Linfoma anaplásico de células grandes asociado a implantes mamarios diagnosticado mediante punción por aguja fina. Caso clínico

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare type of non-Hodgkin T-cell lymphoma, recently defined in the 2016 World Health Organization (WHO) classification of lymphoid neoplasms. It occurs more commonly when textured implants are used and appears clinically as a late seroma. Cytologically, these lesions are composed of large atypical cells with pleomorphic nucleus and an immunophenotype positive for T cell markers and CD30, and negative for ALK1. We report a 56-years-old woman with breast implants who developed a periprosthetic seroma three years after surgery. A fine needle aspiration of the lesion was carried out. Cytology and the immunocytochemical study revealed cells compatible with BIA-ALCL. The flow cytometric study was negative. Excisional biopsy of the capsule was performed, observing that the neoplastic cells were confined to the inner surface of the capsule. Imaging studies did not find evidence of disseminated disease. The present case demonstrates the importance of the study of any late periprosthetic effusion, which can be performed using fine needle aspiration.

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos / Primary cutaneous CD30+ anaplastic large T-cell lymphoma. Series of nine cases

Resumen Introducción: El linfoma anaplásico de células T grandes CD30+ es un linfoma primario cutáneo en el cual no hay evidencia de enfermedad sistémica; para su diagnóstico es necesario el estudio histopatológico. Objetivo: Presentar los casos diagnosticados en el Departamento de Dermatología del Hospital General "Dr. Manuel Gea González" con linfomas anaplásicos de células T grandes primarios cutáneos CD30+ durante un periodo de 24 años. Método: Estudio retrospectivo en el que realizó estadística descriptiva. Se recopiló información de sexo, edad, características clínicas, resultados de pruebas complementarias, tratamientos previos y actuales, reportes de los estudios histopatológicos y de inmunohistoquímica. Resultados: Entre 29 309 expedientes, se encontraron nueve casos (0.000034 %) con diagnóstico de linfoma anaplásico de células T CD30+. Se hizo la confirmación del diagnóstico histopatológico e inmunohistoquímico por dos dermatopatólogos certificados. La edad promedio fue de 61.2 años, hubo predominio del sexo femenino y de lesión papular o nodular y topografía variada como presentación clínica inicial. Conclusiones: El pronóstico del linfoma anaplásico de células T grandes CD30+ en la población estudiada fue dependiente del estadio clínico. El tratamiento en etapas tempranas tiene resultados favorables.

Abstract Introduction: CD30+ anaplastic large T cell lymphoma is a cutaneous primary lymphoma in which there is no evidence of systemic disease; histopathological study is required for its diagnosis. Objective: To present the cases diagnosed with primary cutaneous CD30+ anaplastic large T-cell lymphoma over a 24-year period in Hospital General "Dr. Manuel Gea González" Department of Dermatology. Method: Retrospective study. Descriptive statistics was carried out. Information was collected on gender, age, clinical characteristics, complementary test results, previous and current treatments, histopathological studies reports and immunohistochemistry test results. Results: Of 29 309 records, nine patients (0.000034%) with a diagnosis of CD30+ anaplastic T cell lymphoma were found. Histopathological and immunohistochemical diagnosis was confirmed by two certified dermatopathologists. Average age was 61.2 years, and there was a predominance of the female gender, with initial clinical presentation as a papular or nodular lesion and varied topography. Conclusions: The prognosis of CD30+ anaplastic large T cell lymphoma in the studied population was dependent on clinical stage. The treatment at early stages has favorable results.

Anaplastic Large Cell Lymphoma Involving Anterior Segment of the Eye

A 36-year-old woman was diagnosed with anaplastic large cell lymphoma (ALCL) by excisional biopsy of a left frontal skin lesion. During the first cycle of chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone), the patient complained of right ocular pain and inflammation. Cytologic examination using aqueous humor revealed atypical lymphocytes, suggesting intraocular ALCL involvement. Acute angle closure developed in the anterior chamber due to rapid progression of ALCL, causing pupillary block. Laser and surgical interventions were attempted but failed to relieve the pupillary block. Finally, radiation therapy resolved the pupillary block to restore the anterior chamber and normalize intraocular pressure. This is the first case in the English literature of ALCL involving the iris to cause acute secondary angle closure.

Anaplastic Large Cell Lymphoma Involving Anterior Segment of the Eye

A 36-year-old woman was diagnosed with anaplastic large cell lymphoma (ALCL) by excisional biopsy of a left frontal skin lesion. During the first cycle of chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone), the patient complained of right ocular pain and inflammation. Cytologic examination using aqueous humor revealed atypical lymphocytes, suggesting intraocular ALCL involvement. Acute angle closure developed in the anterior chamber due to rapid progression of ALCL, causing pupillary block. Laser and surgical interventions were attempted but failed to relieve the pupillary block. Finally, radiation therapy resolved the pupillary block to restore the anterior chamber and normalize intraocular pressure. This is the first case in the English literature of ALCL involving the iris to cause acute secondary angle closure.

Primary Central Nervous System ALK Positive Anaplastic Large Cell Lymphoma with Predominantly Leptomeningeal Involvement in an Adult

A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intracerebral lesions. Diagnosis was made based on a brain biopsy showing anaplastic large cell lymphoma (ALCL), immunohistochemical stains revealing positivity for anaplastic lymphoma kinase (ALK) and an absence of involvement in any other organs; specifically, the primary central nervous system ALK+ALCL. Complete remission was achieved following 5 cycles of systemic chemotherapy with a high dose of Methotrexate and a simultaneous 7 cycles of intrathecal triple chemotherapy. Diagnosis of primary leptomeningeal ALK+ALCL is challenging given its rarity and non-specific symptoms along with non-pathognomonic radiologic findings. We present the first case of primary leptomeningeal ALK-positive ALCL where the clinical course, pathologic characteristics and treatment modality are described as well as a review of literature.

Leukemic phase of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) is a distinct type of CD30+ T/null-cell non-Hodgkin's lymphoma that frequently involves nodal and extranodal sites. The presence of leukemic phase in ALCL is extremely rare and occurs exclusively with ALK1-positive ALCL. We describe two patients with ALK1-positive ALCL who developed a leukemic phase with rapid progression of the disease. Immunophenotypic pattern assessed on peripheral blood by flow cytometry revealed CD45, CD30, and CD25 positivity in both cases but NPM-ALK1 was expressed in only one case. Both patients developed leukemic phase as a terminal event of the disease and we share the immunophenotypic features of both cases.

Ataxia telangiectasia: Family management.

Ataxia telangiectasia (AT) is a rare autosomal recessive disease resulting in progressive degeneration of multiple systems in the body. Both A-T homozygote and heterozygote are at increased risk of developing malignancy. We report a family in which three generations were affected by this disorder. Our index case is a 12-year-old female child, born of second degree consanguineous marriage diagnosed to have ataxia telangiectasia at the age of four years, now presented with fever and neck swelling of one month duration. Family history suggestive of ataxia telangiectasia in maternal uncle and younger sibling was present. History of premature coronary artery disease and death in paternal grandfather was present. On evaluation, child was diagnosed to have Alk negative anaplastic large T cell lymphoma. Management included genetic counseling, examination of all the family members, identification of A-T homozygote and providing appropriate care, regular surveillance of the heterozygote for malignancy.

[Evaluation of immunoreactivity of cellular markers of CD15 and CD30 among patients with classic Hodgkin's lymphoma]

Hodgkin's lymphoma [HL] is a curable lymphoid malignancy. Different Immunohistochemical markers specially CD15 and CD30 are used to diagnose HL and differentiate it from other lymphomas including anaplastic large cell lymphoma [ALCL]; this study was carried out in patients with Hodgkin's lymphoma to determine the frequency of these markers in Iranian patients with HL. A cross sectional study was done out on all patients with definite diagnosis of classic Hodgkin's lymphoma in the selected hospitals. Patients were classified according to WHO classification of the HL type into: mixed cellularity, nodular sclerosis, lymphocyte rich and lymphocyte depletion subtypes. CD15 and CD30 immunophenotype were detected by monoclonal immunostaining method. The frequency was determined in each group and C.I. [confidence interval] was calculated for the Iranian population. The studies were done on 65 patients. Mean age was 31.9 +/- 18.1 years. 37 [56.9%] were male. CD15 and CD30 was positive in 50 [76.9%] and 58 [89.2%] respectively. Both markers were positive in 46 [70.8%] and were negative in 3 [4.6%]. There was no significant relationship between CD15 and CD30 positivity and age/ gender [p<0.6]. It seems that there is a high frequency of positivity for CD15 or CD30 in Iranian patients with Hodgkin's lymphoma. These markers are useful in diagnosis of Hodgkin's lymphoma. However, in cases who are CD15 negative and CD30 positive, it is better to use additional markers for avoiding misdiagnosis as anaplastic large cell lymphoma

Anaplastic large-cell lymphoma with florid granulomatous reaction: a case report and review of literature.

Granulomatous reactions have been reported in association with lymphomas, more often with Hodgkins disease than with Non-Hodgkins Lymphoma. Not many reports are available on the association of anaplastic large-cell lymphoma with sarcoid-type granuloma. Herein, we report a case of an elderly female with generalized lymphadenopathy who had a florid granulomatous reaction almost masking the lymphoma cells in the lymph node biopsy. A detailed clinical history, careful histological examination and immunohistochemistry helped in attaining the correct diagnosis.

Anaplastic large cell lymphoma (ALCL) presenting as primary bone and soft tissue sarcoma--a study of 12 cases.

This study highlights the rare presentation of anaplastic large cell lymphoma as primary bone and soft tissue tumour. Twelve cases were studied. Clinical impression was non Hodgkin's lymphoma in 4 cases, sarcoma in 6 (osteosarcoma-2, Ewing's/primitive neuroectodermal tumour-1, and sarcoma NOS-3), and tuberculosis of thoracic spine in 1 and the last case involving the rib had a differential diagnosis of tuberculosis and NHL. Histology revealed round cells with eosinophilic cytoplasm and pleomorphic nuclei. Immunohistochemically all tumours were CD30 positive and 8 of 9 cases (88.9%) showed ALK-1 positivity. The pleomorphic cytomorphology ofALCL leads to confusion with the more frequent bone and soft tissue sarcomas affecting the musculoskeletal system. A high index of suspicion is necessary to initiate the correct panel of immunohistochemical markers to first confirm the lymphomatous nature of this tumour and to subsequently subclassify. This alone will lead to an accurate recognition of ALCL and the appropriate chemotherapy.

Linfomas cutáneos de células T, CD30 (+) / Cutaneous T-cells lymphomas, CD30 (+)

El aspecto de desórdenes linfoproliferativos cutáneos primitivos CD 30 (ki-1) positivos está constituido por la papulosis linfomatoide, el linfoma de células grandes anaplásico y los linfomas inmunoblásticos y pleomórfico de células medianas y grandes. Durante el período enero de 1995 a septiembre de 1998, nuestro Servicio evaluó 7 casos, representados por 3 casos de papulosis linfomatoide (una de las cuales luego de remitir evolucionó a un linfoma de alto grado), 4 casos de linfomas de células grandes anaplásicas, uno de ellos se desarrolló sobre una micosis fungoidea previa. Los estudios de inmunohistoquímica confirmaron CD 30 (+) en todos los casos, 6 presentaron inmunofenotipo T CD 4(+) y 1 tipo null cel. Se observaron remisiones espontáneas parciales en varios casos y recidivas en la zona con aspecto clínico, similar a la lesión previa. Fueron sensibles a la radioterapia y no recidivaron luego de cirugía. Nuestra casuística confirma el buen pronóstico que la CD 30 positividad otorga a estos linfomas cutáneos, a diferencia de la localización cutánea secundaria de un linfoma de origen nodal que tiene mal prónostico

Cutaneous T-cell lymphomas in Oman

Seven cases of cutaneous T-cell lymphomas studied during the period 1990-1995 in Oman are described. There were four cases of mycosis fungoides, and three cases of nonmycosis T-cell lymphomas. The clinical presentations were varied, and often needed multiple biopsies and immunostudies. Recent diagnostic tools of cutaneous T-cell lymphomas are of great help. For proper management, proper clinical follow-up and clinicopathological correlation are necessary

Linfoma maligno a grandes celulas anaplasicas (Ki 1+): tipos morfologicos y sus diagnosticos diferenciales / Anaplastic large cell malignant lymphoma (KI 1+): morphologic types and their differential

Presentamos los tres primeros casos nacionales de linfoma de grandes celulas anaplasticas (Ki 1+), cubriendo las tres variedades morfologicas de este tipo de linfoma descritas en la literatura internacional. Su correcto diagnostico estuvo basado en la deteccion IHQ de BER-H2, obviando las dificultades del diagnostico diferencial basado en hechos exclusivamente morfologicos. A pesar de la frecuencia relativamente baja de este tipo de linfoma, su tipificacion exacta resulta de suma importancia ya que existen pacientes con una evolucion mucho mas favorable que lo que habitualmente tienen los linfomas de alto grado