Diagnosis and treatment of pediatric anaplastic lymphoma kinasepositive large B-cell lymphoma:A case report

BACKGROUND Anaplastic lymphoma kinase-positive(ALK+)large B-cell lymphoma(LBCL)is a rare type of lymphoma with high invasiveness and rapid progression.It occurs in all age groups,but is extremely rare in children.The lesions mainly involve the lymph nodes and may present with extra-nodal involvement.Response to conventional chemotherapies and local radiotherapy is poor,with a 5-year overall survival of less than 40%.Recently,the use of ALK inhibitors for the treatment of this disease has been reported.CASE SUMMARY We present a case of a 12-year-old boy diagnosed with ALK+LBCL.The patient had a 2-mo medical history of a calvarial mass,extensive systemic involvement,and positive bone marrow clathrin heavy chain(CLTC)-ALK fusion gene.Complete remission 1(CR1)was achieved using the modified LMB89 Group C regimen followed by autologous stem cell transplantation.The patient relapsed 3 mo later.He then achieved CR2 with three short courses of chemotherapy(COP,reduceddose ICE,low-dose Ara-c+VP16)and continuous alectinib targeted therapy.Afterward,allogeneic hematopoietic stem cell transplantation(allo-HSCT)was performed.At 16 mo after the allo-HSCT,the patient was still in CR2.CONCLUSION The modified LMB89 Group C regimen and ALK inhibitors are effective.Allo-HSCT should be performed after remission.

Prognostic significance of locally invaded sites and tissue types in patients with nasal extranodal natura-killer/T-cell lymphoma: a single-center retrospective analysis

Background:Extranodal natural killer/T-cell lymphoma (ENKTL), nasal type, is an aggressive entity within the World Health Organization classification of lymphoid tumors. The International Prognostic Index is reported to be prognostically meaningful for ENKTL, but lacks discriminatory power for stage Ⅰ/Ⅱ ENKTL with extensive local invasion. This study aimed to evaluate the prognostic effects of local invasion by site and tissue type in patients with ENKTL. Methods: We retrospectively analyzed data of 86 patients who were diagnosed with ENKTL by the Department of Pathology of Beijing Tongren Hospital from June 2002 to April 2016, and ascertained tumor infiltration of adjacent structures (AS), bone, and soft tissue for each patient, using physical findings and imaging scans. We used univariate and multivariate analysis to assess the association of each involved tissue or site with patients5 overall survival (OS). Results: Of the 86 patients, 71(82.6%) experienced invasion of AS, 22(25.6%) of soft tissue, and 26(30.2%) had bone involvement. Overall, patients with AS involvement did not show significantly shorter survival than those without AS involvement (Log rank X^2 = 1.177, P = 0.278);however, patients who had involved eyeballs or brains showed significantly lower 2-year OS rates than those without eyeball involvement (Log rank x^2 = 4.105, P = 0.043) or brain involvement (Log rank x^2 = 7.126, P = 0.008). Patients with involved local soft tissue or bones, respectively, showed lower 2-year OS rates than those without involved local soft tissue (Log rank x^2 = 10.390, P = 0.001) or bones (Log rank x^2 = 8.993, P = 0.003). Multivariate analysis showed that involvement of the cheek or facial muscles (hazard ratio, HR = 5.471, 95% confidence interval [Cl]: 1.466-20.416, P = 0.011) and the maxilla bone (HR = 6.120, 95% Cl: 1.517-24.694, P = 0.011) were significantly independent predictors of lower 2-year OS rates. Conclusions: Imaging can accurately detect ENKTL invasion of AS, soft tissue, and bone. Involvement of local soft tissue or bone was significantly associated with lower 2-year OS rates. Involvements of the cheek or facial muscle, as well as maxilla bone, are independent predictors of lower 2-year OS rates in ENKTL patients.