Explante de prótese mamária de silicone: reconstrução da mama pela técnica dos retalhos cruzados / Silicone breast prosthesis explant: breast reconstruction using the crossed flap technique

Introdução: Em 1963 Cronin e Gerow introduziram o uso do implante de silicone e seu uso aumentou exponencialmente. Contudo, complicações relacionadas aos implantes surgiram ao longo do tempo. O conjunto de situações adversas ao uso dos implantes de silicone, alimentado pelo crescimento das mídias sociais, culminou em um aumento da retirada definitiva do implante. Muitos casos de explante têm o pedículo inferior comprometido pela lesão dos vasos perfurantes e a técnica dos retalhos cruzados é uma alternativa para a reconstrução das mamas explantadas. Métodos: Foram realizados explantes de silicone com reconstrução imediata da mama sem o uso de um novo implante, motivados por indicação médica ou por desejo próprio do paciente. A técnica dos retalhos cruzados foi utilizada em todos os casos. Ela se vale do cruzamento de retalhos parenquimatosos de pedículo superior, um medial e outro lateral, conforme descrito por Sperli. Resultados: Foram operados 10 casos de 2004 a 2021. O tempo de uso das próteses variou de 3 a 19 anos e a principal motivação para o explante foi contratura capsular. Nenhum caso de necrose foi observado. Conclusões: A técnica dos retalhos cruzados é uma alternativa útil e segura para as cirurgias de reconstrução da mama após explante definitivo.

Introduction: In 1963 Cronin and Gerow introduced the use of the silicone implant and its use increased exponentially. However, complications related to implants emerged over time. The set of adverse situations to the use of silicone implants fueled by the growth of social media culminated in an increase in the permanent removal of the implant. Many cases of explants have the inferior pedicle compromised by injury to the perforating vessels, and the crossed flap technique is an alternative for the reconstruction of explanted breasts. Methods: Silicone explants were performed with immediate breast reconstruction without the use of a new implant, motivated by medical indication or the patients own desire. The crossed flap technique was used in all cases. It uses the crossing of parenchymal patches of the superior pedicle, one medial and one lateral, as described by Sperli. Results: 10 cases were operated from 2004 to 2021. The time of use of the prostheses ranged from 3 to 19 years and the main motivation for the explant was capsular contracture. No cases of necrosis were observed. Conclusions: The crossed flap technique is a useful and safe alternative for breast reconstruction surgeries after definitive explantation.

Clinical characteristics and prognosis analysis of patients with primary systemic anaplastic large cell lymphoma / 白血病·淋巴瘤

Objective:To explore the clinical charateristics and prognostic factors of patients with primary systemic anaplastic large cell lymphoma (ALCL).Methods:The clinicopathological data of 31 patients with newly treated primary systemic ALCL in Liaoning Cancer Hospital from January 2010 to December 2020 were retrospectively analyzed. Kaplan-Meier method was used to make survival analysis and log-rank test was performed. Multivariate analysis of factors influencing overall survival (OS) was performed by using Cox proportional hazards model.Results:Among 31 patients, there were 19 males and 12 females, with a median age of 47 years old, ranging from 16 to 74 years old. There were 18 (58.1%) patients with B symptoms, 16 (51.6%) patients with increased platelet count, 22 (71.0%) patients with Ann Arbor stage Ⅲ-Ⅳ. Until the end of follow-up, 22 cases survived and 9 cases died; the 5-year OS rate was 70.9%, and the median OS time was not reached. The 5-year OS rate of patients receiving CHOPE chemotherapy regimen was higher than that of patients receiving CHOP and other chemotherapy regimens, and the difference was statistically significant ( P = 0.049). There were statistically significant differences in the 5-year OS rates of patients with different platelet count, international prognostic index (IPI) score, chemotherapy regimens and with or without B symptoms (all P < 0.05). Multivariate analysis indicated that IPI score was an independent factor affecting OS ( HR = 2.194, 95% CI 1.078-4.465, P = 0.030). Conclusions:Most primary systemic ALCL patients are diagnosed at advanced stage and it is more common in males. Most patients have B symptoms and high platelet count. IPI score is an important prognostic factor, and CHOPE regimen may be a good choice of the first-line chemotherapy.

Effects of autophagy-mediated crizotinib resistance on cancer stem-like cell subsets in anaplastic lymphoma kinase-positive anaplastic large cell lymphoma and its significance / 白血病·淋巴瘤

Objective:To investigate the effects of autophagy-mediated crizotinib resistance on cancer stem-like cell subsets in anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALK + ALCL). Methods:The preliminary research of our group divided ALK + ALCL Karpas299 cell line into two subgroups: reporter unresponsive (RU) and reporter responsive (RR) cells through the implantation of Sox2 reporter genes, among which the RR cells had the characteristics of stem cells. Fluorescent labeled LC3 overexpressing RR and RU cells (RR-LC3 and RU-LC3) were constructed by lentiviral transfection technique, and the transfection efficiency was verified by using Western blotting and flow cytometry. RU-LC3 and RR-LC3 were treated with crizotinib at different concentrations (0, 250, 500, 1 000 nmol/L). The RED and GEN signals were detected by using double-signal flow cytometry to observe autophagy flux (RED represents the red signal B695 of the next generation of far-red fluorescent protein TagFP635 mKate; GEN represents the green signal from pH-sensitive GFP variant pHluorin B530), and the RED to GEV ratio represents autophagy flux. Real-time quantitative polymerase chain reaction (qRT-PCR) was used to detect autophagy related genes ULK1, WIPI1 and LC3B mRNA expression levels in cells. The effects of different concentrations of crizotinib (250, 500, 1 000 nmol/L) combined with chloroquine (5, 10 μmol/L) on the cell survival were detected by using MTS assay. Results:RU-LC3 and RR-LC3 cells with overexpression of LC3 were successfully constructed. After induction of 250, 500 and 1 000 nmol/L crizotinib, the RED to GEN ratio in RU-LC3 cells was 1.135±0.017, 1.453±0.017 and 1.755±0.021, respectively; the RED to GEN ratio in RR-LC3 cells was 1.193±0.018, 2.116±0.013 and 3.307±0.189, respectively; the RED to GEN ratio in RU-LC3 cells and RR-LC3 cells showed a dose-dependent manner. The RED to GEN ratio in RR-LC3 cells was higher than that in RU-LC3 cells when treated with same concentrations of crizotinib, and the differences were statistically significant (all P < 0.01). The autophagy flux of RR-LC3 cells was larger than that of RU-LC3 cells. When treated without crizotinib, mRNA relative expression levels of ULK1, WIPI1 and LC3B in RR cells were higher than those in RU cells (1.69±0.05 vs.1.01±0.02, t = -1.62, P < 0.01; 1.24±0.04 vs. 1.03±0.05, t = -2.11, P < 0.01; 1.70±0.22 vs. 1.02±0.05, t = -1.74, P = 0.033). In the absence of chloroquine, the half-inhibitory concentration ( IC50) of crizotinib in RR cells was higher than that of RU cells (950 nmol/L vs. 709 nmol/L). After treated with chloroquine, IC50 of RU cells did not change, while IC50 of RR cells was decreased with the increase of chloroquine concentration. Conclusions:Compared with RU cells, autophagy reaction of cancer stem-like RR cells is more rapid and intense, which is considered to be one of the important reasons for their resistance to crizotinib.

Primary central nervous system anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in children: report of one case and review of literature / 白血病·淋巴瘤

Objective:To investigate the clinical features and prognosis of primary central nervous system (CNS) anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) in children.Methods:The clinical data of a child with primary CNS ALK-positive ALCL in Fujian Medical University Union Hospital were retrospectively analyzed, and the relevant literature was reviewed.Results:The child went to other hospitals with headache and fever as the main symptoms. Head magnetic resonance imaging showed a right cerebellar mass, and there was no evidence of lymphoma infiltration outside the CNS before surgery. Later, cerebellar tumor resection was performed. After the surgery, through pathological examination, the child was diagnosed as ALK-positive ALCL, but did not receive chemotherapy in time. The child transferred to Fujian Medical University Union Hospital on the 27th day after surgery, and the tumor had spread to bone marrow, testis, vertebrae, etc., and the peripheral blood NPM-ALK fusion gene was positive. The child received 2 courses of chemotherapy and achieved complete remission, but eventually died of chemotherapy complications.Conclusions:Primary CNS ALK-positive ALCL is rare and easy to be misdiagnosed. The disease progresses quickly, and the overall prognosis is poor. Timely biopsy for diagnosis and early comprehensive treatment based on chemotherapy may improve the prognosis of patients.

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.

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.

Anaplastic lymphoma mimicking breast carcinoma / Linfoma anaplásico mimetizando carcinoma mamário

This case describes an uncommon presentation of ALK-negative anaplastic large T-cell lymphoma with breast infiltration, mimicking triple-negative carcinoma. The incidence of ALK-negative anaplastic large T-cell lymphoma usually occurs in adults in their fifth and sixth decade of life and can affect lymph nodes and extranodal sites, including skin, soft tissue, and gastrointestinal tract. The non-Hodgkin's lymphoma of the breast is uncommon, accounting for 0.04 to 0.05% of all malignant breast tumors. Diagnosis of ALK-negative anaplastic large T-cell lymphoma is challenging both to physicians and pathologists. Based on the complete medical history, clinical and imaging exams and histopathological evaluation of the lesion site biopsy, it is possible to establish an adequate diagnosis. The case describes a woman aged 37 years with palpable nodules in the left breast as well as erythematous lesions on the right leg. The analysis of the breast nodules biopsy shows that they mimic triple-negative carcinoma. However, only with immunohistochemical examination was it possible to verify the expression of the CD30 antigen, and only after a complete systemic evaluation, the diagnosis of ALK-negative anaplastic large T-cell lymphoma was performed. Misdiagnosis can lead to inadequate therapy and result in disease progression or unnecessary damages to the patient.

Este caso descreve uma incomum apresentação de linfoma anaplásico de grandes células T ALK negativo com infiltrado mamário, mimetizando carcinoma triplo negativo. A incidência do linfoma anaplásico de grandes células T ALK negativo, ocorre comumente em adultos na quinta e sexta década de vida e pode acometer linfonodos e locais extranodais, incluindo pele, tecido mole e trato gastrointestinal. O linfoma não-Hodgkin da mama é incomum, compondo 0,04 a 0,05% de todos os tumores de mama malignos. O diagnóstico de linfoma anaplásico de grandes células T ALK negativo é desafiador tanto para clínicos como para patologistas. O estabelecimento de um diagnóstico adequado é possível com base em histórico médico completo, exames clínicos e de imagem e avaliação histopatológica da biópsia do local da lesão. O caso relata uma mulher de 37 anos com nódulos palpáveis na mama esquerda em conjunto com lesões eritematosas na perna direita. Ao se analisar a biópsia dos nódulos da mama, esses mimetizavam carcinoma triplo negativo, no entanto, somente com exame imunohistoquímico foi possível verificar a expressão do antígeno CD30, e, apenas após uma avaliação sistêmica completa, foi realizado o diagnóstico de linfoma anaplásico de grandes células T ALK negativo. O diagnóstico equivocado pode acarretar terapia inadequada e resultar em progressão da doença ou em danos desnecessários ao paciente.

Detection of chromosomal translocations of DUSP22 and TP63 in ALK-negative anaplastic large cell lymphoma by fluorescence in situ hybridization and related clinical relevance / 中华病理学杂志

Objective@#To correlate chromosomal translocations of DUSP22 or TP63 with clinical significance in ALK-negative anaplastic large cell lymphoma (ALK-ALCL).@*Methods@#Thirty-two patients with ALK-ALCL were selected from January 2004 to January 2014 at Fujian Provincial Hospital for the detection of chromosomal translocations of DUSP22 and TP63 by fluorescence in situ hybridization (FISH). The relationship between DUSP22 and TP63 chromosomal translocations and the clinicopathological parameters of ALK-ALCL was analyzed.@*Results@#Among the 32 ALK-ALCL patients, 7(21.8%) had DUSP22 gene rearrangement (DUSP22+ALK-ALCL). Three patients (9.4%) had TP63 gene rearrangement (TP63+ ALK-ALCL). There were 22 patients (68.8%) without rearrangement of either DUSP22 or TP63 (DUSP22-TP63-ALK-ALCL). The patients with DUSP22+ ALK-ALCL were among the younger, and the patients with TP63+ ALK-ALCL were among the elder. The mean age of patients with DUSP22-TP63-ALK- ALCL was between those of DUSP22+ALK-ALCL and TP63+ ALK-ALCL (P<0.05). Based on Ann Arbor staging, incidence of DUSP22 gene rearrangement decreased as the clinical stage of ALK-ALCL increased (P<0.05). Incidence of TP63 gene rearrangement cases increases in patients at more advanced clinical stage(P<0.05). The five-year survival rate and prognosis of patients with DUSP22+ALK-ALCL were the highest. Patients with TP63+ ALK-ALCL had the lower five-year survival and the worse prognosis (P<0.05).@*Conclusion@#Presences of DUSP22 and TP63 chromosomal translocations correlate with the clinical stages and prognosis of ALK-ALCL and may be used for the differential diagnosis, determination of tumor aggressiveness and prognostication of ALK-ALCL.

Expressions and clinical significance of GAS1, IL-1RAP and PRF1 in patients with ALK positive anaplastic large cell lymphoma / 中华血液学杂志

Objective@#To investigate the expressions of growth arrest-specific protein (GAS1), IL-1 receptor accessory protein (IL-1RAP) and perforin (PRF1) in patients with anaplastic lymphoma kinase positive, anaplastic large cell lymphoma (ALK+ ALCL) and their relationships with clinical significances and the prognoses of ALK+ ALCL.@*Methods@#Twenty-six formalin-fixed paraffin-embedded (FFPE) samples of ALK+ ALCL patients who were diagnosed from January 2011 to September 2016 were collected. Twelve FFPE samples of patients with ALK+ALCL, 13 FFPE samples of patients with peripheral T cell lymphoma (not otherwise specified) (PTCL-NOS) and 8 FFPE samples of patients with angioimmunoblastic T-cell lymphoma (AITL) were used as control groups. RQ-PCR and immunohisto-chemical staining were used to detect the mRNA and protein expressions of GAS1, IL-1RAP and PRF1. The clinical data were analyzed.@*Results@#①The expression levels of GAS1, IL-1RAP and PRF1 gene and protein in ALK+ ALCL group were higher than those of the control groups (P<0.05), but the expression levels had no statistically significant differences between the control groups (P>0.05). ②Patients with elevated lactate dehydrogenase (LDH) (0.77 vs 1.38, z=-3.292, P=0.001) or International prognostic index (IPI)≥3(0.62 vs 1.29, z=-2.495, P=0.013) had lower expression level of GAS1. Patients with stage Ⅲ/Ⅳ disease (0.89 vs 1.18, z=-2.212, P=0.027) or IPI≥3 (0.48 vs 1.13, z=-2.008, P=0.045) had lower expression level of PRF1. IL-1RAP expression level was not associated with clinical features. ③ALK+ ALCL patients in complete remission (CR) group had higher expression levels of GAS1 and PRF1 than patients in non-remission (NR) group (P values were 0.016 and 0.009). ④Kaplan-Meier survival analysis showed that patients with high expression levels of GAS1 and PRF1 had longer median overall survival and progression-free survival than patients with low expression levels of GAS1 and PRF1.@*Conclusion@#GAS1, IL-1RAP and PRF1 could be molecular markers for ALK+ ALCL patients. They have potential diagnostic value and can be used for differential diagnosis in some difficult cases. ALK+ ALCL patients with high expression levels of GAS1 or PRF1 have better curative effects and prognoses.

Primary systemic anaplastic lymphoma kinase negative anaplastic large cell lymphoma: clinical analyses of 32 cases / 白血病·淋巴瘤

Objective To investigate the clinical manifestations, treatment outcomes and prognosis factors of the patients with primary systemic anaplastic lymphoma kinase negative anaplastic large cell lymphoma (ALK-ALCL). Methods The clinical data of 32 patients histologically confirmed with ALK-ALCL from January 2011 to December 2017 in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed. Kaplan-Meier method, Log-rank test and Cox proportional hazards model were used for the survival analysis. Results There were 19 males and 13 females in 32 patients, and the median age of onset was 50 years old (14-78 years old). The proportion of the patients with B symptom, extranodal involved, stageⅢ-Ⅳ, International Prognostic Index (IPI) sore 2-3 were 34.4 % (11 cases), 53.1 % (17 cases), 59.4 % (19 cases) and 50.0 % (16 cases) respectively. Chemotherapy alone and chemotherapy plus radiotherapy were the main methods, and a few patients received surgery alone, surgery plus chemotherapy, chemotherapy plus autologous stem cell transplantation or palliative care. The efficacy of 28 cases could be evaluated. The objective effective rate was 85.7 % (24/28), the complete remission (CR) rate was 42.9 % (12/28), and the partial remission (PR) rate was 42.9 % (12/28). The median progression-free survival (PFS) time was 28 months (95 % CI 13-42 months) and the median overall survival (OS) time was 43 months (95 % CI 24-61 months). The anticipated 5-year OS rate was 36.6 % . Single factor analysis results revealed that patients who was female (χ2＝7.654, P＝0.006), with normal serum lactic dehydrogenase (LDH) level (χ2＝7.575, P＝0.006), normal serum β2microglobulin level (χ2＝4.770, P＝0.029) and without B symptom (χ2＝9.418, P＝0.002), IPI score 0-1 (χ2＝4.119, P＝0.042) had a better prognosis. Multivariate survival analysis indicated that symptoms B ( HR＝ 12.460, 95 % CI 2.645-58.708, P＝ 0.001) and IPI score ( HR＝7.521, 95 % CI 1.624-34.841, P＝0.010) were the independent factors for prognosis. Conclusion The prognosis of the patients with primary systemic ALK-ALCL is poor, especially for the worse prognosis of the patients with symptoms B and IPI sore 2-3.

Relato de caso de linfoma anaplásico de células grandes associado a implante mamário em paciente brasileira / Report of a case of anaplastic large cell lymphoma associated with a breast implant in a Brazilian patient

O linfoma anaplásico de células grandes (ALCL) associado a implantes mamários é um distúrbio linfoproliferativo das células T que foi recentemente reconhecido como uma entidade independente na classificação de linfomas da Organização Mundial de Saúde (OMS). Apesar do pequeno número de descrições, o número de casos está crescendo rapidamente. Das poucas centenas de casos que foram publicados até agora, muito poucos vieram do Brasil e nenhum foi relatado às autoridades locais. Encontramos um caso recentemente, e acreditamos que seu relato à comunidade local de cirurgia plástica poderá chamar a sua atenção para essa patologia emergente. O prognóstico é muito bom na maior parte dos casos diagnosticados. Contudo, ainda se sabe pouco sobre como e por que os implantes de silicone poderiam desencadear uma resposta linfoide, culminando num ALCL.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a T-cell lymphoproliferative disorder that has recently been recognized as an independent entity in the World Health Organization (WHO) classification of lymphomas. Despite the small number of reports to date, the number of cases is rapidly increasing. Of the few hundred cases that have been reported so far, very few came from Brazil and none have been reported to the local authorities. We encountered a case of BIA-ALCL and believe that its report to the local plastic surgery community could raise awareness to this emerging pathology. The prognosis is very good in most of the diagnosed cases. However, little is known about how and why silicone implants could trigger a lymphoid response that results in ALCL.

Clinicopathologic characteristics and prognosis of neoplastic cell-rich mixed cellularity classic Hodgkin lymphoma / 中华病理学杂志

Objective@#To investigate the clinicopathologic characteristics of neoplastic cell-rich mixed cellularity classical Hodgkin lymphoma(MCCHL-R) and to compare the prognosis with typical mixed cellularity classic Hodgkin lymphoma(MCCHL).@*Methods@#Fifty-four patients with MCCHL-R(the tumor cells >10%) and 65 patients with typical MCCHL identified from 1 721 Hodgkin lymphomas were reviewed to compare the clinicopathological characteristics including morphologic and immunophenotypic features, EBV infection status, clinical therapy and overall survival.@*Results@#The median age of the patients of MCCHL-R was 28.5 years(range: 9-76 years, male∶female=1.6∶1.0). Twenty-seven patients(50.0%) had B symptoms. Most patients had cervical lymph node involvement(81.5%, 44/54). Mediastinum and spleen involvement were seen in 69.2%(36/54) and 24.1%(13/54), respectively. Extranodal non-lymphoid organ involvement was seen in 41.3%(19/46) cases. Morphologically, lymph node architectures were effaced at various degree with large neoplastic cells of variable morphology, including Hodgkin/Reed-Sternberg(H/RS) cells and anaplastic large cells. There were abundant background heterogeneous admixtures of non-neoplastic inflammatory and accessory cells that were predominant mature small lymphocytes. All tumors were positive for CD30 and weakly positive for PAX5. Epstein-Barr encoded RNA(EBER)detectable by in situ hybridization was seen in 39.0% cases. Forty-six patients had a median follow-up time of 32.5 months(range: 5-128 months) and the 5-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients were 91.7% and 50.1%, respectively(P<0.05). The 5-year survival rate for MCCHL-R was lower than typical MCCHL patients. Single factor analysis showed that age of >45 years, extranodal involvement and stage Ⅲ-Ⅳ were correlated with poorer 5-year survival rate(P<0.05). Multiple factors Cox proportional hazards regression showed that extranodal involvement was the independent prognostic factor(RR: 4.352, 95%CI: 1.122-16.879, P<0.05).@*Conclusions@#MCCHL-R is more common in young people. The tumor has pathological features of classic Hodgkin lymphoma enriched with the tumor cells(>10%) and similar immunophenotype to classical Hodgkin lymphoma. Compared with typical MCCHL, extranodal disease is an independent prognostic factor of MCCHL-R.

Diagnosis and treatment of anaplastic large-cell lymphoma in children and adolescents: a retrospective multicenter survey study / 中华儿科杂志

Objective@#To provide a descriptive review and improve our understanding of the clinical characteristics and treatment outcome of pediatric anaplastic large cell lymphoma (ALCL) in China.@*Method@#The clinical data and outcomes of patients under 16 years of age with newly histopathologically-confirmed ALCL in 10 large single institutions in China between January 2009 and June 2014, were retrospectively analyzed.The event-free survival (EFS) was analyzed by the Kaplan-Meier method.The risk factors of disease progression or relapse were evaluated by logistic regression analysis.Significance was defined as P<0.05.@*Result@#Of the 80 eligible patients (52 male, 28 female), the median age was 8.4 years (range, 1.3-15.7 years). Two patients (3%) were Stage Ⅰ, 9(11%) Stage Ⅱ, 64 (80 %) Stage Ⅲ, and 5(6%) Stage Ⅳ.The median time of follow-up was 25.2 months (range, 7.1-74.8 months), 55 patients survived without disease at the last of follow-up, the 3-year EFS was (65±6)%. Sixty-five patients (81%) were treated with the Chinese Children Cancer Group-B cell-non-Hodgkin Lymphoma 2010 protocol, regimen and 15 cases (19%) were treated with other regimens.The 3-year EFS were (68±5)% vs.(65±20)% (P=0.21). The 3-year EFS was (57±7)% and (78±11)% for patients with or without B symptoms (P=0.01). Twenty-four patients experienced disease progression or relapse. The median time from initial diagnosis to tumor failure was 7.0 months (ranged, 1.5-42.6 months) (median). At the last evaluation, there were 5 patients still alive after disease progression and relapse. By univariate analysis, sex (P=0.04) and B symptoms (P=0.00) were identified as risk factors of disease progression or relapse.Nevertheless, only B symptoms (HR 5.60, 95%CI 1.47-21.27, P≤0.05) were risk factors in multivariate analysis.@*Conclusion@#The presenting features of children and adolescents with ALCL and efficiency in this study were similar to those reported by western countries.Refinement of therapeutic strategies to improve survival for patients with disease progression or relapse should be the priority in future clinical study.

Treatment advances in early-stage primary systemic anaplastic large cell lymphoma / 肿瘤研究与临床

Primary systemic anaplastic large cell lymphoma (ALCL) is a subtype of rare and heterogeneous clinical entity, extremely rare in early stage of disease. There is no standard treatment yet. Based on retrospective studies, systematic chemotherapy followed by involved field radiation after chemotherapy is the mainstay of treatment. The role of radiotherapy in the treatment of early stage ALCL remains unclear due to lack of prospective studies. So do the optimal chemotherapy regimens and hematopoietic stem cell transplantation. The paper will review these issues.

Advances in the diagnosis of hemophagocytic lymphohistiocytosis / 白血病·淋巴瘤

Hemophagocytic syndrome is a dangerous disease,with rapid progression and high mortality,which can be divided into primary and secondary hemophagocytic syndrome.This article reviews the research progress on the diagnosis of hemophagocytic syndrome in recent years.

Anaplastic Lymphoma Kinase-Positive Anaplastic Large Cell Lymphoma Arising in a Patient with Hypersensitivity to Mosquito Bites / 가정의학회지

Hypersensitivity to mosquito bites is defined as the appearance of intense skin reactive lesions and systemic symptoms subsequent to mosquito bites. Most cases of hypersensitivity to mosquito bites reported thus far have been associated with chronic Epstein-Barr virus infection or natural killer cell leukemia/lymphoma. In this study, we describe the case of an 18-year-old Korean boy who had hypersensitivity to mosquito bites associated with primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. After a mosquito bite, the patient developed a progressive cutaneous nodule on his left lower leg and regional lymphadenopathy in the left inguinal area. The histopathological and immunohistochemical findings suggested anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. Positron emission tomography-computed tomography revealed increased fluorodeoxyglucose uptake in the left T4 vertebrae, left external iliac lymph nodes, left inguinal lymph nodes, and lateral subcutaneous region of the left lower leg. According to the clinical, histopathological, and immunohistochemical findings, as well as the imaging data, the patient was diagnosed with primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. Consequently, the patient received a total of 6 cycles of cyclophosphamide + doxorubicin + vincristine + prednisolone chemotherapy at 3-week intervals, after which the lesions regressed.

Primary cutaneous anaplastic large-cell lymphoma - case report / Linfoma cutaneo primario de grandes celulas anaplasicas - relato de caso

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.

Clincopathological observation of gastrointestinal anaplastic large cell lymphoma / 白血病·淋巴瘤

Objective To explore the clinicopathological features,immunohistochemical characteristics of gastrointestinal anaplastic large cell lymphoma (ALCL).Methods Clinical manifestations,histology and immunophenotype of three cases diagnosed with gastrointestinal ALCL were analyzed with the review of related literatures.Results The clinical manifestations of gastrointestinal ALCL were of no characteristical changes.The pathological features were pleomorphic in which some bizarre,horseshoed or kidney-shaped nuclei were seen.The characteristic of one of the three cases was shown as sarcomatoid.All cases expressed LCA,CD3,CD4,CDs,CD45Ro,CD30,two out of three cases expressed ALK protein,otherwise they were negative for B cell related antigens,CD15 and muscle,nerve,epithelium originated antigens.Conclusions ALCL of the gastrointestinal tract is extremely rare as one of the lymphatic hematopoietic system tumors.No definite clinical characteristic is seen in the gastrointestinal ALCL with high grade malignancy.It is easily misdiagnosed as carcinomas or other sarcomas.It is necessary to use biopsy to make a pathological diagnosis.

Prognostic value analysis of detection of endothelial progenitor cell in 30 patients with anaplastic large cell lymphoma / 白血病·淋巴瘤

Objective To check the changes of endothelial progenitor cell (EPC) number of patients with anaplastic large cell lymphoma (ALCL) in the peripheral blood,investigate their clinical significance.Methods The number of EPC in blood was determined by FCM method in 30 patients with ALCL and 10 healthy cases as the control group.Results The number of EPC in the peripheral blood of patients with ALCL before treatment was significantly higher (15.530±28.659/μl) than that in control group (0.515 ±0.294/μl,P ＜ 0.001).The number of EPC of ALCL patients in the high-risk groups (21.521±36.057/μl) and the middle-risk groups (16.830±24.273/μ1) differently increaasd than that of the low-risk group (6.508±7.356/μl,P ＜ 0.01),but between the high-risk groups and the middle-risk groups there was no significant value (P ＞ 0.05).There were significant difference between the number of EPC of ALK+-ALCL (8.367±9.609/μl) and ALK-ALCL (22.541± 20.845/μl) patients (P ＜ 0.01).The survive curve before 60 weeks had significant difference between groups of ＞20/μl and ＜20/μl of EPC.Conclusion EPC may be correlated with progression of the disease in a certain degree.Dynamic observation with the level of EPC may be used to evaluate the treatment outcomes and act as a prognostic marker for ALCL.

Advances in primary systemic anaplastic large cell lymphoma / 白血病·淋巴瘤

Primary systemic anaplastic large cell lymphoma is a subtype of rare and heterogeneous clinical entity.There is no standard treatment as yet.Based on retrospective studies,systematic chemotherapy is the main treatment while combining field radiation with chemotherapy is always used for diseases at early stage. Recently, there are some improvement were made on this kind of disease in the pathology and pathogenesis because of the advancement in molecular biology. This review focuses on the pathogenesis,pathology,treatment and prognosis of primary systemic anaplastic large cell lymphoma.