Gastrointestinal ALK-positive anaplastic large cell lymphoma: a clinicopathological analysis of five cases / 中华病理学杂志

Objective: To investigate the clinicpathological characteristics of ALK-positive anaplastic large cell lymphoma (ALCL) of the gastrointestinal tract, and to discuss its diagnosis and differential diagnosis. Methods: Five cases of gastrointestinal ALK-positive ALCL diagnosed and treated in Xijing Hospital of the Fourth Military Medical University, between 2011 and 2019 were collected. There were three male and two female patients, aged 5-42 years (mean 25 years). These patients clinically presented with fever and night sweats, weight loss, abdominal pain, abdominal mass, ulcers, bleeding, or intestinal obstruction, and underwent surgical resection of the tumors or endoscopic biopsy. The clinical manifestations, auxiliary examinations, histopathological characteristics, immunophenotypes and genetic alterations were analyzed. Results: In this cohort, one case was common type, two cases were monomorphic variant of common type, and two cases were small cell variant. The tumor cells in all cases expressed ALK, CD30, and one or more T lymphocyte markers, while all the markers of B lymphocyte and plasmacyte were negative. Clonality analysis showed that two cases had clonal T cell receptor (TCR) and immunoglobulin (Ig) gene rearrangement, one case had no clonal TCR but Ig gene rearrangement, and one case had no clonal TCR and Ig gene rearrangements. During the 4 to 67 months' follow-up, two patients died of the disease, two were alive with free of disease and one had a relapse. Conclusions: ALK-positive ALCL of the gastrointestinal tract is extremely rare, and has poor prognosis. Lymphoma originating from this site with CD30 and ALK-positive phenotypes may be considered to be ALCL; however differentiation from other tumors that had anaplastic features, expressed CD30 and or ALK, in particular, ALK positive large B-cell lymphoma is necessary.

Brentuximab vedotin in ALK-negative anaplastic large cell lymphoma presenting with alar mass: A case report

@#Anaplastic large cell lymphoma (ALCL) is a rare subtype of T-cell non-Hodgkin lymphoma (NHL) primarily involving the lymph nodes; however, extra-nodal manifestations are also common. Diagnosis can be confirmed by a combination of histopathology and immunohistochemical staining. Complete workup and staging include imaging and bone marrow examination. This presents a case of a 55-year-old male with anaplastic lymphoma kinase (ALK) - negative ALCL presenting with an alar mass. ALCL patients often present with rapidly progressing lymphadenopathy. Extra-nodal manifestations commonly involve the skin, liver, lung, and gastrointestinal tract. Biopsy of the mass showed small to medium-sized anaplastic lymphoid cells that stained positive for CD30, LCA (CD45), CD99, and negative for CD20, ALK (CD246), neuron-specific enolase, CD34, CD5, PAX5, TdT, MPO, CD138, EMA, pancytokeratin, CD3 and synaptophysin. These findings were most compatible with an ALK-negative ALCL. The patient was started on a combination of brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone (BV + CHP) every 21 days for 6 cycles. There was a progressive decrease in the size of the mass, and a resolution was noted after the 5th cycle. FDG-PET/CT scan was done after the 6th cycle of chemotherapy and 6 months after completion of treatment. Both scans showed no evidence of metabolically active nodal or extra-nodal lymphomatous disease. This case showed a unique extra-nodal manifestation of an ALK-negative ALCL presenting as an alar mass with a good response to BV + CHP. However, more evidence is necessary to further establish the role of BV as the first-line treatment regimen for CD30-positive peripheral T-cell lymphoma (PTCL), including ALK-negative ALCL.

Prótesis mamarias texturizadas y linfoma anaplástico de células grandes / Breast implant associated anaplastic large cell lymphoma (BIA-ALCL)

Resumen En los últimos años, han aparecido evidencias que relacionan a un tipo de linfoma mamario con los implantes de silicona, lo que ha causado gran conmoción a nivel mundial. Este linfoma anaplástico de células grandes no Hodgkin (células T monoclonales), se ha visto asociado en mayoría de los casos, a las prótesis mamarias texturizadas. Es relativamente raro, ya que se puede presentar en 1 de cada 2.832 operados (as) y se puede manifestar como un seroma periprotésico o como una tumoración de la cápsula cicatrizal mamaria con o sin compromiso de la glándula y de los tejidos adyacentes.

In recent years, evidence has appeared linking a type of breast lymphoma with silicone implants, which has caused great commotion worldwide. This anaplastic large cell non-Hodgkin lymphoma (monoclonal T cells) has been associated in most cases with textured breast implants. It is relatively rare, since it can occur in 1 in 2832 operated on and it can manifest as a periprosthetic seroma or as a tumor of the mammary scar capsule with or without involvement of the gland and adjacent tissues.

Linfoma anaplásico de células grandes asociado a implante mamario. Reporte de un caso y revisión de la literatura. / Breast Implant Associated-Anaplastic Large Cell Lymphoma (BIA-ALCL) - Argentinian case

El linfoma anaplásico de células grandes asociado a implantes mamarios LACG-AI o BIA-ALCL, abreviatura en inglés de "Breast Implant Associated-Anaplastic Large Cell Lymphoma", es una nueva entidad reconocida por la OMS desde el 2016, de rara incidencia y que aún plantea muchos interrogantes. Desde su primera mención en 1997 (J. Keech - B. Creech) su incidencia ha ido en aumento. En julio de 2020, 953 casos en el mundo según el Registro de la Sociedad Americana de Cirujanos Plásticos (PROFILE), y las publicaciones se multiplican exponensialmente año a año demostrando el interés que suscita. Se ha descripto una fuerte asociación con las superficies texturizadas de los implantes mamarios y con el tipo de material (mayor textura "grado 4" y cubierta de poliuretano mayor riesgo) llegando a describirse tasas tan altas omo 1/2830 en Australia/Nueva Zelanda. Su presentación clínica en casi el 75% es bajo la forma de un seroma tardío y el tiempo de exposición promedio ronda entre los 7 a 11 años. El diagnóstico histo-patológico integra el examen morfológico con la caracterización molecular, visualizándose grandes célular anaplásicas CD30 (+), ALK (-). El tratamiento quirúrgico, capsulectomía bilateral en estadios tempranos es el gold standard. Su pronóstico es excelente con exérsis completas. Objetivo: actualizar la información sobre esta novel enfermedad y comentar un caso propio que presenta todas las características descriptas en la literatura, siendo el 14° registrado en Argentina

The anaplastic large cell lymphoma associated with breast implants, LACCG-AI o BIA-ALCL abbreviation in English, is an entity recognized by the WHO since 2016 of rare incidence and that still raises many questions. Since its firts mention in 1997 (J. Keech - B. Creech) its incidence has been increasing, In july 2020, 953 cases in the world according to the Registry of the America Society of Plastic Surgeons (PROFILE), and the publications multiply exponentially year after year, demonstrating the interest it arouses, A strong association has been described with the textured surfaces of breast implants and with the type of material (greater texture "grade 4" and higher risk polyurethane cover), reaching rates as high as 1/2830 in Australia / New Zealand. Its clinical presentation in almost 75% is in the form of a late seroma and the average exposure time is between 7 to 11 years. The pathological anatomical diagnosis integrates the morphological examination with the molecular characterization, visualizing large anaplastic CD30 (+), ALK (-) cells. Surgical treatment, bilateral capsulectomy in early stages, is the gold standard. Her prognosis is excellent with complete exeresis. Objetive: to update the information on this novel disease and comment on an own case that presents all the characteristics described in the literature, the 14th being registered in Argentina

ALK+ Anaplastic large cell lymphoma with extensive cardiac involvement: A rare case report and review of the literature

Cardiac lymphoma is a rare entity. In this setting, the secondary involvement of the heart is far more frequent than the primary cardiac lymphoma. Herein, we present an autopsy case of a disseminated anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma with a dominant mediastinal involvement. Extensive cardiac infiltration with the near replacement of the myocardial wall by the neoplastic cells was observed. A total of nine isolated case reports of anaplastic large cell lymphoma with cardiac involvement were found in the English-language literature, and a widespread cardiac and thymic infiltration by the systemic ALK-positive anaplastic large cell lymphoma has not been documented. An incidental regenerative nodule was also identified in the liver. The patient died of pulmonary thromboembolism and cardiac arrest.

Systemic ALK-positive anaplastic large cell lymphoma involving implant site: a fortuitous association

Anaplastic lymphoma kinase (ALK) positive, anaplastic large cell lymphoma involving the non-mammary implant is an extremely rare presentation. Irrespective of the type or site, the implant-associated primary ALCL is morphologically and immunophenotypically similar to ALK-negative ALCLs. Herein, we present the case of a 42-year-old male who developed a lytic lesion after an implant for a right femur fracture. The lytic lesion biopsy revealed anaplastic large cell lymphoma with ALK protein expression. Imaging findings showed the widespread dissemination of disease all over the body, entrapping the implant too. ALCL involving the bone implant is a very unusual and rare presentation that needs to be documented.

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.

Clinical characteristics and prognostic factors of 40 cases of primary systemic anaplastic large cell lymphoma / 中华血液学杂志

Objective: To analyze the clinical features and prognostic factors of primary systemic anaplastic large cell lymphoma (ALCL) . Methods: 40 ALCL cases treated in the First Affiliated Hospital of Zhejiang University from January 2013 to December 2018 were retrospectively analyzed. Results: ① With a median age of 41 (14-67) years, there were 29 males and 11 females, 36 patients (90.0%) had Ann Arbor stage Ⅲ-Ⅳ tumors, 23 patients (57.5%) were in high-intermediate or high international prognostic index (IPI) risk group. 25 patients (62.5%) had B symptoms, such as fever, emaciation and night sweat.38 patients (95.0%) had extranodal invasion, 25 patients (62.5%) had higher LDH level, and 25 patients (62.5%) had high expression of Ki-67 (80% or more) . With 22 ALK(+) patients (55.0%) and 18 ALK(-) patients (45.0%) , there was a significantly difference in the median age of the two groups [29 (14-67) years old vs 51.5 (19-67) years old, P=0.003]. ② All patients received chemotherapy, 18 cases were treated with CHOP (cyclophosphamide, doxorubicin, vindesine, prednisone) , 12 cases with ECHOP (cyclophosphamide, doxorubicin, vindesine, prednisone, etoposide) , 10 cases with other treatments and 26 patients (65.0%) obtained complete remission (CR) . ALK(-) (P=0.029, OR=13.458) and Ki-67 expression of 80% or more (P=0.04, OR=14.453) were independent factors of CR rate, the CR rate of ECHOP chemotherapy was higher than CHOP chemotherapy (P=0.026) . ③ LDH level, IPI score, ALK expression and chemotherapy regimen had significantly effect on progression free survival (PFS) and overall survival (OS) (P<0.05) . Conclusion: The study shows that primary systemic ALCL usually occurs in males, the average age of ALK(+) patients were younger than ALK(-) patients. Most patients are in stage Ⅲ-Ⅳ with extranodal invasion, more than half of the patients have B symptoms, elevated LDH, and high expression of Ki-67. The expression level of Ki-67, ALK expression, and chemotherapy regimen have prognostic value for CR rate, the LDH level, IPI score, ALK expression and chemotherapy regimen for PFS and OS. ECHOP is a better choice with improved prognosis.

Outcomes of 33 patients with anaplastic large cell lymphoma treated after hematopoietic stem cell transplantation / 中华血液学杂志

Objective: To explore the efficacy and prognostic factors of hematopoietic stem cell transplantation (HSCT) for the treatment of patients with anaplastic large cell lymphoma (ALCL) . Methods: The clinical records of 33 ALCL patients after HSCT were collected and analyzed retrospectively to evaluate the rates of overall survival (OS) and recurrence after autologous (auto-HSCT) and allogeneic HSCT (allo-HSCT) and the factors influencing prognosis. Results: The median-age of this cohort of 33 ALCL cases at diagnosis was 31 (12-57) years old with a male/female ratio of 23/10, 24 cases (72.7%) were ALK(+) and 9 ones (27.3%) ALK(-). Of them, 25 patients (19 ALK(+) and 6 ALK(-)) underwent auto-HSCT and 8 cases (5 ALK(+) and 3ALK(-)) allo-HSCT with a median follow-up of 18.7 (4.0-150.0) months. Disease states before HSCT were as follows: only 6 patients achieved CR status and received auto-HSCT, 16 patients achieved PR (14 cases by auto-HSCT and 2 ones allo-HSCT) , the rest 11 cases were refractory/relapse (5 cases by auto-HSCT and 6 ones allo-HSCT) . There were 7 cases died of disease progression (5 after auto-HSCT and 2 allo-HSCT) and 5 cases treatment-related mortality (TRM) (2 after auto-HSCT and 3 allo-HSCT) , TRM of two groups were 8.0% and 37.5%, respectively. Both the median progression-free survival (PFS) and OS were 15 months after auto-HSCT, the median PFS and OS after allo-HSCT were 3.7 (1.0-90.0) and 4.6 (1.0-90.0) months, respectively. There was no statistically significant difference in terms of survival curves between the two groups (OS and PFS, P=0.247 and P=0.317) . The 2-year OS rates in auto-HSCT and allo-HSCT groups were 72% and 50%, respectively. The 5-year OS rates in auto-HSCT and allo-HSCT groups were 36% and 25%, respectively. Conclusion: ALCL treated by chemotherapy produced high rates of overall and complete responses. Chemotherapy followed by auto-HSCT remained to be good choice for patients with poor prognostic factors. High-risk patients should be considered more beneficial from allo-HSCT.

Linfoma anaplásico de grandes células relacionado ao implante mamário: revisão sistemática da literatura / Breast implant-associated anaplastic large-cell lymphoma: a systematic literature review

O linfoma anaplásico de grandes células associado ao implante de mama (Breast Implant Associated Anaplastic Large Cell Lymphoma - BIA-ALCL) é uma doença maligna recentemente descoberta, rara e possivelmente associada aos implantes mamários texturizados. Essa revisão da literatura teve como objetivo trazer novas atualizações acerca da epidemiologia, fisiopatologia e fatores de risco para desenvolvimento do BIAALCL. Foi realizado o levantamento de dados do período de dezembro de 2018 a fevereiro de 2019, através das bases de dados PUBMED, LILACS e Scielo sendo selecionados 10 artigos publicados entre 2016 e 2018. Foi encontrada uma incidência variando entre 2,8:100.000 a 1:3 milhões de pacientes com implantes mamários. Os dados coletados corroboram para a teoria de que não há uma relação direta de causa e efeito entre os implantes mamários, mormente os texturizados, e o desenvolvimento do BIA-ALCL, podendo esses ser considerados somente como fatores de risco e não agentes causadores. A teoria fisiopatológica mais aceita é a de que os implantes mamários com maior área de superfície levariam a formação de maior biofilme por maior adesão bacteriana gerando inflamação crônica mais proeminente, levando ao gatilho para a transformação maligna das células T. As informações explicitadas nessa revisão devem auxiliar na ampliação de estudos acerca da doença e criação de políticas públicas para a prevenção e diagnóstico precoce de tal enfermidade. Pelos dados encontrados há necessidade de que cirurgiões plásticos realizem acompanhamento mais próximo de seus pacientes, assim como orientem os pacientes antes das cirurgias sobre a existência da doença.

Breast implant-associated anaplastic large-cell lymphoma (BIA-ALCL) is a newly discovered and rare cancer possibly associated with textured breast implants. This literature review investigates its epidemiology, pathophysiology, and risk factors. PubMed, LILACS, and SciELO databases were searched from December 2018 to February 2019, and 10 articles published between 2016 and 2018 were selected. The incidence of BIA-ALCL ranged from 2.8:100,000 to 1:3 million breast implants. The obtained data corroborate the hypothesis that there is no direct cause and effect relationship between breast implants, especially textured implants, and BIA-ALCL, and these implants can be considered risk factors but not causative factors. The most accepted hypothesis on disease pathophysiology is that breast implants with larger surface areas may promote bacterial adhesion and biofilm formation, leading to severe chronic inflammation, triggering the malignant transformation of T cells. This review provides knowledge on BIA-ALCL and helps develop and implement public policies for disease prevention and timely diagnosis. The data highlight that long-term follow up is necessary and that surgeons should advise patients of the potential risk of developing BIA-ALCL before performing the implant surgery.

Primary multifocal anaplastic large cell lymphoma with eyelid involvement: a case report / Linfoma anaplásico de grandes células primário multifocal com envolvimento de pálpebra: relato de caso

ABSTRACT Ocular adnexal involvement in CD30+ lymphoproliferative disorders is rare. We report the case of a 73-year-old woman with a relapsing primary cutaneous anaplastic large cell lymphoma on her eyelid. A systemic extension study excluded extracutaneous involvement. Systemic chemotherapy resulted in an optimal response, with complete regression of the cutaneous lesions. There has been no recurrence during the 2 years of follow-up.

RESUMO O acometimento ocular adicional nos distúrbios linfoproliferativos CD30+ é raro. Relatamos o caso de uma mulher de 73 anos com linfoma de grandes células anaplásicas primárias recidivantes em sua pálpebra. A avaliação sistêmica excluiu envolvimento extracutâneo. A quimioterapia sistémica resultou em uma resposta ótima, com regressão completa das lesões cutáneas. Não houve recidiva durante 2 anos de acompanhamento.

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.

Primary cutaneous anaplastic large cell lymphoma in a 73-year-old Filipino male

Introduction@#Primary cutaneous anaplastic large cell lymphoma (PCALCL) is an uncommonly encountered subtype of cutaneous lymphoma under the classification of CD30-positive lymphoproliferative disorders which presents histologically as large atypical lymphocytes with pleomorphic and anaplastic cytology that localizes to the dermis. Although recurrent, PCALCL usually carries a good prognosis, with 5-year survival rates ranging from 85% to 95%.@*Case Summary@#We report a 73-year-old elderly male who consulted at our out-patient department with a 3-year and 6-month history of multifocal, gradually enlarging, erythematous nodules with dry, necrotic areas on the scalp, right auricular area, left axillary area, right forearm, and right thigh, accompanied by loss of appetite and nontender cervical, left axillary, and right inguinal lymphadenopathy. Previous skin punch biopsy and immunohistochemical stain done by the patient’s preceding dermatologist was signed out as “suggestive” of pseudolymphoma. However, management with intralesional corticosteroid injections provided no improvement. Skin punch biopsy done at our institution revealed ALK negative (-) anaplastic large cell lymphoma. Patient was then referred to an oncologist, however, the patient was lost to follow-up and succumbed to community acquired pneumonia.@*Conclusion@#This case highlights the importance of a thorough diagnostic assessment as recent studies indicate a poorer prognosis of ALK (-) cases, with overall 5-year survival rates consistently below 50%.

ALK-positive anaplastic large cell lymphoma with a monomorphic small-cell pattern masquerading as inflammatory gastric lesions

@#Introduction: Anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL) with a non-common pattern can be diagnostic challenging. Pathologists can be unavoidably and unintentionally blind to non-descript tumor cells in a lymphohistiocytic- (LH) or small-cell (SC)pattern. We report a case of primary systemic ALK+ ALCL with a SC pattern that presented as secondary gastric lesions with a mixed LH and SC pattern that was masqueraded as inflammatory lesions. Case Report: A 34-year-old woman with intractable epigastric pain was referred to have repeated endoscopy with biopsy. She was found to multiple gastric erosions and nodules that were diagnosed as inflammatory lesions both endoscopically and histologically. Meanwhile, she developed an acute onset of severe back pain associated with a pathologic compression fracture in the T3 thoracic vertebral body. Imaging studies disclosed a disseminated systemic disease involving abdominopelvic lymph nodes and cervical and thoracic vertebral bodies. The needle biopsy of the pelvic lymph node disclosed diffuse proliferation of monomorphic small round cells that were diffusely positive for CD30 and ALK. A diagnosis of ALK+ ALCL with a monomorphic SC pattern was rendered. Discussion: A retrospective review of the gastric biopsies with the aid of immunohistochemistry enabled us to recognise the presence of lymphomatous infiltrates with a mixed LH and SC pattern in every piece of gastric biopsies that were repeatedly misdiagnosed as inflammatory lesions. This case illustrates a significant diagnostic pitfall of the LH- and SC-patterns in ALK+ ALCL, in which the tumour cells featuring lymphoid, plasmacytoid or histiocytoid appearance can be masqueraded as inflammatory cells.

Clinical Features and Prognostic Factors of 18 Children with Anaplastic Large Cell Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical features and to explore the therapeutic efficacy and prognostic factors of children with anaplastic large cell lymphoma (ALCL).@*METHODS@#The clinical data of 18 children with ALCL admitted in Department of Pediatric Hematology, Union Hospital of Fujian Medical University from April 2011 to November 2017 was collected and analyzed.@*RESULTS@#The male to female ratio was 2∶1, the median age of onset was 6 (0.9-11.3) years old, and the B symptom was positive in 13 cases. The most common initial symptom was lymphadenopathy (in 17 cases). All patients were manifested with multiple organ involvements. 4 cases were classified as clinical stage Ⅱ, 11 cases as stage Ⅲ, and 3 cases as stage Ⅳ. Laboratory tests revealed 9 cases with leukocytosis and 8 cases with CRP＞20 mg/L. The pathological results showed all ALK-positive anaplastic large cell lymphoma with Ki-67 rate between 40%-90%. The median follow-up time was 41 months. 2 patients died before treatment, 1 patient was lost to follow-up. 15 patients accepted chemotherapy protocol of CCCG-BNHL-2011. 2 patients relapsed early, the 3 year event-free survival rate was (76.7±10.2)%. Kaplan-Meier survival analysis showed leukocytosis, increased CRP level, bone involvement and clinical stage were factors affecting prognosis.@*CONCLUSION@#ALCL is a relatively rare subtype of childhood non-Hodgkin's lymphoma with high invasiveness. Leukocytosis, increased CRP level, bone involvement and clinical stage are poor factors affecting the prognosis of patients.

Anaplastic Large Cell Lymphoma of the Duodenum in a Teenage Girl: Misdiagnosed as an Intramural Duodenal Hematoma / 대한소아소화기영양학회지

We report a case of a 13-year-old girl who presented with a 2-month history of intermittent abdominal pain. Laboratory examination showed hepatitis and pancreatitis. Because of persistent vomiting, computed tomography (CT) was performed, which revealed a circumferential soft tissue density in the duodenal wall, causing partial obstruction. Supportive therapy failed. Repeat CT showed no significant change from the initial study. The patient underwent upper endoscopy, which revealed a mass in the second portion of the duodenum, which occluded most parts of the lumen. The histopathological finding was consistent with an anaplastic large cell lymphoma, a rare form of small bowel neoplasm. After the third course of chemotherapy, complete resolution of the mass was noted, and her symptoms were relieved.

A Case of Multifocal Primary Cutaneous Anaplastic Large Cell Lymphoma Managed without Surgical Treatment / 대한두경부종양학회지

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.

Hallazgo incidental de microfilarias de Mansonella ozzardi en un paciente pediátrico con linfoma T anaplásico / Incidental finding of Mansonella ozzardi in a paediatric patient with anaplastic lymphoma

Resumen Las filariasis son parasitosis producidas por nemátodos hemáticos de la familia Filariidae, la Mansonella ozzardi, es uno de los agentes etiológicos distribuido ampliamente en el continente americano y en el Caribe. Presentamos el caso de una paciente de 13 años de edad, previamente diagnosticada con linfoma T de célula grande anaplásico. Como parte de la evaluación antes del segundo ciclo B de quimioterapia, se realizó un extendido de sangre periférica en el que se encontró una microfilaría tipificada como Mansonella ozzardi, se dio manejo con una dosis única de ivermectina y se logró resultado negativo en el control a las 24 horas. Actualmente la paciente se encuentra asintomática y sin evidencia de recurrencia de la parasitemia y terminando su tratamiento oncológico.

Abstract Filariasis is caused by nematodes in the blood. Mansonella ozzardi is one of the aetiological agents widely distributed in the Americas and the Caribbean. The case is presented on a paediatric patient previously diagnosed with T-cell anaplastic large cell lymphoma. As part of the evaluation before the second cycle B chemotherapy, a peripheral blood smear was performed, in which were found microfilaria, identified as Mansonella ozzardi. The treatment was a single dose of ivermectin, with a negative result being obtained at 24 hours. The patient is currently asymptomatic and with no evidence of recurrence of the parasitaemia, and able to finish the cancer treatment.