OBJECTIVE: To investigate the clinical characteristics, diagnosis, and treatment of one patient with primary adrenal natural killer/T-cell lymphoma (PANKTCL), and to strengthen the understanding of this rare type of lymphoma. METHODS: The clinical manifestations, diagnosis and treatment process, and prognosis of the patient admitted in our hospital were retrospectively analyzed. RESULTS: Combined with pathology, imaging, bone marrow examination， etc, the patient was diagnosed with PANKTCL (CA stage, stage II; PINK-E score 3, high-risk group). Six cycles of "P-GemOx+VP-16" regimen（gemcitabine 1 g/m² d1 + oxaliplatin 100 mg/m² d 1 + etoposide 60 mg/m² d 2-4 + polyethylene glycol conjugated asparaginase 3 750 IU d 5） was performed, and complete response was assessed in 4 cycles. Maintenance therapy with sintilimab was administered after the completion of chemotherapy. Eight months after the complete response, the patient experienced disease recurrence and underwent a total of four courses of chemotherapy, during which hemophagocytic syndrome occurred. The patient died of disease progression 1 month later. CONCLUSION PANKTCL is rare, relapses easily, and has a worse prognosis. The choice of the "P-GemOx+VP-16" regimen combined with sintilimab help to improve the survival prognosis of patient with non-upper aerodigestive tract natural killer /T-cell lymphoma.
Humans ; Treatment Outcome ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Retrospective Studies ; Etoposide ; Neoplasm Recurrence, Local/drug therapy* ; Asparaginase ; Deoxycytidine ; Lymphoma, T-Cell, Peripheral/drug therapy* ; Lymphoma, Extranodal NK-T-Cell/therapy* ; Oxaliplatin/therapeutic use*

Objective: To explore the prognostic factors of extracellular NK/T cell lymphoma (ENKTL) treated with pegaspargase/L-asparaginase. Methods: The clinical data of 656 ENKTL patients diagnosed at 11 medical centers in the Huaihai Lymphoma Working Group from March 2014 to April 2021 were retrospectively analyzed. The patients were randomly divided into two groups: a training set (460 cases) and a validation set (196 cases) at 7∶3, and the prognostic factors of the patients were analyzed. A prognostic scoring system was established, and the predictive performance of different models was compared. Results: Patients' median age was 46 (34, 57) years, with 456 males (69.5% ) and 561 nasal involvement (85.5% ). 203 patients (30.9% ) received a chemotherapy regimen based on L-asparaginase combined with anthracyclines, and the 5-year overall survival rate of patients treated with P-GEMOX regimen (pegaspargase+gemcitabine+oxaliplatin) was better than those treated with SMILE regimen (methotrexate+dexamethasone+cyclophosphamide+L-asparaginase+etoposide) (85.9% vs 63.8% ; P=0.004). The results of multivariate analysis showed that gender, CA stage, the Eastern Cooperative Oncology Group performance status (ECOG PS) score, HGB, and EB virus DNA were independent influencing factors for the prognosis of ENKTL patients (P<0.05). In this study, the predictive performance of the prognostic factors is superior to the international prognostic index, Korean prognostic index, and prognostic index of natural killer lymphoma. Conclusion: Gender, CA stage, ECOG PS score, HGB, and EB virus DNA are prognostic factors for ENKTL patients treated with pegaspargase/L-asparaginase.
Male ; Humans ; Middle Aged ; Asparaginase/therapeutic use* ; Prognosis ; Retrospective Studies ; Lymphoma, Extranodal NK-T-Cell/drug therapy* ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Etoposide ; Cyclophosphamide ; Methotrexate/therapeutic use* ; DNA/therapeutic use* ; Treatment Outcome

OBJECTIVETo analyze the coagulation function and relevant factors of adults patients with acute lymphoblastic leukemia treated with pegasparase (PEG-ASP) or L-asaraginase (L-ASP).

METHODSThe clinical features of 153 patients with acute lymphoblastic leukemia (ALL) received L-ASP or PEG-ASP in our hospital from January 2010 to January 2015 year were analyzed retrospectively. Among 153 patients, 108 patients received L-ASP treatment and 45 patients received PEG-ASP treatment. The change of coagulation function and the incidence of complications of 2 treated groups were compared, and the influence of differenent using time of L-ASP on above mentioned factors were analyzed.

RESULTSThe age, sex, white blood cell count (WBC) at diagnosis, subtype and risk factors of disease, total effective rate and complication rates showed no significant difference in the 2 groups (P > 0.05). The total infusion of fresh frozen plasma (FFP), cryoprecipitate and fibrinogen (FIB) also showed no significant difference (P = 0.12, 0.65, 0.09). FIB levels decreased slower after treatment of PEG-ASP (9.49 vs 6.90) (P = 0.000) than that after treatment of L-ASP. When L-ASP used at interval, FIB level decreased slower than that of continuous use. However, the risk of bleeding is higher when used at interval early (P = 0.01, 0.013).

CONCLUSIONUsing PEG-ASP can better monitor the coagulation function than L-ASP. L-ASP used at interval can monitor the coagulation function easily, but its early use may cause an increased incidence of complications.

Adult ; Antineoplastic Agents ; therapeutic use ; Asparaginase ; therapeutic use ; Blood Coagulation ; drug effects ; Fibrinogen ; analysis ; Hemorrhage ; Humans ; Leukocyte Count ; Polyethylene Glycols ; therapeutic use ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; Retrospective Studies ; Risk Factors

OBJECTIVETo investigate the clinical characteristics of primary cutaneous γΔ T cell lymphoma and its treatment methods.

METHODSThe clinical data and treatment process of one woman case of primary cutaneous γ Δ T cell lymphoma diagnosed in our department were analysed. The multiple subcutaneous nodules were the main clinical features, the diagnosis of primary cutaneous γΔ T cell lymphoma was comfired by skin biopsy pathology. The immunophenotypes of lymphocytes showed CD20-, CD3+, CD4-, CD8-, CD56+, TIA-1+, Ki-67+ (about 60%); plasma cells kappa+(part)/lambda predominate+(part); histocytes CD4+, CD68/PGM1+; βF1-, epstein-barr (EB) virus showed negative EBER in situ hybridization.

RESULTSBy means of the chemotherapy regimens containing L-Asparaginase, the complete remission (CR) was achieved. Then, the patients were given autologous hematopoietic stem cell transplantation. Neutrophils were implanted after 16 days, and platelet was implanted after 18 days. Now, the patient is still in remission.

CONCLUSIONprimary cutaneous γΔ T cell lymphoma is rare and easy to be misdiagnosed. This disease is aggressive and its prognosis is poor. The large dose chemotherapy with L-asparaginase shows a certain curative efficacy, the autologous hematopoietic stem cells can prolong survival time of the patient.

Asparaginase ; therapeutic use ; Female ; Hematopoietic Stem Cell Transplantation ; Herpesvirus 4, Human ; isolation & purification ; Humans ; Immunophenotyping ; Lymphoma, T-Cell, Cutaneous ; therapy ; Remission Induction ; Transplantation, Autologous

OBJECTIVETo explore the effectiveness and safety of combined chemotherapy with pegasparaginase (PEG-Asp) for treatment of patients with acute lymphoblastic leukemia (ALL) and T cell non-Hodgkin's lymphoma (T-NHL) patients.

METHODSA total of 62 ALL or T-NHL patients were diagnosed and treated in our department and were enrolled in this study. Among them, 22 patients received the combined chemotherapy with PEG-Asp, while the other 40 patients received the standard chemotherapy with L-asparaginase (L-Asp) as the control. Therapeutic effectiveness, adverse effects, duration and expense of hospitalization, treatment-related mortality and survival were evaluated and compared in 2 different groups.

RESULTSIn group of combined chemotherapy with PEG-Asp, the overall response rate was 90.91% (20 cases), among them CR rate and PR rate are 77.27% (17 cases) and 13.64% (3 cases), respectively. In the group of standard chemotherapy with L-Asp, the overall response rate was 87.5% (35 cases), among them CR rate and PR rate were 72.5% (29 cases) and 15% (6 cases), respectively. The difference neither between PEG-Asp and L-Asp chemotherapy groups nor between ALL and T-NHL subgroups was significant (P > 0.05). The 6-month and 12-month overall survival rates were not significantly different between the PEG-Asp and L-Asp chemotherapy groups, respectively (P > 0.05). The adverse effects were identified as degree 1-2 according to the WHO criteria of drug toxicity. Neither the adverse effects identified as degree 3-4 nor the treatment-related death were observed. Expect for allergy and hyperglycaemia, the difference of side-effect incidence between the two groups were not significant (P > 0.05). The treatment for all the patients in PEG-Asp chemotherapy group was completed, while the treatment with L-Asp was completed only in 29 cases. Moreover, both average duration and expense of hospitalization after the combined chemotherapy were less than the control.

CONCLUSIONWith higher response rate, lower drug toxicity and allergy incidence, the combined chemotherapy with PEG-Asp can replace the standard chemotherapy with L-Asp in the treatment of ALL and T-NHL. The optimization of the combined chemotheropeutic protocols for more cases and long-term survival rates need to further and deeply explorate.

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Asparaginase ; therapeutic use ; Humans ; Lymphoma, T-Cell ; drug therapy ; Polyethylene Glycols ; therapeutic use ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; Survival Rate

The chemotherapy agent L-asparaginase (L-asp) has been an important part of acute lymphoblastic leukemia therapy for over 30 years. It is evident that L-asp has a long-term curative effect. However, L-asp is associated with high incidence of adverse reactions. This has prompted the development of pegylated asparaginase (PEG-asp), which has undergone extensive testing. Apparently, PEG-asp has a prolonged half-life with a better tolerance profile while retaining the antileukemic effect. In this review, we attempt to outline the history of clinical application of L-asp, the pharmacological and clinical potential of various preparations of L-asp, the development of PEG-asp, and the clinical application and adverse events of PEG-asp. The literatures reviewed in this article is collected through online search of the major databases both in English and Chinese.
Antineoplastic Agents ; therapeutic use ; Asparaginase ; adverse effects ; pharmacokinetics ; pharmacology ; therapeutic use ; Blood Coagulation Disorders ; chemically induced ; Humans ; Pancreatitis ; chemically induced ; Polyethylene Glycols ; adverse effects ; pharmacokinetics ; pharmacology ; therapeutic use ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy

OBJECTIVETo analyze the clinical characteristics and the course of diagnosis and therapy of PEG-asparaginase associated pancreatitis (AAP) in childhood, and to reveal the pathophysiology of AAP, enhance the ability of diagnosis and treament.

METHODData of 13 cases with AAP in childhood seen from March 2011 to March 2014 were analyzed with regard to clinical manifestations, laboratory findings, imaging feature and treatment.

RESULTAAP was found in 12 of acute lymphoblastic leukemia (ALL) and 1 of non-Hodgkin's lymphoma (NHL), 8 were boys and 5 were girls, with a mean age 6 years. In 12 cases AAP occurred during the induction-remission treatment, in 1 case during the maintenance- intensification phase. AAP occurred after a median of two doses, and 9 d (median) from the latest administration of PEG-asparaginase. The major manifestations of AAP was abdominal pain (11/13) . At the time of AAP diagnosis during the first 48 hours the median peak serum amylase and serum lipase levels were 559 U/L (range 118-1 585, upper normal limit: 125) and 934 U/L (range 221-1 673, upper normal limit: 300). Three cases with serum amylase and serum lipase levels above 3 times upper normal limit were repeatedly complicated with pancreatic pseudocyst; 11 patients had abnormal CT imaging, 8 cases revealed effusion around the pancreas, and 4 cases had pseudocyst. Therapy with ulinastatin, octreotide acetate, glucocorticoid could relieve abdominal pain significantly. Three cases underwent abdominal puncture drainage and 5 cases fulfilled nasojejunal nutrition therapy. Nine of them were cured, 4 developed pseudocyst, in 2 AAP vanished gradually and 2 died with pseudocyst.

CONCLUSIONThe major manifestations of AAP were abdominal pain, but sometimes apparent and sometimes latent. Condition of acute pancreatitis may exacerbate rapidly and easily. Early identification had significance. Pancreatic pseudocyst suggested a poor prognosis.

Acute Disease ; Asparaginase ; adverse effects ; therapeutic use ; Child ; Female ; Humans ; Lymphoma, Non-Hodgkin ; drug therapy ; Male ; Pancreatic Pseudocyst ; Pancreatitis ; chemically induced ; diagnosis ; therapy ; Polyethylene Glycols ; adverse effects ; therapeutic use ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy

OBJECTIVETo observe the efficacy of polyethylene glycol conjugated asparaginase (peg-asp) for induction treatment of children with newly diagnosed acute lymphoblastic leukemia (ALL).

METHODA total of 268 newly diagnosed children with ALL enrolled in CCLG-2008 from January, 2010 to August, 2012 were analyzed. Patients received either native Escherichia coli asparaginase or pegaspargase along with multiagent chemotherapy during remission induction treatment. Status of bone marrow aspiration was assessed on day 15, day 33 (M1, M2, M3).

RESULTOf the 268 patients stratified, 37.3% (n = 100) were SR, 32.1% (n = 86) were IR, and 31.6% (n = 82) were HR; 159 patients received native Escherichia coli asparaginase and 109 patients received pegaspargase. Characteristics of two groups in age, sex, blood count at diagnosis, immunophenotype and response to prednisolone had no significant difference (P > 0.05). Bone marrow status on day 15 in pegaspargase group was M1 in 70 (64.2%) cases, M2 in 23 (21.1%) and M3 in 16 (14.7%), while in native Escherichia coli asparaginase group, M1 in 112 (70.4%) cases, M2 in 21 (13.2%) and M3 in 26 (16.4%), respectively (χ(2) = 2.938, P = 0.230). Bone marrow status on day 33 was M1 in 105 (96.3%), M2 in 3 (2.8%) and M3 in 1 (0.9%) in pegaspargase group, while it was M1 in 154 (96.9%) cases, M2 in 5 (3.1%) and M3 in native Escherichia coli asparaginase group, respectively (χ(2) = 1.494, P = 0.474).

CONCLUSIONDomestic pegaspargase of our country can achieve the similar efficacy in induction treatment for ALL patients as compared with native Escherichia coli asparaginase. The drug could be considered as not only the choice for allergic patients but also a first-line alternative for new patients.

Adolescent ; Antineoplastic Agents ; administration & dosage ; adverse effects ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Asparaginase ; administration & dosage ; adverse effects ; Bone Marrow Cells ; drug effects ; pathology ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Polyethylene Glycols ; administration & dosage ; adverse effects ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; pathology ; Prednisone ; administration & dosage ; adverse effects ; Remission Induction ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo analyze the short-term efficacy, overall survival (OS), and safety in newly diagnosed extranodal NK/T-cell lymphoma (ENKTL) patients with L-asparaginase based regimens or CHOP regimen in combination with radiotherapy as first-line treatment.

METHODSOf the total 181 patients diagnosed by imaging and pathology, 69 patients received CHOP regimen and 112 patients received L-asparaginase based regimens. All the patients received radical radiotherapy(RT)after 6 cycles of chemotherapy.

RESULTSThe overall response rates of L-asparaginase-based group and CHOP group were 90.2% and 72.5%, respectively (P=0.002). The 1, 2, 5-year OS and progression-free survival (PFS) in L-asparaginase-based group were 96.0%, 88.3%, 65.1% and 94.2%, 79.8%, 50.0%, respectively. The 1, 2, 5-year OS and PFS in CHOP group were 82.6%, 61.9%, 28.4% and 63.8%, 44.0%, 21.0% (P=0.000).

CONCLUSIONCompared with CHOP regimen, L-asparaginase-based chemotherapy is more effective and safe for newly diagnosed nasal-type ENKTL.

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Asparaginase ; administration & dosage ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Female ; Humans ; Lymphoma, Extranodal NK-T-Cell ; drug therapy ; Male ; Middle Aged ; Prednisone ; therapeutic use ; Retrospective Studies ; Treatment Outcome ; Vincristine ; therapeutic use

Pediatric diffuse large B-cell lymphoma (DLBCL) is a highly aggressive disease with unique clinical characteristics. This study analyzed the germinal-center type B-cell (GCB) classification and clinical characteristics of Chinese pediatric DLBCL. A total of 76 patients with DLBCL newly diagnosed in Sun Yat-sen University Cancer Center between February 2000 and May 2011, with an age younger than 18 years, were included in the analysis. The male/female ratio was 3.47:1. The median age was 12 years (range, 2 to 18 years), and 47 (61.8%) patients were at least 10 years old. Of the 76 patients, 48 (63.2%) had stage III/IV disease, 9 (11.8%) had bone marrow involvement, 1 (1.3%) had central nervous system (CNS) involvement, and 5 (6.6%) had bone involvement. The GCB classification was assessed in 45 patients: 26 (57.8%) were classified as GCB subtype, and 19 (42.2%) were classified as non-GCB subtype. The modified B-NHL-BFM-90/95 regimen was administered to 50 patients, and the 4-year event-free survival (EFS) rate was 85.8%. Among these 50 patients, 31 were assessed for the GCB classification: 17 (54.8%) were classified as GCB subtype, with a 4-year EFS rate of 88.2%; 14 (45.2%) were classified as non-GCB subtype, with a 4-year EFS rate of 92.9%. Our data indicate that bone marrow involvement and stage III/IV disease are common in Chinese pediatric DLBCL patients, whereas the percentage of patients with the GCB subtype is similar to that of patients with the non-GCB subtype. The modified B-NHL-BFM-90/95 protocol is an active and effective treatment protocol for Chinese pediatric patients with DLBCL.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Asparaginase ; therapeutic use ; Child ; Child, Preschool ; Daunorubicin ; therapeutic use ; Disease-Free Survival ; Female ; Follow-Up Studies ; Germinal Center ; pathology ; Humans ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; pathology ; Male ; Prednisone ; therapeutic use ; Survival Rate ; Vincristine ; therapeutic use