Objective To investigate the clinical characteristics and prognostic factors in patients with primary gastric non-Hodgkin lymphoma (PG-NHL).Methods The pathological data of 51 PG-NHL patients admitted in our hospital from 2003 to 2013 were analyzed retrospectively.Results In 51 patients with PG-NHL,there were 26 males and 25 females.The patients' age ranged from 18 to 80 years old with median age as 56 years old.The median survival time was 32 months (range from 1 to 114 months).The oneyear overall survival (OS),three-year OS and five-year OS were 90.2 ％,82.4 ％ and 80.4 ％,respectively.The surgery did not significantly improve PG-NHL patients' progress free survival and OS.Only 1 (2.0 ％) patient had gastrointestinal hemorrhage and perforation after chemotherapy.However,6 (46.2 ％) patients suffered from early satiety,gastric emptying disorder,alkaline reflux gastritis and dumping syndrome in surgery group.Conclusions Surgery did not improve the survival of PG-NHL patients.The life quality in chemotherapygroup is better than that in surgical group.

Objective To assess the status and severity of bone disease in patients with multiple myeloma (MM) by using chest computerized tomography (CT) and the relationship between clinical prognostic parameters and bone disease.Methods All 46 newly diagnosed MM in-patients received both imaging tests of chest CT and plain X ray.An experienced radiologist reviewed all the imaging data.Clinical laboratory parameters,stages of Durie-Salmon (DS) and International Staging System (ISS) were evaluated.Five cytogenetic abnormalities of bone marrow myeloma cells were tested by fluorescence in situ hybridization (FISH).Results The sensitivity of CT and X ray to determine pathological fractures was comparable,the positive rates of which were 41.3％ (19/46) and 30.4％ (14/46) respectively (P =0.29).Nevertheless,the positive rate of osteolytic lesions ascertained by CT was significantly higher than that by X ray (P ＜ 0.001),60.9％ (28/46) vs 13.0％ (6/46) with diameter 5-10 mm and 50.0％ (23/46) vs 10.9％ (5/ 46) with diameter more than 10 mm.Osteolytic lesion numbers found by CT were more than those by X ray [5(0-21) vs0(0-4) lesions with diameter5-10 mm (P＜0.001),2(0-14) vs0(0-2) lesions with diameter more than 10 mm (P ＜ 0.001),respectively].Patients with positive osteolytic lesions had higher percentage of RB1 gene deletion[46.7％ (14/30) vs 18.8％ (3/16),P ＜0.001],D13s319 deletion [43.3％ (13/30) vs 18.8％ (3/16),P ＜0.001] and high risk cytogenetic abnormalities[50.0％ (15/30) vs 25.0％ (4/16),P ＜ 0.001].Conclusions Chest CT is more sensitive than plain X ray in detecting osteolytic myeloma bone disease.Osteolysis determined by CT is relevant to clinical DS stages and risk stratification of cytogenetic abnormalities.

OBJECTIVETo explore the high risk factors of thrombosis in paroxysmal nocturnal hemoglobinuria (PNH). It has been reported that in Chinese patients with venous thrombosis, the mutation frequency in PROC c.574_576 del (rs199469469), PROC c.565C>T (rs146922325) and THBD c.-151G>T (rs1698852) was higher than that of normal controls, indicating its importance in thrombophilia pathogenesis.

METHODS142 patients with PNH diagnosed between 2009 and 2015 were enrolled in the study. Clinical data were analyzed and thrombophilia risk factors, such as the level of protein C, protein S, antithrombin III, APC resistance, blood fat, phospholipid antibody, were evaluated. Samples from patients and 100 normal controls were detected for the mutations of PROC c.574_576 del (rs199469469), PROC c.565C>T (rs146922325) and THBD c.-151G>T (rs1698852) by Sanger sequence.

RESULTSOf the 142 PNH patients, 21 (14.8%) patients had at least 1 episode of thrombotic event. Only 2 patients had arterial thrombosis and 19 patients had venous thrombosis. The median age of patients with thrombosis was 35 years old, similar to those without episode (40 years old, P=0.687). The ratios of males and females were 1.33 in thrombosis group and 1.57 in non-thrombosis group (P=0.728) , respectively. Patients with thrombosis had the same disease pattern compared with those without episode. Although there was no difference in the level of hemoglobin, WBC and PLT count, and LDH level between patients with thrombosis and those without episode, patients with thrombosis showed higher RBC, higher percentage of CD59(-) granulocytes and RBC, and Flaer(-) granulocytes compared with those without episode. The routine thrombophilia screening tests did not show any difference either between PNH patients and normal controls, or between patients with or without thrombosis. There were two mutations in rs199469469 and rs16984852 sites in patients with PNH, but the mutated patients did not have any thrombosis. Mutation rs146922325 was found in PNH patients. The mutation rate was similar between PNH patients and normal controls, thrombotic PNH and non-thrombotic PNH (P>0.05).

CONCLUSIONSCompared with non-thrombotic patients, PNH thrombotic patients have bigger PNH clone and higher RBC count. There are no differences among the routine thrombophilia factors and the three known venous eligible genes either between PNH patients and normal controls or between thrombotic and non-thrombotic PNH patients.

Adult ; Antithrombin III ; metabolism ; Case-Control Studies ; Clone Cells ; cytology ; Female ; Granulocytes ; cytology ; Hemoglobinuria, Paroxysmal ; genetics ; physiopathology ; Humans ; Leukocyte Count ; Male ; Protein C ; metabolism ; Protein S ; metabolism ; Risk Factors ; Thrombosis ; genetics ; physiopathology

Objective: To assess the value of immunoglobulin heavy/light chain (HLC) immunoassay on therapeutic response in patients with multiple myeloma(MM). Methods: A total of 45 newly diagnosed MM patients were retrospectively enrolled in Peking Union Medical College Hospital from 2013 to 2016, whose 115 serum samples were consecutively collected. HLC was tested to evaluate response and compare with other methods for M protein detection. Results: ①There were 30 males and 15 females in total of whom the monoclonal immunoglobulin was IgG in 27 (IgGκ∶IgGλ 12∶15) and IgA (IgAκ∶IgAλ 9∶9) in 18. The arerage age of the studied population was 59 (range 43-80) . ② In 34 patients with serum sample at diagnosis, 32 (94.1%) had abnormal HLC ratio (rHLC) while 2 patients with IgG had normal rHLC. The percentages of abnormal rHLC was 81.8% (18/22) at partial response、50.0%(9/18) at very good complete response and 16.0%(4/25) at complete response. ③In 25 patients reaching CR, there were 13 with IgG and 12 with IgA. 4 patients equally split of IgG and IgA had abnormal rHLC at complete response. ④By monitoring the rHLC of some patients consecutively, we found that the remission of rHLC was to some extent behind the remission of SPE and IEF, or even rFLC. Conclusion Immunoglobulin HLC detection is one feasible method for minimal residual disease detection.

Objective:To investigate the living status and quality of life (QOL) in type1 Gaucher disease (GD1) patients who underwent long-term enzyme replacement therapy (ERT) and identify possible relevant factors affecting QOL.Methods:Clinical data and SF-36 questionnaires were recorded in 22 adult GD1 patients under regular ERT at Peking Union Medical Colleague Hospital (PUMCH) from January 1995 to June 2017.Results:13 males and 9 females were included in this study. The current median age, age at diagnosis and initial time of ERT were 41 (24-52) , 6 (1-38) and 26 (6-41) years respectively. Of these patients, 68.2% was living in less-developed regions, 86.4% were under college education, and 77.3% had personal annual income less than ￥30 000 RMB. Though after a median 16 (7-22) years of ERT, the QOL of GD1 patients was still significantly worse ( P<0.05) compared with normal Chinese population based on SF-36 questionnaires. History of splenectomy was a negative factor of QOL, mainly in physical health ( P<0.05) . Patients could get benefit from early start of ERT in both physical and mental health ( P<0.05) . Mental health was not affected by history of splenectomy and related bone diseases. Conclusion:Most adult GD1 patients at PUMCH reside in less-developed regions and have low levels of education and annual income. History of splenectomy and time to start ERT are two important factors affecting QOL. Chinese adult GD1 patients are associated with reduced QOL, even after long-term ERT.

A young man with a history of thrombocytopenia for seven years presented with splenomegaly and fever and rapidly evolved to disseminated intravascular coagulation (DIC) and hemorrhagic shock. Spontaneous rupture of the spleen was diagnosed. The critical patient underwent an emergency splenectomy. Pathological examination revealed splenic peliosis, an extremely rare disease with unknown etiology and pathogenesis. Despite the high mortality rate due to spontaneous splenic rupture with DIC, the patient was successfully treated and the details of the case are presented in this report.

A 42-year-old woman was diagnosed with Waldenstr?m macroglobulinemia (WM) with fatigue, anemia, and monoclonal IgM immunoglobulinemia 6 years prior. She experienced persistent severe anemia with only transient remission after initial chemotherapy and after multiple chemotherapy regimens and immunosuppressive therapies, which were accompanied by recurrent high fever with severe complications including urinary infection, sepsis and shock, rectal perforation, and severe obstructive jaundice. The anemia was diagnosed as warm autoimmune hemolytic anemia and aplastic crisis with inflammation anemia. She received ibrutinib 140 mg once a day, and her hemoglobin levels returned to normal. WM remained stable in very good partial remission with no infection.

Objective To evaluate the safety and efficacy of lenalidomide plus rituximab in treatment of the patients with relapsed/refractory B-cell non-Hodgkin lymphoma (B-NHL). Methods The clinical data of the patients with relapsed/refractory B-NHL after the varieties of treatment methods in Peking Union Medical College Hospital between January 2015 and December 2017 were retrospectively analyzed. All the patients were treated with R2 regimen: oral lenalidomide (25 mg/d for day 1-day 21) and rituximab (375 mg/m2 of intravenous infusion on day 1, 28-day of each cycle); the efficacy was evaluated after three cycles. After this induction phase, the patients achieving complete response (CR), partial response (PR), or stable disease (SD) were given R2 regimen until the end of 8 cycles. The major end point was overall response rate (ORR) defined as CR + PR. Secondary end point included 1-year progression free survival (PFS), 1-year overall survival (OS) and grade 3-4 adverse events. T cell and B cell subsets of 7 patients at baseline were measured, and T cell and B cell subsets of 13 patients with good efficacy were dynamically observed. Results A total of 49 patients who received 1-4 chemotherapy regimens were included. The ORR after the R2 treatment for 3 courses was 65% (32/49). Thirty-six patients (9 cases of CR, 22 cases of PR, 5 cases of SD) were enrolled in R2 maintenance treatment. The median follow-up time was 13 months, 1-year PFS rate was 61% and 1-year OS rate was 84% . The most common adverse event was bone marrow suppression, including grade 3-4 neutropenia (27% ), grade 3-4 thrombocytopenia (6% ) and grade 4 anemia (4% ), most of which could be controlled by prolonging interval cycles or reduced lenalidomide dosage. The decreased number of CD19+B cell after treatment could be seen in 13 patients who obtained good efficacy under the dynamic observation. Conclusion Lenalidomide plus rituximab is well tolerated and highly active in the treatment of relapsed/refractory B-NHL.

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm (MPN) featured by clonal proliferation of platelets, thrombosis and hemorrhage. Portal hypertension is a serious complication of ET associated with poor prognosis. We report a patient with ET complicated with acute upper gastrointestinal hemorrhage and intestinal perforation due to portal hypertension. She had an uneventful recovery after surgical and endoscopic treatment.

Objective To evaluate the safety and efficacy of Hyper-CVAD intensive chemotherapy regimen in patients with newly diagnosed aggressive T-cell lymphoma. Methods The efficacy, side effects and survival status were retrospectively analyzed in 34 patients with newly diagnosed aggressive T-cell lymphoma who received Hyper-CVAD regimen as induction therapy in Peking Union Medical College Hospital from September 2009 to December 2010. Results Of 34 patients, 28 cases (82.4 ％) showed treatment response, including 10 cases (29.4 ％) of complete response (CR). Eleven patients underwent stem cell transplantation, including 1 case of human leukocyte antigen-identical siblings allogeneic stem cell transplantation. The median follow-up was 16 months (1-82 months), and the overall survival (OS) rate of 1 or 3-year was 70.2 ％ and 41.1 ％ respectively, and progression-free survival (PFS) rate of 1 or 3-year was 49.3 ％ and 31.6 ％ respectively. The major adverse reaction was myelosuppresion, including 18 cases (52.9％) of myelosuppresion with grade Ⅳ. Three patients died of serious infection. Cox regression multifactor analysis showed CR was the only influencing factor for PFS (HR=6.118, 95％CI 1.327-28.206, P=0.020). Marrow involvement (HR= 0.270, 95 ％CI 0.101-0.722, P= 0.009) and CR (HR= 6.669, 95 ％CI 1.754-25.354, P= 0.005) were independent influencing factors for OS. Conclusions Hyper-CVAD regimen has a high response rate for aggressive T-cell lymphoma, but the lasting effectiveness and the short-term efficacy show unfavorable performances. Meanwhile, myelosuppression is serious and infection incidence is high. Autologous hematopoietic stem-cell transplantation after remission may improve the outcome.