1.The 504th case: Multiple lymph node enlargement, renal insufficiency, blindness, and white matter lesions of the brain
Chong WEI ; Zhe ZHUANG ; Yunlong LI ; Xiaoxiao SHI ; Yubing WEN ; Wei CAO ; Siyuan FAN ; Xiao ZHANG ; Yan ZHANG ; Wei ZHANG ; Daobin ZHOU
Chinese Journal of Internal Medicine 2024;63(3):316-320
A 65-year-old male patient was admitted for recurrent lymph node enlargement for 5 years and elevated creatinine for 6 months. This patient was diagnosed with angioimmunoblastic T-cell lymphoma 5 years ago and underwent multiple lines of anti-tumor therapy, including cytotoxic chemotherapy; epigenetic modifying drugs such as chidamide and azacitidine; the immunomodulator lenalidomide; and targeted therapy such as rituximab, a CD20-targeting antibody, and brentuximab vedotin, which targets CD30. Although the tumor was considered stable, multiple virus activation (including BK virus, JC virus, and cytomegalovirus) accompanied by the corresponding organ damage (polyomavirus nephropathy, cytomegalovirus retinitis, and progressive multifocal leukoencephalopathy) occurred during anti-tumor treatment. Anti-tumor therapy was suspended and ganciclovir was used. The serum viral load decreased and organ functions were stabilized. The purpose of this report was to raise clinicians′ awareness of opportunistic virus reactivation during anti-tumor treatment.
2.Type of CEBPA mutations in acute myeloid leukemia and their effect on prognosis
Yueying MAO ; Hao CAI ; Xinxin CAO ; Jun FENG ; Lu ZHANG ; Daobin ZHOU ; Jian LI
Chinese Journal of Hematology 2024;45(6):556-560
Objective:To demonstrate the type of CEBPA gene mutations among patients with acute myeloid leukemia (AML), clinical characteristics, and prognostic effect on patient outcomes.Methods:Demographic data, clinical features, laboratory characteristics, and data about treatment and follow-up of 57 patients with CEBPA mutated AML diagnosed at Peking Union Medical College Hospital between April 2016 and November 2022 were collected and analyzed.Results:In total, 57 patients with CEBPA mutation accounted for 16.1% of all the 353 patients with AML, among which 28 patients had CEBPA-bZIPinf and 29 had CEBPA-other. Compared with the CEBPA-other group, the CEBPA-bZIPinf group was younger (54 vs 64 years, P=0.010), de novo AML was more common ( P=0.001), and the level of bone marrow blast was higher (68.0% vs 36.3%, P=0.001). Moreover, 24 patients from the CEBPA-bZIPinf group and 19 from the CEBPA-other group received chemotherapy. The one-course complete remission (CR) rate of the CEBPA-bZIPinf group was significantly higher than that of the CEBPA-other (87.5% vs 47.4%, P=0.010) and CEBPA-wt (87.5% vs 50.3%, P=0.002) groups. After a median follow-up of 11 months, the median OS of the CEBPA-bZIPinf group was significantly longer than that of the CEBPA-wt group (not reached vs 22.1 months, P=0.012) . Conclusion:CEBPA-bZIPinf mutated AML is a unique clinical entity, with a younger age of diagnosis, better response to chemotherapy, and better prognosis.
3.Radiographic anatomical ratios between tibial plateau and distal femur and the clinical value in evaluating reduction of Schatzker Ⅳ-C tibial plateau fractures
Yulong LIU ; Rende NING ; Run FANG ; Han-Lin ZHENG ; Chengnan ZHANG ; Daobin ZHOU ; Zulong ZHOU
The Journal of Practical Medicine 2024;40(9):1257-1261
Objective To investigate the radiographic anatomical relationship between tibial plateau and distal femur and evaluate the impact of reset tibial plateau of various widths after reduction of the Schatzker Ⅳ-C tibial plateau fractures on postoperative outcomes.Methods We collected and reviewed the X-ray images of the normal knees of 207 standard neutrally-positioned adults(non-fracture group)and pre-and post-operative immediate anterior-posterior X-ray images of the knees of 60 patients with Schatzker Ⅳ-C fractures(fracture group)in our hospital from August 2012 to August 2022.We measured the proximal tibial joint width(TAW),distal femoral width(DFW),and distal femoral joint width(FAW)in both groups and calculated the TAW/DFW and TAW/FAW ratios.In the fracture group,the cases with TAW between FAW and DFW were assigned to the well-reduced group,while those with TAW outside this range between FAW and DFW to the poorly-reduced group.Both groups were assessed using the Hospital for Special Surgery knee score(HSS)one year after operation.Results In the non-fracture group,there were no significant differences in gender or affected side in terms of TAW/DFW and TAW/FAW ratios(P>0.05),while in the fracture group,there were statistically significant differences in the TAW/DFW and TAW/FAW ratios compared to the non-fracture group(P<0.05).There was a statistically signifi-cant difference in the one-year postoperative HSS scores between the well-reduced and poorly-reduced groups in the fracture group(P<0.05).Conclusion The radiographic anatomical relationship between the tibial plateau and distal femur in normal adults is relatively constant,providing a radiological reference for resetting the tibial plateau to a satisfactory width during reduction of Schatzker Ⅳ-C fractures.TAW/DFW>1 or TAW/FAW<1 indicates a poor reduction of the fracture and predicts poor postoperative recovery of knee joint function.
4.Clinical characteristics and prognostic analysis of prolonged cytopenia after CAR-T cell therapy in LBCL patients
Huiying ZHU ; Danqing ZHAO ; Zhe ZHUANG ; Jing RUAN ; Chao CHEN ; Wei ZHANG ; Daobin ZHOU ; Yan ZHANG
Chinese Journal of Internal Medicine 2024;63(12):1238-1245
Objective:To investigate the clinical features and prognosis of prolonged cytopenia (PC) in patients with large B-cell lymphoma (LBCL) undergoing anti-CD19 chimeric antigen receptor T (CAR-T) cell therapy.Methods:A retrospective case series study was conducted on LBCL patients who received CAR-T cell therapy with a survival time of over one month at the Hematology Department of Peking Union Medical College Hospital from March 2019 to December 2023. Statistical analyses were performed on hematologic changes at 1, 3, 6, and 12 months post-CAR-T infusion, as well as on the progression-free survival (PFS) and post-treatment adverse events, including infections. Patients were categorized into the PC and non-PC groups based on the occurrence of cytopenia at 90 days post-infusion. Differences between groups were compared, and univariate logistic regression analysis was used to identify risk factors.Results:The median age of 27 LBCL patients receiving CAR-T cell therapy was 58 years (range 27-69 years), with 18 males. Among the 27 LBCL patients who received CAR-T cell therapy, PC was observed in 19 patients (70.4%), with instances of neutropenia (48.1%, 13 cases), anemia (37.0%, 10 cases), and thrombocytopenia (22.2%, 6 cases). Univariate logistic regression analysis revealed that prior chemotherapy sensitivity ( OR=18.00, 95% CI 1.56-207.45, P=0.020) and bone marrow suppression ( OR=18.00, 95% CI 1.38-235.69, P=0.028) were associated with PC. The median follow-up time was 13.5 months. The PC group exhibited a higher risk of infection within 3 months (9/19 vs. 1/8) and a shorter mean PFS (19.3 months vs. 24.4 months), although the difference was not statistically significant (both P>0.05). Conclusions:PC is common following CAR-T cell therapy and is associated with an increased risk of infection and poorer prognosis. Prior treatment sensitivity and bone marrow suppression may serve as indicators of PC.
5.The diagnosis and risk factors for postoperative acute pancreatitis following pancreaticoduodenectomy
Daobin WANG ; Ding SUN ; Weigang ZHANG ; Zuxiong TANG ; Junyi QIU ; Lei QIN ; Haixin QIAN ; Xiaofeng XUE
Chinese Journal of General Surgery 2022;37(7):487-491
Objective:To study factors associated with postoperative acute pancreatitis (POAP) in patients following pancreaticoduodenectomy.Methods:This retrospective analysis included 60 patients who underwent pancreaticoduodenectomy at the First Affiliated Hospital of Soochow University from Jan 2020 to Aug 2021. Enhanced computed tomography was used to identify POAP during postoperative period of 4 to 9 days. Univariate analysis and multivariate analysis were used to find out the risk factors of POAP.Results:Of the 60 patients, 13 cases (21.7%) developed POAP. The incidence of clinically related pancreatic fistula with abdominal abscess (76.9% vs.19.1%, χ2=15.71, P<0.000 1), postoperative hospital stay (26 d vs. 18 d, U=141.5, P=0.002 5) and the severity of complications (Clavien-Dindo grade≥Ⅲ: 53.8% vs. 21.3%, χ2=5.32, P=0.02) were significantly higher in the POAP group. But there was no significant deviation between the two groups when it comes to the severe post pancreatectomy delayed hemorrhage (7.7% vs. 0, χ2=3.68, P=0.06) and the delayed gastric emptying (30.8% vs. 21.3%, χ2=0.51, P=0.47). In the univariate analysis, patients with higher body mass index ( P=0.000 3), smaller main pancreatic duct diameter ( P<0.000 1) and softer texture of the pancreas ( P=0.009) were more likely to develop POAP after pancreaticoduodenectomy. In the multivariate analysis, the pancreatic duct diameter≤2 mm ( OR=0.005,95% CI 0.000 06-0.44, P=0.020), the softer texture of pancreas ( OR=0.005, 95% CI 0.000 04-0.47, P=0.023) were risk factors for POAP. Conclusions:Patients with postoperative acute pancreatitis increased the incidence of pancreatic fistula complicating abdominal abscess.Small caliber pancreatic tube, soft texture of pancreas were risk factors of POAP.
6.Utility and prognostic value of PET/CT, bone marrow biopsy and aspirate for detection of bone marrow involvement in diffuse large B-cell lymphoma
Xianyong JIANG ; Dongmei ZOU ; Yingqiang ZHANG ; Zi WANG ; Wei ZHANG ; Daobin ZHOU ; Yan ZHANG
Chinese Journal of Hematology 2022;43(8):663-667
Objective:This study aimed to determine the value of bone marrow biopsy (BMB) , bone marrow aspiration (BMA) , and positron emission tomography combined with computed tomography (PET/CT) in bone marrow (BM) involvement and prognosis evaluation in diffuse large B-cell lymphoma (DLBCL) .Methods:The clinical data of patients with DLBCL who underwent PET/CT, BMB, and BMA of the iliac crest were retrospectively analyzed in Peking Union Medical College Hospital from January 2015 to November 2017. The BM involvement on PET/CT was defined as the ratio of maximal standardized uptake values of iliac crest BM to liver parenchyma intensity ≥1.Results:A total of 76 patients without liver involvement were enrolled, there were 32 males, and the median age was 53 (17-79) . Moreover, 16 patients (21.1%) had BM involvement on PET/CT, 12 (15.8%) had positive BMB, and 13 (17.1%) had positive BMA. Excellent correlation between BMA and BMB ( κ=0.943) was found, including good correlation between PET/CT and BMB/BMA ( κ=0.763 and 0.776, respectively) . After a median follow-up of 52 (0-82) months, BM involvement by BMB ( P=0.037) and BMA ( P=0.007) were poor prognostic factors for overall survival, positive PET/CT had no significant effect on prognosis ( P>0.05) . Conclusion:PET/CT, BMB, and BMA are effective methods to detect BM involvement with great concordance. However, iliac crest BMB and BMA showed superior performance in prognosis evaluation.
7.Clinical characteristics and prediction model of early death in severe/very severe aplastic anemia with immunosuppressive therapy
Miao CHEN ; Junling ZHUANG ; Chen YANG ; Wei WANG ; Yan ZHANG ; Lu ZHANG ; Danqing ZHAO ; Jun FENG ; Jian LI ; Daobin ZHOU ; Bing HAN
Chinese Journal of Hematology 2022;43(11):916-920
Objective:Early death (ED) characteristics and predictive factors analysis in patients with severe/very severe aplastic anemia (SAA/VSAA) treated with intensive immunosuppression therapy and establish an ED prediction model.Methods:The clinical data of 232 patients with SAA/VSAA treated with Antithymocyte immunoglobulin (ATG) at the Peking Union Medical College Hospital from August 2003 to August 2021 were collected. The characteristics and causes of ED within 90 days were analyzed retrospectively. Cox proportional hazards model was used to screen the ED risk factors and build a prediction model.Results:Only 19 patients (8.2% ) developed ED with a median time of 24 (3-85) days among the 232 patients with SAA/VSAA who received ATG treatment. The main cause of ED was infection (84.2% ) , followed by cerebral hemorrhage (10.5% ) . Multivariate analysis showed that VSAA ( HR=15.359, 95% CI 1.935-121.899, P=0.010) , fungal infection prevention by posaconazole ( HR=0.147, 95% CI 0.019-1.133, P=0.066) , lymphocyte count (LYM) ≤ 0.5×10 9/L ( HR=3.386, 95% CI 1.123-10.206, P=0.030) , and PLT ≤ 5×10 9/L ( HR=8.939, 95% CI 1.948-41.019, P=0.005) were ED’s independent influencing factors. To build a clinical prediction model, VSAA, fungal infection prevention by posaconazole, LYM ≤ 0.5×10 9/L, and PLT ≤ 5×10 9/L were scored with 3, -2, 1, and 2, respectively. The integral model AUC=89.324 (95% CI 80.859-97.789) . The ED risk in patients with a score ≥ 3 was 23.1 (95% CI 5.3-100.2) times that in patients with a score<3. Conclusion:ED caused by infection and cerebral hemorrhage is an important challenge for SAA/VSAA to be treated with ATG. VSAA, LYM ≤ 0.5×10 9/L, and PLT ≤ 5×10 9/L patients who did not use posaconazole to prevent fungal infection had a high ED risk.
8.Clinical presentation and prognosis in patients with light-chain amyloidosis with an ultra-high level of serum free light-chain
Huilei MIAO ; Kaini SHEN ; Wei SU ; Lu ZHANG ; Xinxin CAO ; Daobin ZHOU ; Jian LI
Chinese Journal of Hematology 2021;42(3):199-204
Objective:To investigate the clinical features and outcomes of patients with light-chain (AL) amyloidosis with an ultra-high level of serum free light-chain (FLC) .Methods:Five hundred and ninety-five patients with AL amyloidosis were retrospectively reviewed between January 2009 and January 2020 at Peking Union Medical College Hospital. We analyzed the clinical features and prognosis of patients with ultra-high FLC levels [difference between involved and uninvolved light chains (dFLC) >500 mg/L; n=124] and those without ultra-high FLC levels (dFLC≤500 mg/L; n=471) . Results:Patients with ultra-high FLC presented with more frequent cardiac involvement (82.3% vs 70.1%, P=0.007) , and a higher percentage of patients with 2004 Mayo Ⅲ stage (41.8% vs 33.8%, P=0.029) , but less frequent renal involvement than patients without an ultra-high FLC (59.7% vs 71.8%, P=0.009) . Patients with an ultra-high FLC achieved a lower proportion of hematologic (72.4% vs 82.3%, P=0.048) and cardiac response (37.3% vs 54.7%, P=0.016) and had shorter overall survival (13.0 months vs not reached, P<0.001) and a higher early death rate within 3 months (28.2% vs 11.3%, P<0.001) than those without an ultra-high FLC. Ultra-high FLC independently predicted worse prognosis in patients with AL amyloidosis ( HR=2.279, 95% CI 1.685-3.083, P<0.001) . Conclusions:Patients with an initially ultra-high FLC represented a subgroup with more common cardiac involvement, more advanced cardiac stages, and extremely poor prognosis.
9.Clinical characteristics and outcome of patients with type Ⅱ cryoglobulinemia
Hongxiao HAN ; Xinxin CAO ; Wei SU ; Kaini SHEN ; Lu ZHANG ; Daobin ZHOU ; Jian LI
Chinese Journal of Hematology 2021;42(6):447-451
Objective:To explore the clinical characteristics and outcome of patients with type Ⅱ cryoglobulinemia in our center.Methods:Clinical data of 61 patients diagnosed with type Ⅱ cryoglobulinemia in Peking Union Medical College hospital from May 2015 to January 2020 were retrospectively analyzed.Results:A total of 61 patients were enrolled in the study, including 26 (42.6%) males, with a median age of 53 (range 28-79) years. The primary diseases identified were hepatitis C virus infection (21.3%) , hepatitis B virus infection (21.3%) , autoimmune diseases (14.8%) , and hematological tumors (11.5%) . Idiopathic cryoglobulinemia patients accounted for 19 cases (31.1%) . The major symptoms presented were purpura, proteinuria, hematuria, renal failure, fever and arthralgia. The median concentration of cryoglobulin level was 215.9 (22.0-17 075.8) g/L, and the IgM-monoclonal component of cryoglobulin was identified in 54 patients (88.5%) . Rheumatoid factor increased in 93.2% of patients. C3 decreased in 57.6% of patients. C4 decreased in 61.0% of patients. Treatment was initiated in 49 patients (80.3%) , and the total clinical remission rate was 75.5%. The expected 3-year overall survival was 89.3%.Conclusion:Type Ⅱ cryoglobulinemia was a systemic disease with multi-organ involvement. Most type Ⅱ CGs were secondary to hepatitis virus infection. Early diagnosis and proper treatment could bring better outcome.
10.A pediatric-inspired regimen for adolescents and young adults with Philadelphia chromosome-negative acute lymphoblastic leukemia: results from a single center
Jun FENG ; Lu ZHANG ; Xinxin CAO ; Yan ZHANG ; Chen YANG ; Huacong CAI ; Miao CHEN ; Wei WANG ; Wei ZHANG ; Tienan ZHU ; Minghui DUAN ; Jian LI ; Daobin ZHOU
Chinese Journal of Hematology 2020;41(5):399-404
Objective:To investigate the efficacy of using a pediatric-inspired regimen for adolescents and young adults (AYA) with Philadelphia chromosome-negative (Ph -) acute lymphoblastic leukemia/lymphoblastic lymphoma (ALL/LBL) at a single center in China. Methods:Clinical data of 71 consecutive newly diagnosed AYA patients with Ph - ALL/LBL on a pediatric-inspired regimen in Peking Union Medical College Hospital from January 2012 to November 2018 were retrospectively analyzed. Results:Median age at diagnosis was 20 years (range: 15-38) , and 46 patients (64.8%) were male. Forty-nine (69.0%) had B-ALL/LBL. Among 62 ALL patients, 22 (35.5%) were high-risk. Complete remission rate was 93.0%. At follow-up with a median time of 44 months, the estimated 5-year disease-free survival (DFS) and overall survival (OS) was 56.3% and 64.3%, respectively. There was no significant difference in 5-year OS between allogeneic hematopoietic stem cell transplantation group and the continuous chemotherapy group after completion of 4 courses of chemotherapy. The 5-year DFS and OS for the non-high-risk group was 63.1% and 73.7%, respectively, which were significantly higher than 32.0% and 44.4% for the high-risk group, respectively ( P<0.001) . Conclusions:The use of pediatric-inspired regimen for AYAs with Ph - ALL/LBL was feasible and effective.

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