Neoplasia de células dendríticas plasmocitoides blásticas, un reporte de caso / Blast plasmacytoid dendritic cell neoplasia, a case report

Introducción: La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una patología agresiva y representa menos del 1% de neoplasias hematológicas, se caracteriza por lesiones cutáneas nodulares violáceas sin evidencia de adenopatías en la mayoría de casos. Estudios observacionales demuestran que el Protocolo de quimioterapia Hyper-CVAD y la consolidación con tras-plante de células progenitoras hematopoyéticas se han asociado con una mayor supervivencia general. Caso Clínico: mujer de 82 años con antecedentes de Diabetes Mellitus tipo con cinco meses de lesiones hiperpigmentadas, elevadas, induradas, violáceas no dolorosas en mejilla, brazos, tórax anterior y posterior y piernas. Evolución: En citometría de flujo se determinó un fenotipo compatible con células patológicas (5.86%) con CD123++, HLADR+++, NG2++, CD56+++, CD4++, que sugiere una NCDPB. La biopsia de médula ósea presentó infiltración. PET CT posterior a terapia corticoide: no evidencia enfermedad tumoral macroscópica metabólicamente activa. Se inicia tratamiento con Dexametasona, con lo que las lesiones cutáneas disminuyeron en un 80%. Se inició Quimioterapia Protocolo CHOP like, ha recibido 6 ciclos hasta octubre del 2021, actualmente en remisión completa. Conclusión: En el presente caso el curso clínico de la NCDPB no fue agresivo hasta el momento del cierre del caso presentando disminución del 80% de las lesiones.

Introduction: blast plasmacytoid dendritic cell neoplasia (BPDCN) is an aggressive pathology and represents less than 1% of hematological neoplasms, it is characterized by violaceous nodular skin lesions without evidence of adenopathy in most cases. Observational studies show that the Hyper-CVAD chemotherapy protocol and consolidation with transplantation of hematopoietic progenitor cells have been associated with greater overall survival. Clinical case: a 82-year-old woman with a history of type Diabetes Mellitus with five months of hyperpigmented, raised, indurated, non-painful violaceous lesions on the cheek, arms, anterior and posterior thorax and legs. Evolution: Flow cytometry determined a phenotype compatible with pathological cells (5.86%) with CD123 ++, HLADR +++, NG2 ++, CD56 +++, CD4 ++, which suggests a BPDCN. The bone marrow biopsy showed infiltration. PET CT after corticosteroid therapy: there is no evidence of metabolically active macroscopic tumor disease. Dexamethasone treatment was started, with which skin lesions decreased by 80%. The CHOP-like Chemotherapy Protocol was started, she has received 6 cycles until October 2021, currently in complete remission. Conclusion: In the present case, the clinical course of NCDPB was not aggressive until the moment of closure of the case, presenting a decrease of 80% of the lesions.

Mycosis fungoides and Sézary syndrome: focus on the current treatment scenario

Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

Research Progression of Subcutaneous Panniculitis-like T-Cell Lymphoma--Review / 中国实验血液学杂志

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare cutaneous malignant lymphoma derived from cytotoxic T cells that mainly involves subcutaneous adipose tissue rather than epidermis and dermis. It usually occurs in young and middle-aged population, and the etiology is currently unclear. Clinically, SPTCL is characterized by subcutaneous plaques, nodules, and skin ulcers with swell and ache, mainly presenting in limbs and trunk. SPTCL has been restricted to cases that express α/β phenotype, whereas cases with γ/δ phenotype are categorized to cutaneous γ/δ

Linfomas cutáneos: serie de casos en México / A case series of cutaneous lymphomas in Mexico

Resumen Introducción: Los linfomas cutáneos primarios son un grupo heterogéneo de neoplasias de células T y B que se presentan en la piel, sin ninguna evidencia de enfermedad extracutánea en el momento del diagnóstico, y muestran diferencias considerables en histologia, fenotipo y pronóstico. Se consideran neoplasias poco frecuentes. Casos clínicos: Se presentan cinco casos de linfomas cutáneos diagnosticados en el Hospital Infantil de México Federico Gómez durante el periodo de 2010 a 2018. Las presentaciones clínicas más frecuentes en estos pacientes fueron dermatitis, costras hemáticas y úlceras necróticas. El inmunofenotipo más común fue el linfoma cutáneo no Hodgkin T/NK extranodal nasal primario. El esquema de tratamiento que se utilizó en la mayoría de los pacientes fue SMILE. El promedio de tiempo al diagnóstico fue de 7 meses. Conclusiones: El pronóstico depende del estadio de la enfermedad al diagnóstico, grado de afectación de la piel y presencia o ausencia de enfermedad extracutánea. Los linfomas cutáneos primarios son neoplasias poco frecuentes. Debido al diagnóstico tardío, el estadio de la enfermedad suele ser avanzado, por lo que, generalmente, el comportamiento es agresivo.

Abstract Background: Primary cutaneous lymphomas are a rare heterogeneous group of T and B cell skin neoplasms without any evidence of extracutaneous disease at the time of diagnosis, which show considerable differences in histology, phenotype and prognosis. Case reports: Five cases of cutaneous lymphomas treated at the Hospital Infantil de México Federico Gómez from 2010 to 2018 are described. The most frequent clinical presentations in these patients were dermatitis, blood scabs, and necrotic ulcers. The most common immunophenotype was non-Hodgkin T/NK primary nasal extranodal cutaneous lymphomas. The treatment scheme used in most patients was SMILE. The average time to diagnosis was 7 months. Conclusions: The prognosis depends on the stage of the disease at diagnosis, the degree of skin involvement, and the presence of extracutaneous disease. As primary cutaneous lymphomas are infrequent neoplasms, the stage of the disease is usually advanced and generally shows an aggressive behavior due to a late diagnosis.

Mycosis Fungoides and Variants of Mycosis Fungoides: A Retrospective Study of 93 Patients in a Chinese Population at a Single Center

Case for diagnosis. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Abstract: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease, with an indolent evolution and benign course. The classic presentation is a solitary nodule on the face or trunk. The disorder's rarity and clinical and histopathological characteristics, can make the diagnosis difficult. We present the case of a 36-year-old Caucasian woman with a purplish erythematous nodule, hardened, shiny, asymptomatic, on the left nasal ala, which had grown progressively for 45 days. Histopathological examination and immunohistochemistry panel demonstrated alterations consistent with primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was complete remission of the condition within 60 days of treatment with potent occlusive corticosteroids.

Treatment of primary cutaneous anaplastic large cell lymphoma

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.

Cutaneous T-cell lymphomas in the revised 4th edition of World Health Organization classification of tumors of hematopoietic and lymphoid tissues (2017)

Abstract: Recently, the World Health Organization published the revised 4th edition of its classification of tumors of hematopoietic and lymphoid tissues. The present paper is a concise comparative review of the main primary cutaneous T-cell hematopoietic tumors, with emphasis on their immunohistochemical profiles.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides

Abstract: Background: Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. Objective: To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. Methods: This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. Results: One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. Study Limitations: Small sample and its retrospective analysis. Conclusions: Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.

Mycosis Fungoides Responsive to Oral Alitretinoin and Ultraviolet B Phototherapy: A Case Report / 대한피부과학회지

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. Patients with early stage disease usually respond well to conventional therapies, with a relatively favorable prognosis. However, a few patients are refractory to treatment and need alternative strategies, even at the patch and plaque stages. We report the case of a middle-aged woman with long-standing and refractory mycosis fungoides that responded to combination therapy with the 308-nm excimer laser and oral alitretinoin.

The Incidence of Other Primary Cancers in Patients with Cutaneous Lymphoma

BACKGROUND: Skin cancer is the most common other primary cancer in patients with lymphoma. However, an intriguing association between cutaneous lymphoma and other primary cancers has been suggested in a few studies. OBJECTIVE: This study investigated other primary cancers in patients with cutaneous lymphoma to evaluate the risk for occurrence of each type of cancer. METHODS: We screened for other primary cancers in 428 patients with cutaneous lymphoma. Clinical features were analyzed according to the lineage and origin of the lymphomas. We calculated the standardized incidence ratio with statistical analysis for each group according to age. RESULTS: Among 330 patients with cutaneous T cell lymphoma and 98 with cutaneous B cell lymphoma, a total of 43 cancers in 38 patients were finally included. Other primary cancers were prevalent in patients with cutaneous B cell lymphoma and patients with secondary cutaneous lymphoma. However, those differences were not significant when the age was calibrated by multiple logistic regression. Metachronously higher standardized incidence ratios were observed for primary lung (standardized incidence ratio [SIR], 14.81; 95% confidence interval [CI], 3.05~39.54), skin (SIR, 68.05; 95% CI, 14.03~181.62), and breast (SIR, 12.91; 95% CI, 1.56~41.41) cancers with statistical significance. CONCLUSION: Other primary cancers more preferentially occurred in patients with cutaneous lymphoma. Clinicians should carefully examine patients with cutaneous lymphoma for other cancers, especially lung, skin, and breast cancers.

Follicular mycosis fungoides: A report of 2 cases in Filipino octogenarians

Introduction@#Folliculotropic mycosis fungoides is a rare and aggressive subtype of mycosis fungoides characterized by small to medium-sized malignant T-cells that typically infiltrate the hair follicle. It represents 4% of primary cutaneous lymphomas and less than 10% of patients with MF.@*Case Summary@#We report 2 cases of 80-year old female, who presented with a 3-year history of an erythematous plaque on the left infraorbital area and forehead, and the other with an 8-month history of multiple erythematous indurated plaques on the left cheek, left eyebrow and malar area. Clinical presentation, histopathology and immunohistochemistry findings revealed a diagnosis of folliculotropic mycosis fungoides.@*Conclusion@#Folliculotropic mycosis fungoides has distinct clinical and microscopic features. Evaluation of clinical, pathologic, and immunophenotypic findings are important to identify this rare form of cutaneous T-cell lymphoma.

Clinical outcomes and prognostic factors in patients with mycosis fungoides who underwent radiation therapy in a single institution

PURPOSE: We aimed to evaluate clinical outcomes including progression-free survival (PFS), overall survival (OS), partial response, and complete response in patients who underwent radiation therapy (RT) for mycosis fungoides (MF). Also, we sought to find prognostic factors for clinical outcomes. MATERIALS AND METHODS: Total 19 patients confirmed with MF between 1999–2015 were retrospectively reviewed. Clinical and treatment characteristics, clinical outcomes, and and toxicities were analyzed. RESULTS: Eleven patients were treated with total skin electron beam radiotherapy (TSEBT) and 8 patients with involved field radiation therapy (IFRT) with median dose of 30 Gy, respectively. The median time interval from diagnosis to RT was 2.6 months (range, 0.4 to 87.3 months). The overall response rate was 100%; 11 patients (57.9%) had a complete response and 8 patients (42.1%) a partial response. The presence of positive lymph node at the time of consultation of RT was associated with lower OS (p = 0.043). In multivariate analysis, PFS was significantly lower for patients with increased previous therapies experienced following RT (p = 0.019) and for patients showing PR during RT (p = 0.044). There were no reported grade 3 or more skin toxicities related with RT. CONCLUSION: Both IFRT and TSEBT are effective treatment for MF patients. Patients with short disease course before RT or complete response during RT are expected to have longer PFS. Positive lymph node status at the initiation of RT was associated woth poor OS, suggesting other treatment modalities such as low-dose RT for patients with low life-expectancy.

Epitheliotropic lymphoma with heart and kidney metastasis in a dog: case report / Linfoma epiteliotrópico em cão com metástase cardíaca e renal: relato de caso

A female adult dog, with a four-month history of pain and intense pruritus, which eventually resulted in sudden death, was referred for necropsy. Postmortem examination showed thoracic and abdominal serum-sanguineous exudates, multifocal infiltrative renal masses, and similar tumors in the heart. Histopathology revealed midsize infiltrative neoplastic proliferation composed of round cells, sparse cytoplasm, and large hyperchromatic nuclei. Immunohistochemistry revealed CD3+ and CD20-immunoexpression. Histopathological and immunohistochemical findings confirmed the diagnosis of epitheliotropic lymphoma with cardiac and renal metastasis.(AU)

Foi encaminhado para necropsia um cão adulto do sexo feminino, com histórico de dor e prurido intenso com evolução de quatro meses, que acabou resultando em morte súbita. O exame post mortem mostrou presença discreta de exsudato serossanguinolento em cavidades torácica e abdominal, massas renais infiltrativas multifocais e tumores semelhantes no coração. O exame histopatológico revelou proliferação neoplásica infiltrativa composta de células redondas, com citoplasma escasso, e grandes núcleos hipercromáticos. A análise imuno-histoquímica mostrou imunoexpressão CD3+e CD20. Os achados histopatológicos e imuno-histoquímico confirmaram o diagnóstico de linfoma epiteliotrópico com metástase cardíaca e renal.(AU)

Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute

Abstract: Background: Primary cutaneous T-cell lymphomas constitute a heterogeneous and rare group of diseases with regional particularities in Latin America. Objective: To determine the clinicopathological features, relative frequency and survival among patients from a Peruvian institution. Methods: Primary cutaneous T-cell lymphomas were defined based on the absence of extracutaneous disease at diagnosis. Classification was performed following the 2008 World Health Organization Classification of Neoplasms of the Hematopoietic and Lymphoid tissues. Risk groups were established according to the 2005 World Health Organization-EORTC classification for cutaneous lymphomas. Data of patients admitted between January 2008 and December 2012 were analyzed. Results: 74 patients were included. Mean age was 49.5 years. In order of frequency, diagnoses were: mycosis fungoides (40.5%), peripheral T-cell lymphoma not otherwise specified (22.95%), adult T-cell lymphoma/leukemia (18.9%), CD30+ lymphoproliferative disorders (6.8%), hydroa vacciniforme-like lymphoma (5.4%), extranodal NK/T-cell lymphoma (4.1%) and Sézary syndrome (1.4%). Predominant clinical patterns were observed across different entities. Mycosis fungoides appeared mainly as plaques (93%). Peripheral T-cell lymphoma not otherwise specified and adult T-cell lymphoma/leukemia presentation was polymorphic. All patients with hydroa vacciniforme-like lymphoma presented with facial edema. All cases of extranodal NK/T-cell lymphoma appeared as ulcerated nodules/tumors. Disseminated cutaneous involvement was found in 71.6% cases. Forty-six percent of patients were alive at 5 years. Five-year overall survival was 76.4% and 19.2%, for indolent and high-risk lymphomas, respectively (p<0.05). High risk group (HR: 4.6 [2.08-10.18]) and increased DHL level (HR: 3.2 [1.57-6.46]) emerged as prognostic factors for survival. Study limitations: Small series. Conclusion: Primary cutaneous T-cell lymphomas other than mycosis fungoides or CD30+ lymphoproliferative disorders are aggressive entities with a poor prognosis.

Granulomatous slack skin: a rare subtype of mycosis fungoides

Abstract: We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.

Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos con compromiso facial / Indolent CD8-positive t-cell lymphoid proliferation: on purpose three cases of facial commitment

Resumen La proliferación linfoide indolente cutánea CD8 positiva es una variante recientemente descrita de linfoma T cutáneo que se caracteriza por un nódulo, pápula o placa eritematosa de crecimiento lento que puede afectar la región facial o extrafacial. En el estudio de patología se caracteriza por un infiltrado monomorfo de linfocitosTalo largo de la dermis con presencia de zona de Grenz y ausencia de epidermotropismo. El infiltrado es característicamente CD8+ así como CD3+, TIA-1+, CD4-, CD56- CD30-, PD-1-, Granzima B- y EBER negativo. El índice de proliferación Ki-67 es inferior al 10% y se observan reordenamientos clonales de los genes del receptor de antígeno de la célula T, TCR. El seguimiento clínico es favorable y no se ha observado compromiso sistémico. Se presentan tres casos con compromiso facial (dos casos en pabellón auricular y un caso con compromiso nasal), con presentación clínica y hallaz gos histopatológicos típicos (curiosamente un caso con cambio de célula clara), y además se realizaron estudios de clonalidad.

Abstract Primary cutaneous indolent CD8-positive lymphoid proliferation is a recent variant of cutaneous T lymphoma that is characterized by nodule, papule or plaque erythematous with slow growth that can affect the facial or extrafacial region. In the histopathology study it is characterized by an infiltration of monomorphic T lymphocytes throughout the dermis with presence of Grenz zone and absence of epidermotropism. The infiltrate is characteristically CD 8+ and CD3+ TIA-1+ CD4-, CD56- CD30, PD-1, Granzyme B- and negative EBER. Ki-67 Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos proliferation index is less than 10% and clonal T-cell receptor gene rearrangements. Clinical follow-up is favorable and has not been observed systemic involvement. We present three cases with facial involvement (two cases in ear and one case with nasal commitment) with typical clinical presentation, histopathological findings (curiously a case with clear cell change) and clonality studies.

Micosis fungoide ampollar de presentación en palmas y plantas / Mycosis fungoide blister presentation in palms and plants

La micosis fungoide es el linfoma cutáneo primario de células-T más común y se caracteriza por presentar un amplio rango de variantes clínicas e histopatológicas [1]. La presentación ampollar de esta patología es muy rara y se han encontrado menos de una veintena de reportes en la literatura [2], por lo que se estima que la asociación con una presentación palmaris et plantaris sea aún menos común. A continuación, se presenta el caso clínico de una paciente de la ciudad de Quito-Ecuador quien cursaba con manifestaciones cutáneas de esta enfermedad sin afectación extracutánea.