30+ LPD often shows epidermal hyperplasia, hyperkeratosis, crusting, and ulceration and it is difficult to distinguish from carcinoma such as keratoacanthoma (KA) or squamous cell carcinoma (SCC). Several cases of pseudocarcinomatous hyperplasia mimicking KA or SCC in CD30+ LPD have been reported. The relationship between CD30+ LPD and epithelial proliferations has not yet well understood. It was reported that a variety of mediators, including epidermal growth factor (EGF), transforming growth factor-α and EGFR from CD30+ LPD could attribute to epidermal hyperplasia. However, separate and distinct SCC occurring in CD30+ LPD has rarely been reported. Herein, we present a rare case of coexistence of SCC and cutaneous ALCL located on the same region.


Subject(s)
Carcinoma, Squamous Cell , Epidermal Growth Factor , Epidermis , Epithelial Cells , Hyperplasia , Keratoacanthoma , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell , Lymphomatoid Papulosis , Lymphoproliferative Disorders , Ulcer
9.
Rev. méd. Chile ; 143(10): 1351-1355, oct. 2015. ilus, tab
Article in Spanish | LILACS | ID: lil-771720

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is within the spectrum of cutaneous CD30-positive lymphoproliferative disorders. It presents as localized or multifocal tumors or plaques and carries an excellent long-term prognosis even in cases with regional and/or ipsilateral lymph node involvement or in cases of recurrent disease. We report a 34 year-old female with a thigh lesion. Skin biopsy confirmed the diagnosis of PCALCL. The patient was strictly monitored but no treatment was instituted and the tumor regressed spontaneously. After 24 months of follow-up the patient remains free of disease without new lesions.


Subject(s)
Adult , Female , Humans , Lymphoma, Primary Cutaneous Anaplastic Large Cell/pathology , Skin Neoplasms/pathology , Biopsy
10.
Korean Journal of Medicine ; : 353-357, 2015.
Article in Korean | WPRIM | ID: wpr-216641

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare subtype of malignant non-Hodgkin lymphoma, in which 40% of the cases show spontaneous regression without aggressive treatment. Surgery and focal radiation therapy are the primary forms of treatment for this disease; however, if pcALCL is accompanied by multifocal skin lesions, chemotherapy is also common. The prognosis for pcALCL is generally excellent, with a 5-year survival rate of 85-100%. However, pcALCL with extensive limb disease typically has a poor prognosis. Here, we present a case of pcALCL with extensive limb disease that resulted in the patient's death, despite the use of aggressive chemotherapy.


Subject(s)
Drug Therapy , Extremities , Lymphoma , Lymphoma, Non-Hodgkin , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Prognosis , Skin , Survival Rate
11.
Korean Journal of Dermatology ; : 304-309, 2015.
Article in Korean | WPRIM | ID: wpr-135057

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.


Subject(s)
Aged , Female , Humans , Biopsy , Chromogranin A , Cytoplasm , Desmin , Eosinophils , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Magnetic Resonance Imaging , Melanoma , Myoglobin , Neuroectodermal Tumors , Neuroendocrine Tumors , S100 Proteins , Synaptophysin , Ulcer , Vimentin
12.
Korean Journal of Dermatology ; : 304-309, 2015.
Article in Korean | WPRIM | ID: wpr-135056

ABSTRACT

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.


Subject(s)
Aged , Female , Humans , Biopsy , Chromogranin A , Cytoplasm , Desmin , Eosinophils , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Magnetic Resonance Imaging , Melanoma , Myoglobin , Neuroectodermal Tumors , Neuroendocrine Tumors , S100 Proteins , Synaptophysin , Ulcer , Vimentin
14.
Chinese Journal of Pathology ; (12): 508-511, 2014.
Article in Chinese | WPRIM | ID: wpr-304463

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the frequency of different types of mature T- and NK-cell lymphomas diagnosed in a 4-year period at Sun Yat-sen University Cancer Center, and to study baseline CD30 for potential anti-CD30 targeted therapy in mature T- and NK-cell lymphoma.</p><p><b>METHODS</b>All cases of mature T- and NK-cell lymphoma diagnosed at Sun Yat-sen University Cancer Center from September 1, 2009 to August 31, 2013, were reviewed. Paraffin-blocks of available 164 consecutive cases were stained for CD30 immunohistochemistry using EnVision protocol.</p><p><b>RESULTS</b>A total of 625 cases of mature T- and NK-cell lymphomas were diagnosed and the most common type was extranodal NK/T cell lymphoma (ENKTL), nasal type 319 (51.0%) cases, followed by angioimmunoblastic T-cell lymphoma (AITL) (119 cases, 19.0%), peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) (81 cases, 13.0%), and anaplastic large-cell lymphoma (ALCL), including 48 cases (7.7%) of systematic ALCL and 11 cases (1.8%) of primary cutaneous ALCL. Besides ALCL, ENKTL had the highest expression rate of CD30 among the 164 cases, with positivity observed in 41 cases (62.1%, 41/66). Only 1 case of PTCL-NOS was CD30 positive. CD30 was not expressed in all 28 cases of AITL and other rare types of mature T- and NK-cell lymphoma.</p><p><b>CONCLUSIONS</b>The frequency of different types of mature T- and NK-cell lymphoma encountered at Sun Yat-sen University Cancer Center was similar to that seen in other areas of China and other Asia countries. CD30 expression is different among several types of mature T- and NK-cell lymphoma. In addition to ALCL, ENKTL has the highest expression rate of CD30, which may be a candidate disease for anti-CD30 targeted therapy.</p>


Subject(s)
Humans , China , Epidemiology , Immunohistochemistry , Killer Cells, Natural , Lymphoma, Extranodal NK-T-Cell , Epidemiology , Pathology , Lymphoma, Large-Cell, Anaplastic , Epidemiology , Pathology , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Epidemiology , Pathology , Lymphoma, T-Cell, Peripheral , Epidemiology , Pathology , T-Lymphocytes
15.
An. bras. dermatol ; 88(6,supl.1): 132-135, Nov-Dec/2013. graf
Article in English | LILACS | ID: lil-696806

ABSTRACT

Primary cutaneous anaplastic large-cell lymphoma is part of the spectrum of CD30+ lymphoproliferative cutaneous processes, characterized by single or multifocal nodules that ulcerate, are autoregressive and recurrent. Extracutaneous dissemination may occur, especially to regional lymph nodes. Histology shows a diffuse, non-epidermotropic infiltrate , anaplastic large lymphoid cells of immunohistochemistry CD30+, CD4+, EMA-/+, ALK-, CD15- and TIA1-/+. Prognosis is good and does not depend on lymphatic invasion. Radiotherapy, removal of the lesion and/or low-dose methotrexate are the treatments of choice. The present study reports the case of a 57-year-old-woman presenting Primary cutaneous anaplastic large-cell lymphoma with multifocal lesions. The pacient evolved with pulmonary involvement 7 years later. She showed a good response to the treatment with low-dose methotrexate prescribed weekly.


Linfoma cutâneo primário de grandes células T anaplásicas faz parte do espectro de processos linfoproliferativos cutâneos CD30+ e caracteriza-se por nódulos únicos ou multifocais, ulcerados, autorregressivos e recidivantes. Pode haver disseminação extracutânea, principalmente para linfonodos regionais. O histológico mostra infiltrado difuso, não-epidermotrópico, grandes células linfóides anaplásicas de imunohistoquímica CD30+, CD4+, EMA-/+, ALK-, CD15- e TIA1-/+. O prognóstico é bom e independe da invasão ganglionar. Radioterapia, retirada da lesão e/ou metotrexato em baixas doses são os tratamentos de escolha. Este estudo relata o caso de uma mulher, 57 anos, com Linfoma cutâneo primário de grandes células T com lesões multifocais e que, após 7 anos, evoluiu com acometimento pulmonar. Apresentou boa resposta ao tratamento com metotrexato em baixas doses semanais.


Subject(s)
Female , Humans , Middle Aged , Lymphoma, Primary Cutaneous Anaplastic Large Cell/pathology , Skin Neoplasms/pathology , Biopsy , Dermatologic Agents/therapeutic use , Immunohistochemistry , Lung Neoplasms , Lymphoma, Primary Cutaneous Anaplastic Large Cell/drug therapy , Methotrexate/therapeutic use , Skin Neoplasms/drug therapy , Skin/pathology , Treatment Outcome
16.
Rev. MED ; 20(2): 118-121, jul.-dic. 2012. ilus
Article in Spanish | LILACS | ID: lil-681745

ABSTRACT

El linfoma de células B del tipo de la pierna, es una neoplasia rara y agresiva, con características clínicas, morfológicas e inmunofenotípicas distintivas. Está clasificada dentro del grupo de linfomas cutáneos primarios de células B y se presenta en edades avanzadas, con predominio en mujeres y altas tasas de recurrencia. Presentamos el caso de una mujer de 63 años de edad, con diagnóstico clínico e inmunofenotípico de linfoma cutáneo de células B del tipo de la pierna.


Primary cutaneous B−cell lymphoma, leg type, is an uncommon and aggressive neoplasm with unique clinical features, morphology and immunophenotype. It is classified as a primary B−cell cutaneous lymphoma and it occurs in advanced age and women with high rates of recurrences. We present the case of a 63−year−old woman, with clinical and immunophenotypic diagnosis of primary cutaneous B−cell lymphoma, leg type.


O linfoma de células B do tipo da perna é uma neoplasia rara e agressiva, com características clínicas, morfológicas e imunofenotípicas distintivas. Está classificado dentro do grupo de linfomas cutâneos primários de células B e se apresenta em idades avançadas, com predomínio em mulheres e altas taxas de recorrência. Apresentamos o caso de uma mulher de 63 anos de idade, com diagnóstico clínico e inmunofenotípico de linfoma cutâneo de células B do tipo da perna.


Subject(s)
Humans , Middle Aged , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, Follicular , Lymphoma, Large B-Cell, Diffuse
17.
Rev. colomb. cancerol ; 15(4): 178-189, dic. 2011. tab
Article in Spanish | LILACS | ID: lil-661940

ABSTRACT

Objetivos: Describir los diferentes linfomas cutáneos conforme a la clasificación WHO- EORTC, observados en el Instituto Nacional de Cancerología (INC) entre el 1° de enero de 1995 y abril de 2008. Métodos: Estudio retrospectivo, descriptivo, donde se incluyó a los pacientes con diagnóstico de linfoma cutáneo en el INC desde enero de 1995 hasta abril de 2008. La ubicación de las historias clínicas se realizó utilizando la base de datos del Departamento de Patología. El análisis estadístico se realizó mediante el programa Epi info 2008. Resultados: Se revisaron 252 historias y se incluyó a 160 pacientes en el estudio: 139 linfomas T (87%) y 21 linfomas B (13%). El linfoma más común fue la micosis fungoide (63% de los casos). Dentro de las variantes descritas de micosis fungoide (MF) llamó la atención la frecuencia de MF hipopigmentadas (13%). El grupo de enfermedades linfoproliferativas CD30+ fue el segundo en frecuencia dentro de los linfomas T. En el grupo de linfomas B los linfomas indolentes de excelente pronóstico se presentaron en un 5% de los casos. Conclusiones: Los linfomas cutáneos primarios son tumores raros. Predominan los linfomas de células T sobre los B. La mayoría de los casos son linfomas de bajo grado, y deben tratarse con terapias dirigidas a la piel. Dentro de cada categoría existen linfomas que van a progresar y comprometer órganos internos.


Objectives: To describe different cutaneous lymphomas in accordance with WHO-EORTC classification under observation at the National Cancer Institute (NCI) between January 1, 1995 and April, 2008. Methods: A descriptive, retrospective study was carried out which included patients diagnosed with cutaneous lymphoma at the NCI from January 1995 until April 2008. Clinical cases were taken from Pathology Department data base. Statistical analysis was performed with Epi 2008 info program. Results: A total of 252 case histories were reviewed, and 160 patients were included in the study: 139 T-cell lymphomas (87%) and 21 B-cell lymphomas (13%). The most common (63% of cases) was mycosis fungoides (MF). Among the variations of MF described, the frequency of hypopigmented MF (13%) stood out. The lymphoproliferative CD30+ disease group was the second most frequent among T-cell lymphomas. In the B-cell lymphoma group, the indolent lymphomas with excellent prognosis made up 5% of cases. Conclusions: Primary cutaneous lymphomas are rare tumors. T-cell lymphomas predominate over B-cell. Most cases are low grade lymphomas and should be treated with skin therapies. Within each category, lymphomas exist that will progress and compromise internal organs.


Subject(s)
Humans , Male , Female , Young Adult , Aged , Cohort Studies , Epidemiologic Studies , Epidemiology, Descriptive , Lymphoma, Non-Hodgkin/classification , Mycosis Fungoides/classification , Retrospective Studies , Colombia/epidemiology , Histiocytoma, Benign Fibrous/classification , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous/classification
18.
Dermatol. argent ; 17(4): 283-293, jul.-ago.2011. ilus, graf
Article in Spanish | LILACS | ID: lil-724152

ABSTRACT

Introducción. Los síndromes linfoproliferativos que expresan CD30+ en la piel (SLP CD30+) incluyen las formas cutáneas primarias (SLP-CP) CD30+ (papulosis linfomatoide (LyP), linfoma anaplásico a células grandes primario cutáneo (PC-ALCL) y casos borderline) que tienen un buen pronóstico y las secundarias a otro linfoma (linfoma anaplásico de células grandes (ALCL) secundario a micosis fungoide (MF-T), síndrome de Sézary, LyP, enfermedad de Hodgkin), así como tipos infrecuentes de linfomas primarios que expresan este marcador, cuyo comportamiento es más agresivo (leucemia/ linfoma de células T del adulto o el linfoma angiocéntrico cutáneo de células T de la infancia tipo hidroa). También hay que considerar a las infiltraciones cutáneas secundarias a un ALCL sistémico de origen nodal. Es evidente que basándose sólo en los hallazgos histopatológicos es virtualmente imposible diferenciar estas entidades, y por lo tanto los datos de la presentación clínica e incluso muchas veces de la evolución, son cruciales para establecer un diagnóstico definitivo. Objetivos. Describir las características clínicas, histopatológicas y el pronóstico de los pacientes asistidos en la Unidad de Dermatología del Hospital General de Agudos Dr. Cosme Argerich con diagnóstico de SLP CD30+, y realizar una revisión de la literatura. Materiales y métodos. Estudio retrospectivo de revisión de la base de datos de pacientes con diagnóstico histológico e inmunohistoquímico confirmado de SLP CD30+ desde noviembre de 1995 hasta septiembre de 2010. Se evaluó diagnóstico, edad al inicio de la enfermedad, sexo, estadio, tiempo de evolución, respuesta al tratamiento y sobrevida. Resultados. Fueron evaluados 26 pacientes con diagnóstico confirmado de SLP CD30+. De los 26 casos incluidos, 19 (73%) correspondieron a SLP-CP CD30+, 11 (58%) LyP (de las cuales 4 se asociaron a MF), 3 (16%) borderline, y 5 (26%) PC-ALCL...


CD30(+)-lymphoproliferative disorders (CD30(+)-LPD) constitute a heterogeneous groupof neoplasms with different therapeutic approaches and outcomes. They can be primary cutaneous,including lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (PC-ALCL)andborderlinecases. A second group is secondary to another lymphoma, such as anaplastic large celllymphoma (ALCL) occurring after mycosis fungoides (MF-T), Sezary syndrome, LyP, and Hodgkin’s disease.Another different group of disorders showing CD30(+) expression, not belonging to the groups abovementioned, must be kept in mind in order to perform a differential diagnosis, including rare primarylymphomas, such as adult T cell leukemia/lymphoma, or angiocentric cutaneous T-cell lymphomaof childhood (hydroa-like lymphoma), and secondary skin involvement due to a systemic ALCL. Asdifferential diagnosis among them is virtually impossible based on histologic features alone, data on theclinical presentation and evolution are crucial to establish a correct diagnosis.


Subject(s)
Humans , Lymphoproliferative Disorders , Lymphoma, Primary Cutaneous Anaplastic Large Cell
19.
Folia dermatol. peru ; 22(1): 29-33, ene.-abr. 2011. ilus, tab
Article in Spanish | LILACS, LIPECS | ID: lil-664996

ABSTRACT

El linfoma cutáneo primario periférico de células T no especificado representa un grupo de linfomas que por sus características clínicas, histológicas e inmunofenotípicas no pueden ser clasificados dentro de las categorías de linfomas cutáneos primarios de células T, es por lo tanto un diagnóstico de exclusión. Estos linfomas presentan un curso desfavorable, con un pronóstico muy pobre. Presentamos el caso de un paciente varón de 79 años con diagnóstico de linfoma cutáneo de células T periférico, sin antecedente de micosis fungoides y con evolución desfavorable.


Primary cutaneous peripheral T-cell not unspecified lymphoma represents a group of lymphomas that cannot be classified among primary cutaneous T cells by theirs clinical, histological and immunophenotypic features; it is therefore a diagnosis of exclusion. These lymphomas have an unfavorable course with a poor prognosis. We report the case of a 79 year old male patient diagnosed with peripheral cutaneous T-cell lymphoma with no history of mycosis fungoides and unfavorable outcome.


Subject(s)
Humans , Male , Aged , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous
20.
Korean Journal of Dermatology ; : 931-935, 2011.
Article in Korean | WPRIM | ID: wpr-228812

ABSTRACT

Mycosis fungoides (MF) is the most frequent cutaneous T cell lymphoma (CTCL). Since the major tumor cell of MF is the helper T cell, positive markers are usually CD3, CD4 and CD45RO. Some MFs show CD30 positivity and the major differential diagnosis for MF with CD30 positivity includes transformed MF and MF concurrent with primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. As each disease shows a different prognosis, an exact diagnosis is crucial for proper treatment. We now report a case of 44-year-old male patient with mycosis fungoides which developed several papules on preexisting MF patches. On biopsy of the newly formed papules, CD 30 positive cells were observed and the histologic features were consistent with lymphomatoid papulosis. Both the lesions of lymphomatoid papulosis and MF responded well to narrow band ultraviolet B phototherapy.


Subject(s)
Adult , Humans , Male , Biopsy , Diagnosis, Differential , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Lymphomatoid Papulosis , Mycosis Fungoides , Phototherapy , Prognosis
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