Primary Anaplastic Large Cell Lymphoma Research Articles

Anaplastic Large Cell Lymphoma With Intraocular or Ocular Adnexal Involvement: A Case Report and Systematic Review.

To review the existing literature on patients with anaplastic large cell lymphoma (ALCL) affecting the globe and/or ocular adnexa, and to present a report documenting the clinical course of a patient with ALCL that involved their globe and ocular adnexa. PubMed, Scopus, and Google scholar were systematically searched for all cases of ALCL involving intraocular or adnexal ocular structures from inception to May 2023. Moreover, a new reported case added to the cases found in searches. The review identified 1680 studies, with 8 meeting inclusion criteria. A total of 9 patients were included with a mean age of 29.7 years (median: 30.0, range: 1.3-48). Primary ALCL was present in 5/9 (55.6%) patients. The most common ophthalmic manifestations included periorbital swelling (5/8), chemosis (5.8), and decreased vision (5/7). Misdiagnoses were initially made in 3 patients, and the lag time to correct diagnosis from 3 weeks to 3 months. CD30 expression was positive in all cases, and 6/9 patients were positive for anaplastic lymphoma kinase, resulting in 6/9 patients being diagnosed with anaplastic lymphoma kinase-positive ALCL. In terms of management modalities, chemotherapy was administered in 8/9 patients, while radiation therapy was utilized in 4/9 patients, and 2 underwent autologous stem cell transplantation. Five (55.6%) patients succumbed to ALCL while 4 (44.4%) were alive and disease-free at the last follow-up. The median times from the initial presentation of ALCL to death, ophthalmic presentation to death, and diagnosis to death were 4.12 months (range: 1.1-168.0), 2.62 months (range: 1.1-144), and 4.00 months (range: 0.10-168), respectively. The median follow-up duration was 21.0 months (range: 1.1-168.0). ALCL involving the globe and ocular adnexa is a rare and highly malignant tumor that can mimic benign clinical conditions. Early biopsy and aggressive treatment with chemotherapy regimens such as CHOP and radiation therapy may be useful.

P-176 - Primary anaplastic large cell lymphoma in children and adolescents: case reports and literature review

P-176 - Primary anaplastic large cell lymphoma in children and adolescents: case reports and literature review

Predictors of radiation-induced skin reactions in patients with T-cell skin lymphomas after total skin irradiation: retrospective cohort study

INTRODUCTION: Skin reactions negatively affect a patient’s quality of life and may require radiation therapy premature interruption, what impairs illness control. Numerous agents determine tissue radiosensitivity, but demographic and lifestyle factors remain poorly understood. Those kind of patients herapeutic algorithm demands studies.OBJECTIVE: Radiation-induced skin reactions (RISR) formation and severity prognostic factors determination for primary malignant skin lymphomas patients.MATERIALS AND METHODS: 45 patients malignant T-cell skin lymphoma study, aged 38 to 82 years (29 mycosis fungoides patients, 2 lymphomatoid papulosis, 14 patients with primary anaplastic large cell skin lymphoma) received fast electrons total skin irradiation on the basis of the Federal State Institution «Russian Radiology and Surgical Technologies Scientific Center named after Academician A.M.Granov» of the Ministry of Health of the Russian Federation from 2012 to June 2022. Radiation therapy performed in the usual fractionation mode on linear electron accelerators Elekta Precise with 4–6 MeV bremsstrahlung total doses from 14 to 30 Gy (22±2.4 Gy). In order to identify the role of possible prognostic factors of skin toxicity — gender, age, body mass index (BMI), smoking status, lymphoma body surface area (BSA), the presence of concomitant diseases (diabetes mellitus and arterial hypertension) and previous chemotherapy.Statistics: Statistical analysis was performed using the IBM SPSS Statistics v. 26 program (developed by IBM Corporation). The value of p<0.05 was considered statistically significant. Verification of the sample belonging to the normal general population was carried out using the Shapiro–Wilk criterion. Spearman's rank correlation coefficient was used for correlation analysis. The regression parameters were estimated using the least squares method.RESULTS: The analysis revealed positive associations between the severity of RISR and predictor factors: BMI (r=0.799, p<0.01), smoking status (r=0.655, p<0.01), age (r=0.534, p<0.05), presence of diabetes mellitus (r=0.456, p<0.05), arterial hypertension (r=0.227, p<0.05), previous chemotherapy (r=0.422, p<0.01) and lymphoma body surface area (r=0.378, p<0.01). Severity of RISR scale value increase by those parameters. Negative links found between the severity of RISR and the total focal dose (SOD) of the beginning of their formation (r=–0.109, p<0.05).CONCLUSION: Formation and severity prognostic factors for the RCC upon fast electrons total skin exposure for patients with primary malignant T-cell skin lymphomas (mycosis fungoides, lymphomatoid papulosis, primary anaplastic large cell lymphoma of the skin) were identified.

Primary Breast Anaplastic Large-Cell Lymphoma

Abstract We reported a rare case and experience of successfully treating primary anaplastic large-cell lymphoma (ALCL) of the breast with no history of breast implants, using a combination of novel brentuximab vedotin and chemotherapy. A 78-year-old woman presented with a painless lump over the left breast. The computed tomography scan revealed a 2.6-cm tumor in the left upper quadrant of the breast, along with enlarged lymph nodes in the left axilla, exhibiting similar imaging features to breast carcinoma. The definitive diagnosis was based on histopathology and immunohistochemistry stains consistent with ALCL. The patient received brentuximab vedotin in combination with cyclophosphamide, doxorubicin, and prednisone (CHP) chemotherapy, as well as involved site radiation therapy (ISRT). The following image showed almost complete remission.

A Case of Primary Cutaneous Anaplastic Large Cell Lymphoma that Presented as Multiple Cutaneous Masses and Required Differentiation from Adult T-cell Leukemia-Lymphoma

A Case of Primary Cutaneous Anaplastic Large Cell Lymphoma that Presented as Multiple Cutaneous Masses and Required Differentiation from Adult T-cell Leukemia-Lymphoma

Primary Central Nervous System ALK-Negative Anaplastic Large Cell Lymphoma: A Case Report and Literature Review

Primary Central Nervous System ALK-Negative Anaplastic Large Cell Lymphoma: A Case Report and Literature Review

Primary Anaplastic Large Cell Lymphoma of Skeletal Muscle Around the Left Hip Joint

Primary Anaplastic Large Cell Lymphoma of Skeletal Muscle Around the Left Hip Joint

Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement

Anaplastic large cell lymphomas (ALCL) are mature T-cell neoplasms, approximately half of which harbor rearrangements of the ALK gene that confer a good prognosis. Recent studies have demonstrated that a significant proportion of ALK-negative ALCLs demonstrate rearrangements of the IRF4/DUSP22 locus that also are typically associated with a favorable prognosis. ALCL with primary involvement of the central nervous system (CNS) is extremely rare. We report what may be the first case of ALK-negative ALCL with IRF4/DUSP22 rearrangement involving the brain in a 55-year-old man. Magnetic resonance imaging demonstrated signal abnormalities in the periventricular region, corpus callosum and cingulate gyrus. Biopsy revealed a diffuse parenchymal and angiocentric infiltrate of CD30-positive cells that showed IRF4/DUSP22 rearrangement by fluorescence in situ hybridization. We also review the clinical and pathologic features of primary CNS ALK-negative ALCLs in the literature and highlight the need for awareness of this entity to optimize appropriate management.

Bio-O1-02 - Deciphering discrepancies in cutaneous lymphomas genetics

In several instances, our group has reported opposite findings to others when analyzing the genetic features of primary cutaneous T-cell or B-cell lymphomas (CTCL or CBCL). This was especially the case for the absence of NAV3 rearrangements in CTCL, the low rate of BCL2 or MYC rearrangement in primary cutaneous follicle center lymphomas (CFCL) or primary cutaneous large B-cell lymphomas, leg-type (CLBCL, LT), respectively. Here, we review bias accounting for such discrepancies. Primary cutaneous lymphoma usually present with characteristic clinical and pathological features which serve as universal criteria for initial diagnosis and treatment. When required, extensive staging including CT scan, blood analysis and for some groups bone marrow staging, is mandatory to eliminate a secondary CTCL or CBCL. Early spreading may change the initial diagnosis and a long-term follow-up is also necessary to determine the risk for extracutaneous involvement. Non-uniform inclusion criteria and follow-up time may contribute to discrepancies, especially for studies claiming for a high rate of BCL2 rearrangement in CBCL. Most CBCL or CTCL usually display an indolent course except for aggressive subtypes arising de novo such as Sézary syndrome (SS), tumor or transformed mycosis fungoides (T-MF) or CLBCL-LT. Borderline cases between indolent and aggressive cutaneous lymphomas exist and may be called for example as leukemic CTCL or CBCL, not-otherwise specified (NOS) leading to heterogeneous terminology between centers. Moreover, aggressive CL are rare occurring mainly in adults or elderly that limits therapeutic options and comparison of small groups in retrospective series. The distribution of diagnosis between the three main primary CBCL including marginal-zone lymphoma (MZL), CFCL and CLBCL, LT has considerably evolved and its heterogeneity between centers may also hamper uniform findings. Our proposal to classify large CBCL according to germinal center or non-germinal center origin has so far not been validated by others while in systemic lymphomas genomic profiling appears mandatory to identify patients with impaired prognosis. In the most frequent cutaneous lymphomas, no highly recurrent genetic mutation is commonly used for diagnosis purpose. Some genetic lesion such as CCND1 rearrangement in mantle cell lymphoma can serve a positive criteria of secondary skin involvement. Alternatively, exceptional primary anaplastic large cell lymphoma (ALCL) bear ALK rearrangement preventing to use such criteria as a hallmark of secondary skin ALCL. BCL2 rearrangement in primary CFCL contributes to identify patients at risk for extracutaneous spreading. Most reports found negative data for NAV3 rearrangement in CTCL that may not be a primary oncogenic driver but a secondary event. Interestingly, MYC rearrangement or double-hits may have a low impact on prognosis in CBCL while they are usually associated with impaired prognosis in systemic or nodal lymphomas. Alternatively, some highly recurrent mutation such as those of MYD88 in CLBCL, LT should be routinely assessed. While clinical presentation will remain a basis for CL diagnosis and treatment, it is now time to integrate molecular profiling in research studies or clinical trials to standardize diagnosis and to identify patients which will exhibit therapeutic resistance to conventional first-line treatment.

Clinical Features and Prognostic Factors for Primary Anaplastic Large Cell Lymphoma of the Central Nervous System: A Systematic Review.

Simple SummaryPrimary anaplastic large cell lymphoma (ALCL) of the central nervous system (CNS) is a subtype of primary central nervous system lymphoma (PCNSL). ALCL is divided into anaplastic lymphoma kinase (ALK)-positive ALCL and ALK-negative ALCL, according to ALK expression. ALK-positive cancers tend to develop at a younger age and tend to have a better prognosis. Almost all past articles on primary ALCL of the CNS have been case reports and there have been no randomized trials or cohort studies on this subject. We thus performed a systematic review of primary ALCL of the CNS. According to the author’s survey, 36 case reports have been published in English-language journals. In this paper, we have summarized the clinical features and prognostic factors for primary ALCL of the CNS based on previous studies.Primary anaplastic large cell lymphoma (ALCL) of the central nervous system (CNS) is a subtype of primary CNS lymphoma (PCNSL). There are very few comprehensive reports on this extremely rare tumor. Therefore, it is necessary to investigate the clinical features and prognostic factors for primary ALCL of the CNS. We performed a systematic review of the published literature. Past cases were comprehensively searched using PubMed, Cochrane Library, and Web of Science. Clinical information, such as age, sex, anaplastic lymphoma kinase (ALK) status, lesion sites, treatment methods, and survivorship were extracted. Thirty-nine cases with information on ALK status and treatment course were identified. The average observation period was 13 months, and the overall 2-year survival rate was 58%. Univariate analyses showed a statistically significantly better prognosis among patients < 40 years of age (p = 0.039, HR 0.32 (0.11–0.95)) and in relation to ALK positivity (p = 0.010, HR 0.24 (0.08–0.71) and methotrexate treatment (p = 0.003, HR 0.17 (0.05–0.56)). Because of the sparsity of cases, it is necessary to accumulate cases in order to perform more detailed analyses.

Cutaneous T-cell lymphoma in Asian patients: a multinational, multicenter, prospective registry study in Asia.

Cutaneous T-cell lymphomas (CTCLs) are a group of T-cell lymphomas with low incidence. Due to their indolent characteristics, treatment strategies have not yet been established for advanced CTCLs. In this study, relative incidence of CTCLs in Asia was estimated and the therapeutic outcomes presented based on various treatments currently used in clinics for advanced CTCLs. As part of a prospective registry study of peripheral T-cell lymphoma (PTCL) conducted across Asia, including Korea, China, Taiwan, Singapore, Malaysia, and Indonesia, subgroup analysis was performed for patients with CTCLs. Among 486 patients with PTCL, 37 with CTCL (7.6%) were identified between April 2016 and February 2019. Primary cutaneous ALK-negative anaplastic large cell lymphoma (ALCL, 35.1%) was the most common subtype. With a median follow-up period of 32.1months, median progression-free survival (PFS) was 53.5months (95% CI 0.0-122.5), and overall survival was not reached. 14 patients (48.2%) underwent subsequent treatment after the first relapse, but the response rate was 20% with a PFS of 2.2months (95% CI 0.3-4.0). Six patients received autologous stem cell transplantation (auto-SCT). However, auto-SCT did not result in better outcomes. Additional studies are needed on standard care treatment of advanced or refractory and relapsed CTCLs.

Aberrant expression and genetic alteration of c-MYC in anaplastic large cell lymphoma.

c-MYC plays an important role in regulating cellular growth and apoptosis, and it is aberrantly expressed in many human malignancies. Although c-MYC has been extensively investigated in Burkitt lymphoma and diffuse large B cell lymphoma, little has been reported in anaplastic large cell lymphoma (ALCL). The aim of this study was to investigate the expression and genetic alterations of c-MYC in primary systemic ALCL, characterize its clinicopathologic features and immunophenotypes, and discuss their implications in prognosis. Tissue microarrays using samples from 85 ALCL patients were used to evaluate expression of c-MYC and anaplastic lymphoma kinase (ALK). c-MYC and ALK genetic alterations were detected using fluorescence in situ hybridization. The Kaplan-Meier and multivariate Cox regression methods were used for survival analysis. c-MYC was expressed in 24 of 85 samples (28.2%), and ALK was expressed in 54 (63.5%). c-MYC and ALK were co-expressed in 16 samples (18.8%). c-MYC expression and c-MYC and ALK co-expression increased with ALCL clinical stages and the International Prognostic Index (IPI) score (p < 0.05). Fifty of the samples (58.8%) had ALK rearrangement, and 18 (22.1%) had aneuploidy. c-MYC rearrangement was not detected, but aneuploidy was observed in 19 cases (22.4%). c-MYC aneuploidy was significantly different based on c-MYC expression and the IPI score (p < 0.05). c-MYC was a significant independent prognostic factor for progression-free survival and overall survival in patients with ALCL. c-MYC protein expression and c-MYC aneuploidy could predict worse survival in patients with ALCL.

Young survivor of a rare primary anaplastic large cell lymphoma of the trachea

Primary anaplastic large cell lymphoma of the trachea is a very rare tumor. Diagnosis is often missed for a long time because the presentation often resembles that of obstructive airway disease. A 24-year-old non-smoker male presented at an outpatient clinic complaining of gradual development of stridor and shortness of breath over a recent period of four weeks. Imaging workup revealed a large lobulated, soft tissue, mildly enhancing, eccentrically placed intraluminal proximal tracheal mass, which showed extension outside of the tracheal lumen in the peritracheal fat. The patient underwent surgery. A biopsy of the resected mass reported anaplastic large cell lymphoma. Post-operation, the patient underwent chemotherapy consisting of four cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone). Follow-up imaging showed non-visualization of the mass. Primary lymphoma (anaplastic large cell lymphoma, which is a rare type of non-Hodgkin’s lymphoma) of the trachea is a rare tumor. Early curative resection with post-operative chemotherapy has a favorable outcome, as in our case.

Five-year clinical monitoring of the right ventricle with right ventricular strain measurement after primary cardiac anaplastic large cell lymphoma resection.

Five-year clinical monitoring of the right ventricle with right ventricular strain measurement after primary cardiac anaplastic large cell lymphoma resection.

Primary anaplastic lymphoma kinase-negative anaplastic large cell lymphoma of cervical spine presenting with quadriplegia: A case report and literature review

Background: An anaplastic large cell lymphoma (ALCL) involving the cervical spine and leading to quadriplegia is very rare.Case Description: A 48-year-old immunocompetent male presented with quadriplegia that warranted an anterior cervical corpectomy/fusion. He was previously being presumptively treated for cervical disease attributed to tuberculosis. The histopathology and immunohistochemistry revealed an ALCL that was anaplastic lymphoma kinase (ALK) negative. The patient had a favorable response to surgery followed by CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisolone) chemotherapy.Conclusion: ALK-negative ALCL presenting with quadriplegia due to primary involvement of cervical spine is extremely rare, but must be diagnosed and appropriately managed.

Recurrence of Primary Cutaneous Anaplastic Large Cell Lymphoma at a New Location After Wide Surgical Excision

Abstract not available.

Cerebrospinal fluid cytology in a young adult with seizures

Involvement of the central nervous system (CNS) by anaplastic large cell lymphoma (ALCL) is rare with majority of the reported cases being primary CNS ALCLs. There are very occasional reports of CNS involvement in systemic ALCL. Establishing a diagnosis of CNS infiltration by ALCL, although difficult, carries immense prognostic significance and involves thorough clinical neurological assessment, neuroimaging studies, and cerebrospinal fluid cytology. Herein, we present a case of CNS involvement in a patient with systemic ALCL, diagnosed on CSF cytology complemented by CSF flow cytometry and immunocytochemistry on the cyto-spots.

PRIMARY PULMONARY ANAPLASTIC LARGE CELL LYMPHOMA IN A YOUNG MALE

SESSION TITLE: Fellows Lung Cancer Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma which is subclassified into primary systemic anaplastic lymphoma kinase (ALK)-positive, primary systemic ALK-negative, primary cutaneous ALCL, and breast implant-associated ALCL (1). ALCL is considered an extremely rare type of primary pulmonary lymphomas (2). We report a case of primary pulmonary ALCL in a young immunocompetent male presenting with dyspnea. CASE PRESENTATION: 43 year-old male with no past medical history presented to the emergency department with dyspnea, fatigue and lower extremity swelling which have been worsening over a month. Physical exam showed 3+ pitting edema bilaterally and bilateral decreased breath sound. Laboratory workup showed an elevate d-dimer and an elevated absolute monocytes. Chest X-ray showed numerous nodules throughout the lungs. CT angiogram of the chest, abdomen and pelvis showed a large infiltrative opacity extending from the right hilum to the pleural surface in the right lower lobe, with some extension into the central right middle lobe and innumerable pulmonary and hepatic metastases and lymphadenopathy in the thoracic, abdominal and pelvic lymph node chains. Tumor markers were negative, with mildly elevated CA-125. A right supraclavicular lymph node biopsy showed necrotizing lymphadenitis with no evidence of metastatic disease. Bronchoscopy revealed an extensive obstruction of the bronchial lumen with an endobronchial lesion in the right lower lobe bronchus. Tissue biopsy was consistent with anaplastic large cell lymphoma. Patient was critically ill in the intensive care unit and was intubated due to acute respiratory failure. He had a poor prognosis due to the extensive metastasis of his ALCL. DISCUSSION: ALCL is characterized by large lymphoid cells with pleomorphic nuclei, and uniform CD30/Ki-1 expression (2). The most common form of ALCL presents as primary cutaneous lymphoma and may secondarily involve the lungs. However, primary pulmonary ALCL is extremely rare with 10 published cases between 1990 and 2015 (3). ALK protein expression is an important prognostic indicator for ALCL as ALK-positive lymphomas have a favorable prognosis with a much higher 5-year survival rate (2). The diagnosis of ALCL includes histopathology and immunohistochemistry staining, with strong immunoreactivity for CD30. Our patient had a primary pulmonary ALCL which was ALK-negative, indicating poor overall prognosis, with a strong immunoreactivity for CD30 and Ki-1. The treatment of primary pulmonary ALCL has not been standardized. However, first-line therapy includes anthracycline-based regimen, which is the standard treatment for non-Hodgkin lymphomas. CONCLUSIONS: Our case underlines the importance of maintaining a high index of awareness for the occurrence and impact of the recognition of primary pulmonary ALCL and its implications on the clinical course of critically ill patients. Reference #1: Laurent, Camille; Haioun, Corinne; Brousset, Pierre; Gaulard, Philippe (1 September 2018). “New insights into breast implant-associated anaplastic large cell lymphoma”. Current Opinion in Oncology. 30 (5): 292–300. Reference #2: J.-F. Cordier, E. Chailleux, D. Lauque et al., “Primary pulmonary lymphomas; a clinical study of 70 cases in nonimmunocompromised patients,” CHEST, vol. 103, no. 1, pp. 201–208, 1993. Reference #3: Q. Zhao, Y. Liu, H. Chen et al., “Successful chemo-radiotherapy for primary anaplastic large cell lymphoma of the lung: A case report and literature review,” American Journal of Case Reports, vol. 17, pp. 70–75, 2016. DISCLOSURES: No relevant relationships by Obed Adarkwah, source=Web Response No relevant relationships by Raheel Anwar, source=Web Response No relevant relationships by Louis Gerolemou, source=Web Response No relevant relationships by Wael Kalaji, source=Web Response No relevant relationships by jad sargi, source=Web Response No relevant relationships by Viswanath Vasudevan, source=Web Response

Primary anaplastic large cell lymphoma of the central nervous system in a child

Introduction:To report the clinical characteristics of primary central nervous system T-cell lymphoma with anaplastic lymphoma kinase-1 (ALK-1) positive in an 8-year-old male.Patient concerns:The patient presented cognitive impairment, dizziness, vomiting, fever, and convulsions during the disease, followed by progressive and persistent severe headache, progressive increase of intracranial pressure, indifference, disorder of consciousness, mild increase in white blood cells in cerebrospinal fluid, progressive decrease of sugar, progressive increase of protein, abnormal signal of left parietal-occipital, local meningeal enhancement, and cerebrospinal fluid cytology.Diagnosis:He was diagnosed with ALK-1-positive central nervous system T-cell lymphoma.Interventions:Meropenem and vancomycin were administered to counter the infection, while dexamethasone alleviated the inflammation.Outcomes:The patient died of cerebral hernia due to intracranial hypertension in the eighth week of the disease.Conclusions:PCNS ALK-1-positive anaplastic large cell lymphoma is extremely rare. Also, it is difficult to distinguish from central meningeal lymphoma and central nervous system infection, which might lead to delayed diagnosis. However, early diagnosis depends on the pathological diagnosis of brain tissue biopsy.

Clinical characteristics and prognostic factors of 40 cases of primary systemic anaplastic large cell lymphoma

目的分析原发系统型间变性大细胞淋巴瘤（ALCL）的临床特征及预后因素。方法回顾性分析2013年1月至2018年12月期间在浙江大学医学院附属第一医院住院治疗的40例ALCL患者的临床资料，总结其临床特征并分析其预后相关因素。结果①40例患者中男29例，女11例，中位年龄41（14～67）岁，Ann Arbor分期Ⅲ～Ⅳ期36例（90.0％），IPI评分中高危、高危23例（57.5％）；25例（62.5％）有B症状，发热最为常见，其次为消瘦、盗汗；38例（95.0％）有结外侵犯；25例（62.5％）LDH升高；25例（62.5％）Ki-67≥80％；ALK阳性22例（55.0％），中位年龄29（14～67）岁，ALK阴性18例（45.0％），中位年龄51.5（19～67）岁，年龄差异有统计学意义（P＝0.003）。②40例患者均接受化疗，采用CHOP（环磷酰胺+多柔比星+长春地辛+泼尼松）方案18例，ECHOP（依托泊苷+CHOP）方案12例，其他方案10例；26例（65.0％）获得完全缓解（CR），ALK阴性（P＝0.029，OR＝13.458）和Ki-67≥80％（P＝0.04，OR＝14.453）是CR的独立影响因素，ECHOP方案的CR率高于CHOP方案［83.3％（10/12）对38.9％（7/18），P＝0.026］。③LDH水平、IPI评分、ALK表达情况以及化疗方案的选择对无进展生存（PFS）和总生存（OS）影响有统计学意义（P<0.05）。结论ALCL发病男性居多，青少年至中老年均有发病，ALK阳性中年轻患者居多，诊断时临床分期晚，多数患者有结外侵犯，半数以上患者有B症状、LDH升高、Ki-67高表达；ALK表达与否、Ki-67表达水平以及化疗方案的选择可影响疗效，LDH水平、IPI评分、ALK表达与否以及化疗方案的选择可影响PFS和OS；ECHOP是较优的化疗方案，可改善预后。