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.
Adolescent ; Adult ; Child ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, Large-Cell, Anaplastic/therapy* ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Transplantation, Autologous ; Transplantation, Homologous ; Treatment Outcome ; Young Adult

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.
Aged, 80 and over ; Diagnosis ; Drug Therapy ; Forehead ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell ; Skin ; Viola

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.
Abdominal Pain ; Adolescent ; Drug Therapy ; Duodenum ; Endoscopy ; Female ; Hematoma ; Hepatitis ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Pancreatitis ; Vomiting

To determine clinical and pathologic profiles for anaplastic large cell lymphoma (ALCL).
 Methods: The clinical data of 22 patients with ALCL were analyzed retrospectively. Therapentie effect of different treatment strategies on ALCL was evaluated.
 Results: The median age for these patients was 32(9-70) years old and the patients with positive ALK accounted for 68.2% (15/22). All patients underwent chemotherapy, including regiments of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), CHOPE (CHOP plus etoposide) or BEACOP (CHOP plus etoposide and bleomycin). Fourteen (63.6%) patients achieved initial complete remission (CR) and the CR rate for patients with ALK+ was significantly higher than that of patients with ALK- (P<0.05), while the age, gender, stage, beta 2-microglobulin (2-MG) level, lactate dehydrogenase (LDH) level, B symptoms had no significant effect on the rate of CR (P>0.05). After a median follow-up of 41 (2-150) months, 12 patients were overall survival, the median progression free time was 22.5 (2-150) months, and the age, gender, stage, IPI index, ALK expression level, beta 2-MG level, LDH level, and B symptoms had no significant effect on the rate of overall survival (P>0.05).
 Conclusion: ALK-positive occurs mainly in ALCL patients. The chemotherapy is still the main treatment, and CHOPE regimen is a better initial treatment scheme because the most patients show good prognosis.
Adolescent ; Adult ; Age Factors ; Aged ; Alkaline Phosphatase ; Antineoplastic Combined Chemotherapy Protocols ; administration & dosage ; therapeutic use ; Bleomycin ; administration & dosage ; Child ; Cyclophosphamide ; administration & dosage ; Doxorubicin ; administration & dosage ; Etoposide ; administration & dosage ; Female ; Humans ; Lymphoma, Large-Cell, Anaplastic ; drug therapy ; enzymology ; mortality ; Male ; Middle Aged ; Prednisone ; administration & dosage ; Prognosis ; Retrospective Studies ; Sex Factors ; Treatment Outcome ; Vincristine ; administration & dosage ; Young Adult

BACKGROUND: Among pediatric non-Hodgkin lymphomas, there are 4 major subtypes: Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma, and anaplastic large-cell lymphoma. Understanding of other rare subtypes derives only from small pediatric case series. We report our institutional experience with rare pediatric NHLs.METHODS: Thirty-six cases of rare NHL subtypes diagnosed at the Asan Medical Center from 1995 to 2015 were evaluated. We retrospectively reviewed the clinical and pathologic features and outcomes of these patients, excluding peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), and extranodal NK/T-cell lymphoma (ENKL), on which we have previously reported.RESULTS: There were 23 cases of T-cell lineage (13 PTCL, 6 ENKL, 2 subcutaneous panniculitis-like T-cell lymphoma, 1 primary cutaneous CD4+2016-11-22 small/medium sized T-cell lymphoma, 1 enteropathy-associated T-cell lymphoma) and 13 cases of B-cell lineage lymphoma (5 marginal zone lymphoma, 6 primary mediastinal large B-cell lymphoma, 2 immunoblastic and plasmablastic lymphoma). All patients were treated with chemotherapy with or without surgery, except 4 out of 5 patients with marginal zone lymphoma who received surgery only. Two patients died and 6 patients relapsed. One patient with primary mediastinal large B-cell lymphoma received autologous peripheral blood stem cell transplantation. The 5-year overall survival and event-free survival rates of rare pediatric NHL excluding PTCL, NOS, and ENKL was 80.0% and 72.0%, respectively.CONCLUSION: Children diagnosed with rare pediatric NHL other than PTCL, NOS, and ENKL showed variable incidence and treatment outcomes. Multicenter studies in larger cohorts are needed for better understanding of these rare NHL subtypes in childhood.
B-Lymphocytes ; Burkitt Lymphoma ; Child ; Chungcheongnam-do ; Cohort Studies ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Peripheral Blood Stem Cell Transplantation ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; T-Lymphocytes

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare subtype of malignant non-Hodgkin lymphoma, in which 40% of the cases show spontaneous regression without aggressive treatment. Surgery and focal radiation therapy are the primary forms of treatment for this disease; however, if pcALCL is accompanied by multifocal skin lesions, chemotherapy is also common. The prognosis for pcALCL is generally excellent, with a 5-year survival rate of 85-100%. However, pcALCL with extensive limb disease typically has a poor prognosis. Here, we present a case of pcALCL with extensive limb disease that resulted in the patient's death, despite the use of aggressive chemotherapy.
Drug Therapy ; Extremities* ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Prognosis ; Skin ; Survival Rate

Patients with lymphomatoid papulosis have an increased risk (approx. 5% to 20%) of developing a malignant lymphoma such as mycosis fungoides, anaplastic large cell lymphoma (ALCL) and Hodgkin's disease before, during, or after lymphomatoid papulosis occurs. However, it is very rare that lymphomatoid papulosis occurs after ALCL, especially in childhood. An 11-year-old boy who had been diagnosed with ALCL 3 years prior and treated with chemotherapy and peripheral blood stem cell transplantation developed multiple scaly papules on his trunk and both extremities. Histopathologic and immunohistochemical examination of the scaly papules revealed lymphomatoid papulosis. The patient was cured with narrow band UVB treatment and there has been no relapse in lesions 10 years later. We report a case of lymphomatoid papulosis following allogenic stem cell transplantation for ALCL.
Child* ; Drug Therapy ; Extremities ; Hodgkin Disease ; Humans ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic* ; Lymphomatoid Papulosis* ; Male ; Mycosis Fungoides ; Peripheral Blood Stem Cell Transplantation ; Recurrence ; Stem Cell Transplantation

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.
Adolescent ; Culicidae* ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Electrons ; Herpesvirus 4, Human ; Humans ; Hypersensitivity* ; Killer Cells, Natural ; Leg ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma* ; Lymphoma, Large-Cell, Anaplastic* ; Male ; Prednisolone ; Skin ; Spine ; Vincristine

BACKGROUND: Anaplastic large cell lymphoma (ALCL) is uncommon in children, accounting for approximately 15% of all cases of childhood non-Hodgkin lymphoma. Despite many studies attempting new treatment strategies, treatment outcomes have not significantly improved, and the optimal treatment for pediatric ALCL has not been established. METHODS: The records of newly diagnosed ALCL patients at our institute between July 1998 and April 2013 were reviewed. We evaluated the general characteristics of the patients, chemotherapy regimens, overall survival (OS) rates, and event-free survival (EFS) rates. RESULTS: Twenty-eight ALCL patients were eligible. The median age at diagnosis was 10.8 years. Lymph node involvement was the most common presentation (79%). CCG-5941, a multi-agent T-cell lineage chemotherapy, was the predominant treatment regimen (57%). The five-year OS and EFS rates were 88% and 69%, respectively. Stage, the presence of B symptoms, lung involvement, and bone marrow involvement were significant prognostic factors for EFS (P=0.02, 0.01, 0.01, and 0.02, respectively). Eight patients relapsed, and three died during the study period. Four of the eight patients who relapsed were treated with high-dose chemotherapy and autologous stem cell transplantation (HDCT-ASCT). Two of the four who had undergone HDCT-ASCT developed secondary relapses and were subsequently treated with allogeneic SCT or brentuximab. CONCLUSION: We found that treatment outcomes with multi-agent chemotherapy in children with ALCL were similar to those of previous reports, and that relapsed patients could be salvaged with HDCT-ASCT or allogeneic SCT. A prospective, larger cohort study is warranted to define the optimal treatment for pediatric ALCL.
Bone Marrow ; Child* ; Cohort Studies ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Lung ; Lymph Nodes ; Lymphoma, Large-Cell, Anaplastic* ; Lymphoma, Non-Hodgkin ; Prognosis ; Recurrence ; Stem Cell Transplantation ; T-Lymphocytes

BACKGROUND: Treatment outcomes of children with non-Hodgkin lymphoma (NHL) have dramatically improved in recent years. However, there are few studies on the outcomes of pediatric NHL in Korea.METHODS: We retrospectively analyzed the outcomes of 34 children diagnosed with NHL and treated at Kosin University Gospel Hospital from Jan. 1987 to Dec. 2009, according to age, lactate dehydrogenase (LDH) level, histology, stage and involved site.RESULTS: The mean age of the subjects was 9.0 years. The abdomen and head/neck regions were the most common primary sites. On histologic classification, Burkitt lymphoma was the most common, followed by lymphoblastic lymphoma, diffuse large B-cell lymphoma, anaplastic large cell lymphoma, and unclassifiable, with respective incidences of 35.3%, 23.5%, 17.6%, 17.6%, and 5.9%. Various combination chemotherapies according to the diagnosis with mean treatment duration of 14.9 months showed 5 year event free survival (EFS) and 5 year overall survival (OS) rate of 67.7+/-8.0% and 79.3+/-7.0%, respectively. Nine out of the 34 patients relapsed, and the 5 year OS rates for those who relapsed vs. 25 patients without relapse were 44.4+/-16.6%, vs. 92.0+/-5.4%, respectively (P<0.01). Although 5 year EFS rate varied according to stage, 5 year OS rate were not different according to age, sex, LDH, stage, histology, or treatment period.CONCLUSION: The outcome of children with NHL treated in our setting was comparable to those of other large centers in Korea. No factor other than stage, including LDH, histologic subtype showed significant prognostic value.
Abdomen ; Burkitt Lymphoma ; Child ; Classification ; Diagnosis ; Disease-Free Survival ; Drug Therapy, Combination ; Humans ; Incidence ; Korea ; L-Lactate Dehydrogenase ; Lymphoma, B-Cell ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Non-Hodgkin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; Retrospective Studies