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Abstract Background An increased risk of Secondary Malignancies (SMs) in Mycosis Fungoides (MF) has been suggested previously. However, the relationship between this risk and the features of MF is not well-known. Objective To investigate the rate and types of SMs in a large cohort of MF patients focusing on the associated features of these patients. Methods The demographic features, subtype, and stage of MF, as well as the temporal relationship between the diagnosis of MF and the development of SMs were determined. Major clinical features of MF in this group were compared with MF patients without association of SMs. Results Among 730 MF patients with a mean follow-up period of 67.9 ± 52.4 months, 56 SMs were identified in a total of 52 (7.1%) patients. While 28.8% of patients were previously diagnosed with other malignancies, then subsequently had a diagnosis of MF, it was vice versa in 53.8% of patients. Most of the SM-associated MF patients had early-stage (80.7%) and classical type of MF (86.5%) without a significant difference from MF patients without association of SMs; 85.5% and 72.5%, respectively. The most commonly identified SMs were hematologic malignancies (64.3%) including lymphomatoid papulosis (n = 22), Hodgkin's lymphoma (n = 4), non-Hodgkin's lymphoma (n = 5), polycythemia vera (n = 2). Other most commonly associated malignancies were breast cancer (n = 4), prostate cancer (n = 3), renal cell carcinoma (n = 2), melanoma (n = 2), and Kaposi's sarcoma (n = 2). Study limitations A single tertiary dermatology center study with a retrospective design. Conclusion Apart from the well-known lymphomatoid papulosis association, systemic hematological malignancies were also quite common in the large cohort of MF patients.
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RESUMEN Se presenta el caso de un paciente masculino de 21 años, quien el 30 de diciembre de 2017 comenzó con aumento de volumen, rubor y calor en región lateral derecha del cuello, posterior a la presencia de un forúnculo en región occipital del cuero cabelludo. Se inició tratamiento con antibiótico, pero aparecieron lesiones eritematopapulosas en tronco y extremidades que se interpretaron como una reacción adversa al medicamento. Comenzó con fiebre mantenida de 380C, decaimiento marcado, pérdida de peso, hepatomegalia y adenopatías pequeñas y no dolorosas en regiones inguinales y axilares, por lo que fue ingresado el 14 de enero de 2018 en el Hospital Gustavo Aldereguía Lima, de Cienfuegos y se impuso tratamiento con antibiótico ante un perfil hematológico que indicaba sepsis asociada. Se realizó aspiración con aguja fina que resultó negativa y se hicieron coordinaciones para biopsia. El 15 de enero empeoró el estado clínico del paciente con fiebre elevada, desorientación, marcada excitación, toma de conciencia e insuficiencia respiratoria, por lo que falleció el día 16. La autopsia reveló como causa básica de muerte la granulomatosis linfomatoide. Por ser esta una entidad infrecuente, desconocida por gran cantidad de clínicos y cuyo tratamiento y pronóstico no está definido, se decidió la presentación del caso.
ABSTRACT The case of a 21-year-old male patient is presented, who on December 30, 2017 began with increased volume, flushing and heat in the right lateral region of the neck, after the presence of a boil in the occipital region of the scalp. Treatment with antibiotic was started, but erythe-matopapular lesions appeared on the trunk and extremities that were interpreted as a medication adverse reaction. He started with a maintained fever of 38 0C, malaise, weight loss, hepatomegaly and small non-painful adenopathies in the inguinal and axillary regions, so he was admitted on January 14, 2018 at the Gustavo Aldereguía Lima Hospital in Cienfuegos and was treated with antibiotic before a hematological profile which indicated associated sepsis. A fine-needle aspiration was performed, which was negative and arrangement for biopsy was made. On January 15, the clinical condition of the patient worsened with high fever, disorientation, marked excitement, awareness and respiratory insufficiency, and he died on the 16th. Autopsy revealed lymphomatoid granulomatosis as the main cause of death.
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Purpose To investigate the clinicopathological features of primary subcutaneous lymphomatoid granulomatosis (LYG). Methods A case of primary subcutaneous LYG was observed by analysis of the clinical, histological features, immunophenotype and molecular pathology with review of the related literature. Results The male patient, 78-year-old, inadvertently found a mass of right axillary for more than 10 days. The boundary of the mass was clear, it seemed to have a capsule, the cut surface was grayish yellow and grayish red, the texture was medium. A large amount of coagulative necrosis was observed in the center of the mass under microscope. The peripheral area showed a morphological change of panniculitis, accompanied by pleomorphic lymphoid infiltration, showed central and vascular destructive infiltration, pathological mitosis was occasionally observed. Immunophenotyping showed that atypical large lymphoid cells expressed CD45 RB, CD20, CD30, while CD3, CD15, CD56, TIA-1, Granzyme B, ALK, CD21, Langerin and CD1 a, S-100 and CK (AE1/AE3) were negative. The proliferation index of Ki-67 ranged from 50％ to 60％. EBER in situ hybridization showed that positive cells were> 20/HPF.Neither acid fast staining nor TB-DNA testing supported tuberculosis. Molecular pathology found clonal Ig K gene rearrangement, TCRB + TCRG gene rearrangement showed the absence of monoclonal proliferating T cell population. Conclusion The primary subcutaneous LYG is a rare tumor. which can be diagnosed by combination of morphology, immunophenotype and molecular pathology.
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Lymphomatoid papulosis type E (LyP) is a recently described subtype of LyP characterized by an angioinvasive infiltrate of atypical lymphocytes expressing CD30. We present a case of type E LyP with extensive cutaneous necrosis in the histopathological evaluation which was misdiagnosed as an ulcerative form of bacterial skin infection. The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.
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Lymphomatoid papulosis type E (LyP) is a recently described subtype of LyP characterized by an angioinvasive infiltrate of atypical lymphocytes expressing CD30. We present a case of type E LyP with extensive cutaneous necrosis in the histopathological evaluation which was misdiagnosed as an ulcerative form of bacterial skin infection. The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.
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Context: Lymphomatoid granulomatosis (LYG) is a rare B-lymphoproliferative disorder characterised by an angiocentric and angiodestructive pattern along with Epstein - Barr virus (EBV) association. It is one of the diagnostic challenges in lymphoma pathology. Deregulation of EBV immune surveillance is one of the narrated hypotheses in the literature. Extrapulmonary manifestations are rare with LYG. Morphological grading is done based on the number of EBV-positive B cells, which is useful to strategize treatment protocol. Aims: We report here a series of nine cases of LYG to discuss the clinical, histological, and immunohistochemistry findings. Settings and Design: This is the first case series from India in published literature. Subjects and Methods: We reviewed cases of LYG diagnosed at our center for the past 11 years (2006-2016). A total of nine cases were included in this study. Histomorphology was studied in conjunction with immunohistochemistry and clinical details. Cases without classical morphology and negative for EBV immunostain were excluded from the study. Results: There were nine patients in our study (7 males and 2 female; M:F ratio 3.5:1). The age of these patients ranged from 4 years to 57 years (mean age: 30 years). The most common site involved was the lung (4, 44%), followed by the skin (2, 22%), central nervous system (2, 22%) and lymph node (1, 11%). One patient had primary immunodeficiency. Another patient had undergone renal transplant 11 years before the development of the lesion. Angiocentricity and angioinvasion were appreciated in all nine cases (9/9) with necrosis in four cases (44%) and ill-defined histiocytic aggregates in three cases (33%). The histological features were as follows: Grade 1(4 cases, 44%), Grade 2(2 cases, 22%), and Grade 3(3 cases, 33%). Conclusion: LYG is a rare EBV driven angiodestructive disease with predominantly lung involvement as well as isolated extrapulmonary sites as seen in our study. It is often progressive and ultimately fatal in the absence of appropriate treatment. Grading of the lesion helps to initiate the appropriate treatment of choice.
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Lymphomatoid granulomatosis (LG) is a kind of Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. Pathologically, it is characterized by angioinvasion and large EBV-positive atypical B cells in an inflammatory background with numerous T cells. Clinically, it mainly involves the bilateral lungs in the mid and lower lung fields, extra-pulmonary sites such as central nervous system, skin have also been reported. There are still no uniform treatment protocols about the disease, and pathological grading is a main reference. Overall, the prognosis is poor. Because of the rarity, lack of typical symptoms and special imaging characteristics, LG was often misdiagnosed. The article summarizes the diagnosis, treatment and prospects of LG to improve the clinician's understanding of the disease.
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Objective@#To report the first case of lymphomatoid gastropathy in China, and to demonstrate the clinical characteristics, diagnostic approach, treatment and prognosis in this kind of patients.@*Methods@#One patient was diagnosed as lymphomatoid gastropathy at Peking Union Medical College Hospital, and her clinical characteristics, lab data, treatment and follow-up outcomes were reviewed.@*Results@#A case of a 51-year-old female was presented, who underwent esophagogastroduodenoscopy (EGD) due to slight epigastric discomfort. EGD revealed multiple ulcers and erosions. Biopsies showed atypical lymphocytes infiltration with CD3(+), CD56(+), CD20(-), CD8(-), TIA(+), Granzyme B(-) and Ki-67 (75%). Epstein-Barr virus-encoded RNA in situ hybridization was negative. Four months later, repeated EGD examination showed regression of the lesions without specific treatment.@*Conclusion@#Lymphomatoid gastropathy was a unique disease entity mimicking NK/T-cell lymphomas in pathology, with the quite different profile of treatment and prognosis. It’s important to consider this issue during the differential diagnosis to avoid any excessive treatment.
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Objective: To report the first case of lymphomatoid gastropathy in China, and to demonstrate the clinical characteristics, diagnostic approach, treatment and prognosis in this kind of patients. Methods: One patient was diagnosed as lymphomatoid gastropathy at Peking Union Medical College Hospital, and her clinical characteristics, lab data, treatment and follow-up outcomes were reviewed. Results: A case of a 51-year-old female was presented, who underwent esophagogastroduodenoscopy (EGD) due to slight epigastric discomfort. EGD revealed multiple ulcers and erosions. Biopsies showed atypical lymphocytes infiltration with CD3(+), CD56(+), CD20(-), CD8(-), TIA(+), Granzyme B(-) and Ki-67 (75%). Epstein-Barr virus-encoded RNA in situ hybridization was negative. Four months later, repeated EGD examination showed regression of the lesions without specific treatment. Conclusion: Lymphomatoid gastropathy was a unique disease entity mimicking NK/T-cell lymphomas in pathology, with the quite different profile of treatment and prognosis. It's important to consider this issue during the differential diagnosis to avoid any excessive treatment.
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Femenino , Humanos , Persona de Mediana Edad , Biopsia , China , Inmunofenotipificación , Hibridación in Situ , Enfermedades Linfáticas , Linfoma de Células T Periférico , GastropatíasRESUMEN
La papulosis linfomatoide forma parte del espectro de los procesos linfoproliferativos cutáneos primarios de células T CD30+. Es una enfermedad rara de etiopatogenia incierta y compleja. El diagnóstico diferencial puede a veces resultar muy difícil. Se describió el caso de una mujer de 80 años con el diagnóstico, particularmente atípico desde la visión histopatológica, en el cual la correlación anatomoclínica ha sido un importante aspecto que lo hace interesante. El objetivo es comunicar un caso de presentación poco frecuente en la práctica médica (AU).
Lymphomatoid papulosis is part of the primary skin lymph proliferative processes of the T CD30+ cells. It is a rare disease of complex and uncertain etiopathogenesis. The differential diagnosis could be very difficult sometimes. The described case was the one of a female patient, aged 80 years with that diagnosis, particularly atypical from the histopathological point of view, where the anatomoclinical correlation has been an important aspect making it interesting. The objective is to inform a case of infrequent presentation in the medical practice (AU).
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Humanos , Femenino , Adulto , Neoplasias Cutáneas/epidemiología , Papulosis Linfomatoide/epidemiología , Registros Médicos , Papulosis Linfomatoide/complicaciones , Papulosis Linfomatoide/diagnóstico , Papulosis Linfomatoide/patología , Diagnóstico Diferencial , Lesiones por Desenguantamiento/diagnóstico , Linfoma/diagnósticoRESUMEN
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)
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Humanos , Femenino , Adulto , Linfoma , Linfoma Anaplásico Cutáneo Primario de Células Grandes , Papulosis Linfomatoide , Micosis Fungoide , Linfocitos TRESUMEN
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.
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Humanos , Masculino , Femenino , Adolescente , Persona de Mediana Edad , Anciano de 80 o más Años , Neoplasias Cutáneas/patología , Linfoma Anaplásico Cutáneo Primario de Células Grandes/patología , InmunohistoquímicaRESUMEN
Objective@#To study the clinicopathologic characteristics and immunophenotype of lymphomatoid papulosis(LyP), followed by exon mutation analysis with focus on gene mutations involved in apoptosis pathway and other possible pathogenic genes.@*Methods@#Clinical data analysis and immunohistochemical staining were carried out in 20 cases of LyP. Whole exome sequencing technology was employed in 2 cases of type C of LyP.@*Results@#Of the 20 cases, there were 9 males and 11 females with a median age of 28.6 years. Nineteen patients presented with multiple papules and nodules, and one case presented with only one tumor nodule. Of the fifteen cases with available followed-up data, all were alive (20-155 months). Histologically, the tumors primarily involved the dermis and subcutaneous layer, in which 6 were type A, 3 were type B, 10 were type C and 1 was type D. Main infiltration patterns included wedge-shaped, band-like, sheets and large nodular. Immunohistochemistry showed that most cases expressed CD30 in the large tumor cells. Sixteen cases expressed CD3, 17 cases expressed CD4 and 8 cases expressed CD8. Sixteen cases expressed TIA1. Ten cases expressed GrB and 1 case expressed CD15. All but one case did not expressed CD20. All cases did not express ALK1.A total of 101 common non-synonymous mutations were detected in 2 cases of LyP type C by whole exome sequencing, including 87 missense mutations, 6 missense mutation/frame-shift deletions, 2 missense mutation/nonframe-shift deletions, 5 frame-shift deletions, 1 missense mutations/synonymous mutation. Syndecan-1(SDC1), COL4A1, Laminin-5 were involved in the extracellular matrix receptor pathway.@*Conclusions@#Clinical presentations are crucial for the diagnosis of LyP. LyP has a favorable prognosis. SDC1, COL4A1 and Laminin-5 gene mutations may be associated with tumor recurrence or progression into a higher gradelymphoma.
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Three histological subtypes of lymphomatoid papulosis (LyP), type A (histiocytic), type B (mycosis fungoides like) and type C (anaplastic large cell lymphoma like) are well recognized. Two new histological variants, type D (simulating an aggressive epidermotropic cytotoxic lymphoma) and type E (angioinvasive type) has been described recently. We describe a 27‑year‑old man presented with a history of asymptomatic erythematous papules on both upper and lower limbs noted since 10 years of age. There were no systemic symptoms. Biopsy revealed an atypical dermal lymphoid infiltrate with epidermotropism, and the immunohistochemical markers showed a diffuse positivity for CD3, CD8, CD56, T1A and granzyme B with the focal positivity of CD30. All other relevant tests were normal. In this case report of a recently described delineated variant of LyP we emphasize the indolent course of this entity although the histology would suggest a more aggressive disease.
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Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.
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Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.
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Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive neoplastic proliferation of B and T lymphocytes commonly involving the lungs. Epstein-Barr virus is commonly detected in lesional cells. We report a case of a 54-year-old female with underlying monoclonal gammopathy of unknown significance who presented with a 4 week history of dyspnea and cough. Computed tomography scan of the chest showed multiple lung nodules as well as endobronchial narrowing causing atelectasis at the left upper lobe. Bronchoscopic findings revealed obstruction at the lingula segment due to endobronchial mass as a rare presentation. Bronchoscopic biopsy was diagnosed with LYG grade 1. After treatment, the endobronchial mass and lung lesions were completely resolved. However, the patient eventually evolved to malignant lymphoma after 1 year.