1.Clinicopathologic characteristics and prognostic analysis of testicular diffuse large B-cell lymphoma.
Yue WANG ; Zi Yang SHI ; Qing SHI ; Shuo WANG ; Mu Chen ZHANG ; Rong SHEN ; Yang HE ; Hui Ling QIU ; Hong Mei YI ; Lei DONG ; Li WANG ; Shu CHENG ; Peng Peng XU ; Wei Li ZHAO
Chinese Journal of Hematology 2023;44(4):321-327
		                        		
		                        			
		                        			Objective: To analyze the clinicopathologic characteristics and prognosis of testicular diffuse large B-cell lymphoma (DLBCL) . Methods: A retrospective analysis was performed on 68 patients with testicular DLBCL admitted to Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine from October 2001 to April 2020. The gene mutation profile was evaluated by targeted sequencing (55 lymphoma-related genes) , and prognostic factors were analyzed. Results: A total of 68 patients were included, of whom 45 (66.2% ) had primary testicular DLBCL and 23 (33.8% ) had secondary testicular DLBCL. The proportion of secondary testicular DLBCL patients with Ann Arbor stage Ⅲ-Ⅳ (P<0.001) , elevated LDH (P<0.001) , ECOG score ≥ 2 points (P=0.005) , and IPI score 3-5 points (P<0.001) is higher than that of primary testicular DLBCL patients. Sixty-two (91% ) patients received rituximab in combination with cyclophosphamide, adriamycin, vincristine, and prednisone (R-CHOP) -based first-line regimen, whereas 54 cases (79% ) underwent orchiectomy prior to chemotherapy. Patients with secondary testicular DLBCL had a lower estimated 5-year progression-free survival (PFS) rate (16.5% vs 68.1% , P<0.001) and 5-year overall survival (OS) rate (63.4% vs 74.9% , P=0.008) than those with primary testicular DLBCL, and their complete remission rate (57% vs 91% , P=0.003) was also lower than that of primary testicular DLBCL. The ECOG scores of ≥2 (PFS: P=0.018; OS: P<0.001) , Ann Arbor stages Ⅲ-Ⅳ (PFS: P<0.001; OS: P=0.018) , increased LDH levels (PFS: P=0.015; OS: P=0.006) , and multiple extra-nodal involvements (PFS: P<0.001; OS: P=0.013) were poor prognostic factors in testicular DLBCL. Targeted sequencing data in 20 patients with testicular DLBCL showed that the mutation frequencies of ≥20% were PIM1 (12 cases, 60% ) , MYD88 (11 cases, 55% ) , CD79B (9 cases, 45% ) , CREBBP (5 cases, 25% ) , KMT2D (5 cases, 25% ) , ATM (4 cases, 20% ) , and BTG2 (4 cases, 20% ) . The frequency of mutations in KMT2D in patients with secondary testicular DLBCL was higher than that in patients with primary testicular DLBCL (66.7% vs 7.1% , P=0.014) and was associated with a lower 5-year PFS rate in patients with testicular DLBCL (P=0.019) . Conclusion: Patients with secondary testicular DLBCL had worse PFS and OS than those with primary testicular DLBCL. The ECOG scores of ≥2, Ann Arbor stages Ⅲ-Ⅳ, increased LDH levels, and multiple extra-nodal involvements were poor prognostic factors in testicular DLBCL. PIM1, MYD88, CD79B, CREBBP, KMT2D, ATM, and BTG2 were commonly mutated genes in testicular DLBCL, and the prognosis of patients with KMT2D mutations was poor.
		                        		
		                        		
		                        		
		                        			Male
		                        			;
		                        		
		                        			Adult
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Prognosis
		                        			;
		                        		
		                        			Retrospective Studies
		                        			;
		                        		
		                        			Myeloid Differentiation Factor 88
		                        			;
		                        		
		                        			China/epidemiology*
		                        			;
		                        		
		                        			Testicular Neoplasms/drug therapy*
		                        			;
		                        		
		                        			Cyclophosphamide
		                        			;
		                        		
		                        			Rituximab/therapeutic use*
		                        			;
		                        		
		                        			Lymphoma, Large B-Cell, Diffuse/drug therapy*
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Doxorubicin/therapeutic use*
		                        			;
		                        		
		                        			Vincristine/therapeutic use*
		                        			;
		                        		
		                        			Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
		                        			;
		                        		
		                        			Immediate-Early Proteins/therapeutic use*
		                        			;
		                        		
		                        			Tumor Suppressor Proteins
		                        			
		                        		
		                        	
2.Dose-adjusted EPOCH-R vs. R-CHOP in frontline management of Waldeyer's ring diffuse large B-cell lymphoma: a retrospective study from a single institution.
Yuanzheng LIANG ; Xindi LIU ; Jing YANG ; Henan WANG ; Yingshi PIAO ; Liqiang WEI ; Liang WANG
Chinese Medical Journal 2023;136(2):167-175
		                        		
		                        			BACKGROUND:
		                        			To compare the efficacy and safety of dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin plus rituximab (DA-EPOCH-R) with standard rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) in Waldeyer's ring diffuse large B-cell lymphoma (WR-DLBCL) at a single institution.
		                        		
		                        			METHODS:
		                        			This retrospective study included 115 newly diagnosed patients with WR-DLBCL, of whom 68 patients received R-CHOP, and 47 patients received DA-EPOCH-R as their first-line treatment. The baseline features of the two groups were well balanced using a 1:1 propensity score matching method, and a total of 84 cases were obtained, including respective 42 cases in the R-CHOP and DA-EPOCH-R groups, for further survival and prognosis analysis. The primary objectives included progression-free survival (PFS) and overall survival (OS).
		                        		
		                        			RESULTS:
		                        			During a median follow-up of 45 months, there were nine (21.4%) deaths in the R-CHOP group and two (4.8%) in the DA-EPOCH-R group. Kaplan-Meier analysis showed statistically significant improvements in PFS and OS in patients with DA-EPOCH-R compared with those treated with R-CHOP (log-rank test, P  = 0.025 and P  = 0.035, respectively). The 2-year PFS and OS rates in the DA-EPOCH-R group were 90.1% (95% confidence interval [CI]: 81.4-99.8%) and 95.2% (95% CI: 89.0-100.0%), respectively, and 80.5% (95% CI: 69.3-93.6%) and 90.5% (95% CI: 52.8-99.8%) in the R-CHOP group. Patients without B symptoms and elevated lactate dehydrogenase levels had a higher PFS in the DA-EPOCH-R group, with P values of 0.038 (hazard ratio [HR]: 0.11; 95% CI: 0.01-0.88) and 0.042 (HR: 0.19; 95% CI: 0.04-0.94), respectively. There were no statistically significant differences in clinical responses and treatment-related toxicities between the two groups.
		                        		
		                        			CONCLUSION
		                        			Compared with patients received R-CHOP, those treated by DA-EPOCH-R had superior PFS, OS, and controlled toxicity in patients with WR-DLBCL.
		                        		
		                        		
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Rituximab/therapeutic use*
		                        			;
		                        		
		                        			Vincristine/therapeutic use*
		                        			;
		                        		
		                        			Retrospective Studies
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Etoposide/therapeutic use*
		                        			;
		                        		
		                        			Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
		                        			;
		                        		
		                        			Lymphoma, Large B-Cell, Diffuse/drug therapy*
		                        			;
		                        		
		                        			Cyclophosphamide/therapeutic use*
		                        			;
		                        		
		                        			Doxorubicin/therapeutic use*
		                        			
		                        		
		                        	
3.Clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis: a retrospective analysis.
Rui-Yan WANG ; Hui CHEN ; Zhi-Xin HUANG ; Yong CHEN ; Jian-Min ZHONG
Chinese Journal of Contemporary Pediatrics 2023;25(10):1034-1039
		                        		
		                        			OBJECTIVES:
		                        			To investigate the clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis (OMG).
		                        		
		                        			METHODS:
		                        			A retrospective analysis was conducted on 130 children with OMG who were treated in the Department of Neurology, Jiangxi Children's Hospital, from February 2018 to February 2023. According to the treatment regimen, they were divided into four groups: glucocorticoid (GC) group (n=29), mycophenolate mofetil (MMF) group (GC+MMF; n=33), methotrexate (MTX) group (GC+MTX; n=30), and tacrolimus (FK506) group (GC+FK506; n=38). Treatment outcomes and adverse reactions were compared among the groups.
		                        		
		                        			RESULTS:
		                        			After 3 months of treatment, the FK506 group had significantly lower scores of Myasthenia Gravis Quantitative Scale and Myasthenia Gravis-Specific Activities of Daily Living than the other three groups (P<0.05). After 3 months of treatment, the FK506 group had a significantly lower dose of prednisone than the GC group, and after 6 and 9 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower dose of prednisone than the GC group (P<0.05). After 12 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower incidence rate of GC-related adverse reactions than the GC group (P<0.05).
		                        		
		                        			CONCLUSIONS
		                        			For children with OMG, the addition of various immunosuppressants can reduce the dosage of GC and adverse reactions. Among them, FK506 shows superior efficacy compared to other immunosuppressants in the early treatment of OMG.
		                        		
		                        		
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Child
		                        			;
		                        		
		                        			Prednisone/adverse effects*
		                        			;
		                        		
		                        			Tacrolimus/adverse effects*
		                        			;
		                        		
		                        			Retrospective Studies
		                        			;
		                        		
		                        			Activities of Daily Living
		                        			;
		                        		
		                        			Immunosuppressive Agents/adverse effects*
		                        			;
		                        		
		                        			Myasthenia Gravis/drug therapy*
		                        			;
		                        		
		                        			Glucocorticoids/therapeutic use*
		                        			;
		                        		
		                        			Mycophenolic Acid/adverse effects*
		                        			
		                        		
		                        	
4.Expression of IGLL1 Gene and Its Clinical Significance in Pediatric T-ALL.
Shui-Yan WU ; Xin-Ran CHU ; Qi JI ; Xiao-Chen LIN ; Zhen-Jiang BAI ; Jian-Qin LI ; Jian PAN ; Zi-Xing CHEN ; Shao-Yan HU
Journal of Experimental Hematology 2023;31(4):999-1004
		                        		
		                        			OBJECTIVE:
		                        			To detect the relative expression of IGLL1 (immunoglobulin lambda-like polypeptide 1) mRNA in bone marrow of children with T-cell acute lymphoblastic leukemia (T-ALL), and analyze its correlation with the clinical characteristics and prognosis of the patients, so as to clarify the clinical significance of IGLL1 in pediatric T-ALL patients.
		                        		
		                        			METHODS:
		                        			A total of 56 pediatric T-ALL patients hospitalized in Children's Hospital of Soochow University from June 2012 to December 2017 and treated with CCLG-ALL 2008 regimen were selected. Transcriptome sequencing technology was used to detect the transcription level of IGLL1 gene in children with T-ALL. According to 25% of the IGLL1 transcription level (cutoff value:448), the enrolled children were divided into IGLL1 low expression group (17 cases) and IGLL1 high expression group (39 cases). Combined with clinical data, the correlation between the expression level of IGLL1 and prognosis of the patients was analyzed.
		                        		
		                        			RESULTS:
		                        			The comparative analysis showed that the transcription level of IGLL1 was not correlated with the clinical characteristics of the patients, such as sex, age, bone marrow blast, white blood cell (WBC) count at initial diagnosis. The 5-year OS rate of patients with high IGLL1 expression was significantly higher than that of patients with low IGLL1 expression (76.9%±6.7% vs 47.1%±12.1%, P =0.018). Further comparison of relapse-free survival (RFS) rate between the two groups showed that the 5-year RFS rate of patients with high IGLL1 expression was higher than that of patients with low IGLL1 expression, but the difference between the two groups was not statistically significant (P =0.095). Multivariate COX analysis was conducted on common clinical prognostic factors (age, sex, WBC count at diagnosis, prednisone response on the 7th day, bone marrow response on the 15th day after treatment) and IGLL1 expression level, and the results showed that IGLL1 expression (P =0.012) and prednisone response (P =0.017) were independent risk factors for overall survival in pediatric T-ALL patients.
		                        		
		                        			CONCLUSION
		                        			In pediatric T-ALL, the OS rate of children with high expression of IGLL1 gene was significantly higher than that of children with low expression of IGLL1 gene, and the expression level of IGLL1 gene was an independent factor affecting the survival of children with T-ALL, which suggests that IGLL1 is a marker of good clinical prognosis of children with T-ALL.
		                        		
		                        		
		                        		
		                        			Child
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
		                        			;
		                        		
		                        			Clinical Relevance
		                        			;
		                        		
		                        			Disease-Free Survival
		                        			;
		                        		
		                        			Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/genetics*
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Prognosis
		                        			;
		                        		
		                        			Recurrence
		                        			;
		                        		
		                        			Immunoglobulin Light Chains, Surrogate/genetics*
		                        			
		                        		
		                        	
5.Composite B-cell and T-cell lymphomas: clinical, pathological, and molecular features of three cases and literature review.
Xueli JIN ; Hui LIU ; Jing LI ; Xibin XIAO ; Xianggui YUAN ; Panpan CHEN ; Boxiao CHEN ; Yun LIANG ; Fengbo HUANG
Journal of Zhejiang University. Science. B 2023;24(8):711-722
		                        		
		                        			
		                        			Composite lymphoma (CL) involving B-cell lymphoma and T-cell lymphoma is extremely rare. Herein, we report three such cases using immunohistochemistry, flow cytometry, and the next-generation sequencing (NGS) to identify the pathological and molecular characteristics of CL. In the first case, the patient was admitted to hospital for generalized pruritic maculopapular rash over the whole body. An excisional biopsy of the skin lesions showed T-cell lymphoma. At the same time, the staging bone marrow (BM) biopsy revealed a diffuse large B-cell lymphoma (DLBCL). After R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapies, the patient produced a good response with substantial dissipation of the rashes and relief of skin. The other two patients were admitted to hospital due to lymphadenopathy and were diagnosed with DLBCL and follicular lymphoma (FL) after core needle biopsy of lymph nodes, BM biopsy, BM aspiration, and flow cytometry. Following R-CHOP and R-COP (rituximab, cyclophosphamide, vincristine, and prednisone) therapies, they achieved complete remission unconfirmed (CRu) and complete remission (CR). However, one or two years later, they suffered a relapse of lymphadenopathy. The shocking fact was that re-biopsy of lymphadenopathy revealed peripheral T-cell lymphoma (PTCL) and angioimmunoblastic T-cell lymphoma (AITL). NGS findings identified DNA methyltransferase 3a (DNMT3a), isocitrate dehydrogenase 2 (IDH2), Ras homolog gene family, member A (RHOA), splicing factor 3B subunit 1 (SF3B1), and tumor protein p53 (TP53) mutations. After immunochemotherapy, these patients achieved CRu and CR again. Nevertheless, they suffered a second relapse of T-cell lymphoma. Finally, they died due to progression of disease. We found that the occurrence of CL is associated with Epstein-Barr virus infection and DNMT3a, IDH2, and TP53 mutations, and the prognosis of the disease is closely related to the T-cell lymphoma components.
		                        		
		                        		
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Rituximab/therapeutic use*
		                        			;
		                        		
		                        			Vincristine/therapeutic use*
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Epstein-Barr Virus Infections/drug therapy*
		                        			;
		                        		
		                        			Herpesvirus 4, Human
		                        			;
		                        		
		                        			Neoplasm Recurrence, Local
		                        			;
		                        		
		                        			Lymphoma, T-Cell/drug therapy*
		                        			;
		                        		
		                        			Cyclophosphamide/therapeutic use*
		                        			;
		                        		
		                        			Lymphoma, Large B-Cell, Diffuse/pathology*
		                        			;
		                        		
		                        			Doxorubicin/therapeutic use*
		                        			;
		                        		
		                        			Lymphadenopathy/drug therapy*
		                        			;
		                        		
		                        			Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
		                        			
		                        		
		                        	
6.A case of duodenal ulcer as prominent manifestation of IgG4-related disease.
Min FENG ; Zhe CHEN ; Yong Jing CHENG
Journal of Peking University(Health Sciences) 2023;55(6):1125-1129
		                        		
		                        			
		                        			A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported. A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months. Four years ago, the patient went to hospital for cutaneous pruritus and abdominal pain. Serum IgG4 was 3.09 g/L (reference value 0-1.35 g/L), alanine aminotransferase 554 U/L (reference value 9-40 U/L), aspartate aminotransferase 288 U/L (reference value 5-40 U/L), total bilirubin 54.16 μmol/L (reference value 2-21 μmol/L), and direct bilirubin 29.64 μmol/L (reference value 1.7-8.1 μmol/L) were all elevated. The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling, common bile duct stenosis, and secondary obstructive dilation of the biliary system. The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily. As jaundice and abdominal pain improved, prednisone was gradually reduced to medication discontinuation. Two months ago, the patient developed melena, whose blood routine test showed severe anemia, and gastrointestinal bleeding was diagnosed. The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals. Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer. After treatment with omeprazole, the fecal occult blood was still positive. The PET-CT examination was performed, and it revealed no abnormality in the metabolic activity of the duodenal wall, and no neoplastic lesions were found. IgG4-related disease was considered, and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment. The patient had a right submandibular gland mass resection history and diabetes mellitus. After the patient was admitted to the hospital, the blood test was reevaluated. The serum IgG4 was elevated at 5.44 g/L (reference value 0.03-2.01 g/L). Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened, with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels. We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4. Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue, the number of IgG4-positive cells was 20-30 cells per high-powered field, and the ratio of IgG4/IgG-positive plasma cells was more than 40%. The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide, and then the duodenal ulcer was healed. IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis. It is a systemic disease that affects nearly every anatomic site of the body, usually involving multiple organs and diverse clinical manifestations. The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers. This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.
		                        		
		                        		
		                        		
		                        			Aged
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			Abdominal Pain/drug therapy*
		                        			;
		                        		
		                        			Acute Disease
		                        			;
		                        		
		                        			Bilirubin
		                        			;
		                        		
		                        			Duodenal Ulcer/etiology*
		                        			;
		                        		
		                        			Immunoglobulin G
		                        			;
		                        		
		                        			Immunoglobulin G4-Related Disease/diagnosis*
		                        			;
		                        		
		                        			Pancreatitis/drug therapy*
		                        			;
		                        		
		                        			Positron Emission Tomography Computed Tomography
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Pruritus/drug therapy*
		                        			
		                        		
		                        	
7.Pituitary immune-related adverse events induced by programmed cell death protein 1 inhibitors in advanced lung cancer patients: A report of 3 cases.
Yang Chun GU ; Ying LIU ; Chao XIE ; Bao Shan CAO
Journal of Peking University(Health Sciences) 2022;54(2):369-375
		                        		
		                        			
		                        			Pituitary immune-related adverse events induced by programmed cell death protein 1 inhibitors in advanced lung cancer patients: A report of 3 cases SUMMARY Programmed cell death protein 1 (PD-1) and its ligand 1 (PD-L1) have been widely used in lung cancer treatment, but their immune-related adverse events (irAEs) require intensive attention. Pituitary irAEs, including hypophysitis and hypopituitarism, are commonly induced by cytotoxic T lymphocyte antigen 4 inhibitors, but rarely by PD-1/PD-L1 inhibitors. Isolated adrenocorticotropic hormone(ACTH) deficiency (IAD) is a special subtype of pituitary irAEs, without any other pituitary hormone dysfunction, and with no enlargement of pituitary gland, either. Here, we described three patients with advanced lung cancer who developed IAD and other irAEs, after PD-1 inhibitor treatment. Case 1 was a 68-year-old male diagnosed with metastatic lung adenocarcinoma with high expression of PD-L1. He was treated with pembrolizumab monotherapy, and developed immune-related hepatitis, which was cured by high-dose methylprednisolone [0.5-1.0 mg/(kg·d)]. Eleven months later, the patient was diagnosed with primary gastric adenocarcinoma, and was treated with apatinib, in addition to pembrolizumab. After 17 doses of pembrolizumab, he developed severe nausea and asthenia, when methylprednisolone had been stopped for 10 months. His blood tests showed severe hyponatremia (121 mmol/L, reference 137-147 mmol/L, the same below), low levels of 8:00 a.m. cortisol (< 1 μg/dL, reference 5-25 μg/dL, the same below) and ACTH (2.2 ng/L, reference 7.2-63.3 ng/L, the same below), and normal thyroid function, sex hormone and prolactin. Meanwhile, both his lung cancer and gastric cancer remained under good control. Case 2 was a 66-year-old male with metastatic lung adenocarcinoma, who was treated with a new PD-1 inhibitor, HX008, combined with chemotherapy (clinical trial number: CTR20202387). After 5 months of treatment (7 doses in total), his cancer exhibited partial response, but his nausea and vomiting suddenly exacerbated, with mild dyspnea and weakness in his lower limbs. His blood tests showed mild hyponatremia (135 mmol/L), low levels of 8:00 a.m. cortisol (4.3 μg/dL) and ACTH (1.5 ng/L), and normal thyroid function. His thoracic computed tomography revealed moderate immune-related pneumonitis simultaneously. Case 3 was a 63-year-old male with locally advanced squamous cell carcinoma. He was treated with first-line sintilimab combined with chemotherapy, which resulted in partial response, with mild immune-related rash. His cancer progressed after 5 cycles of treatment, and sintilimab was discontinued. Six months later, he developed asymptomatic hypoadrenocorticism, with low level of cortisol (1.5 μg/dL) at 8:00 a.m. and unresponsive ACTH (8.0 ng/L). After being rechallenged with another PD-1 inhibitor, teslelizumab, combined with chemotherapy, he had pulmonary infection, persistent low-grade fever, moderate asthenia, and severe hyponatremia (116 mmol/L). Meanwhile, his blood levels of 8:00 a.m. cortisol and ACTH were 3.1 μg/dL and 7.2 ng/L, respectively, with normal thyroid function, sex hormone and prolactin. All of the three patients had no headache or visual disturbance. Their pituitary magnetic resonance image showed no pituitary enlargement or stalk thickening, and no dynamic changes. They were all on hormone replacement therapy (HRT) with prednisone (2.5-5.0 mg/d), and resumed the PD-1 inhibitor treatment when symptoms relieved. In particular, Case 2 started with high-dose prednisone [1 mg/(kg·d)] because of simultaneous immune-related pneumonitis, and then tapered it to the HRT dose. His cortisol and ACTH levels returned to and stayed normal. However, the other two patients' hypopituitarism did not recover. In summary, these cases demonstrated that the pituitary irAEs induced by PD-1 inhibitors could present as IAD, with a large time span of onset, non-specific clinical presentation, and different recovery patterns. Clinicians should monitor patients' pituitary hormone regularly, during and at least 6 months after PD-1 inhibitor treatment, especially in patients with good oncological response to the treatment.
		                        		
		                        		
		                        		
		                        			Adenocarcinoma of Lung/drug therapy*
		                        			;
		                        		
		                        			Adrenocorticotropic Hormone/therapeutic use*
		                        			;
		                        		
		                        			Aged
		                        			;
		                        		
		                        			B7-H1 Antigen/therapeutic use*
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Hydrocortisone/therapeutic use*
		                        			;
		                        		
		                        			Hyponatremia/drug therapy*
		                        			;
		                        		
		                        			Hypopituitarism/drug therapy*
		                        			;
		                        		
		                        			Immune Checkpoint Inhibitors
		                        			;
		                        		
		                        			Lung Neoplasms/pathology*
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			Methylprednisolone/therapeutic use*
		                        			;
		                        		
		                        			Middle Aged
		                        			;
		                        		
		                        			Nausea/drug therapy*
		                        			;
		                        		
		                        			Pituitary Gland/pathology*
		                        			;
		                        		
		                        			Pneumonia
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Programmed Cell Death 1 Receptor/therapeutic use*
		                        			;
		                        		
		                        			Prolactin/therapeutic use*
		                        			
		                        		
		                        	
8.A comparison of R-CHOP and R-DA-EPOCH as a first-line regimen treatment of diffuse large B cell lymphoma with non-GCB subtypes: a retrospective study.
Zi Yan HE ; Wen Juan YU ; Shan Shan SUO ; Jing Han WANG ; Hai Tao MENG ; Wen Yuan MAI ; Ju Ying WEI ; Min YANG ; Li Ping MAO ; Jie JIN
Chinese Journal of Hematology 2022;43(4):346-348
		                        		
		                        		
		                        		
		                        			Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
		                        			;
		                        		
		                        			Cyclophosphamide/therapeutic use*
		                        			;
		                        		
		                        			Doxorubicin/therapeutic use*
		                        			;
		                        		
		                        			Etoposide
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Lymphoma, Large B-Cell, Diffuse/pathology*
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Prognosis
		                        			;
		                        		
		                        			Retrospective Studies
		                        			;
		                        		
		                        			Rituximab/therapeutic use*
		                        			;
		                        		
		                        			Vincristine/therapeutic use*
		                        			
		                        		
		                        	
9.Clinical analysis of 5 cases of systemic juvenile idiopathic arthritis with coronary artery dilatation.
Sheng Nan LI ; Jian Ming LAI ; Min KANG ; Tong YUE ; Xiao Lei WANG
Chinese Journal of Pediatrics 2022;60(5):462-465
		                        		
		                        			
		                        			Objective: To investigate the clinical characteristics of systemic juvenile idiopathic arthritis combined with coronary artery dilatation. Methods: A retrospective analysis was performed on the clinical data, including clinical manifestations, blood routine, inflammatory factors, echocardiography, vascular ultrasound and CT angiography, treatment and outcomes, etc, of 5 cases with systemic juvenile idiopathic arthritis combined with coronary artery dilation admitted to Department of Rheumatology in the affiliated Children's Hospital of Capital Institute of Pediatrics from May 2019 to June 2021. Results: There were 2 males and 3 females among 5 cases. The onset age ranged from 7 months to 4 years 7 months.The diagnostic time ranged from 1.5 months to 3.0 months.Four cases were diagnosed as atypical Kawasaki disease. Three cases showed unilateral coronary artery dilation.Two cases showed bilateral coronary artery dilation.Four cases developed multiple organ injuries.Three cases developed macrophage activation syndrome.Three cases developed lung injury.Two cases developed pericardial effusion.One case developed pulmonary hypertension.As for treatment, 3 cases treated with methylprednisolone pulse therapy and methotrexate combined with cyclosporine, improved after the final application of biological agents, and have stopped prednisone. The other 2 cases were treated with adequate oral prednisone and gradually reduced, and methotrexate was added at the same time, 1 case relapsed in the process of reduction. No other vascular involvement was found in 5 cases. Coronary artery dilation recovered completely after 1 to 3 months of treatment. Conclusions: Systemic juvenile idiopathic arthritis combined with coronary artery dilatation has the clinical characteristics of small onset age, long diagnostic time, prone to multiple organ injuries. Corticosteroids and conventional immunosuppressive agents are not sensitive, and biological agents should be used as soon as possible.The prognosis of coronary artery dilation is good after timely treatment.
		                        		
		                        		
		                        		
		                        			Arthritis, Juvenile/drug therapy*
		                        			;
		                        		
		                        			Biological Factors/therapeutic use*
		                        			;
		                        		
		                        			Child
		                        			;
		                        		
		                        			Coronary Aneurysm/etiology*
		                        			;
		                        		
		                        			Coronary Artery Disease/therapy*
		                        			;
		                        		
		                        			Dilatation
		                        			;
		                        		
		                        			Dilatation, Pathologic
		                        			;
		                        		
		                        			Female
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Infant
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			Methotrexate
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Retrospective Studies
		                        			
		                        		
		                        	
10.Clinical characteristics and prognostic features of 63 HIV-associated diffuse large B-cell lymphoma: a single-center real-world study in China.
Chao Yu WANG ; Jun LIU ; Xi Ping LIANG ; Bing Lin GUO ; Ren Zhi HU ; Yao LIU
Chinese Journal of Hematology 2022;43(3):203-208
		                        		
		                        			
		                        			Objective: This study aimed to look into the clinical characteristics and prognosis of patients with human immunodeficiency virus (HIV) -associated diffuse large B-cell lymphoma (DLBCL) . Methods: Retrospective review of the clinical data of 63 HIV-infected patients with DLBCL diagnosed at Chongqing University Cancer Hospital between July 2008 and August 2021. The Kaplan-Meier method was used to calculate survival curves, and the log-rank test method was used to compare survival between groups. The Cox proportional hazards model was used for multivariate analysis. Results: In 63 patients with HIV-associated DLBCL, 57 (90.5% ) were men, and the median age was 49 (23-87) years. The most common pathological subtype was the germinal center B-cell-like lymphoma (74.6% ) ; 46.0% (29/63) were combined with extranodal lesions. Seventeen of 63 (27.0% ) patients had large masses (≥7.5 cm) . Twenty of 63 (31.7% ) patients had B symptoms. The median CD4(+) T cell count was 203 (4-1022) ×10(6)/L. A total of 49% (25/51) patients had CD4(+) cell count <200×10(6)/L, 56.9% (33/58) had high (3-5) International Prognostic Index (IPI) scores, and 43.1% (25/58) had low (0-2) IPI scores. Further, 78% (46/59) were diagnosed with Ann Arbor Stage Ⅲ/Ⅳ, and 25.4% (16/63) didn't receive chemotherapy. A total of 22.2% (14/63) of patients received less than four cycles of chemotherapy, and 52.4% (33/63) received four or more cycles of chemotherapy. Among patients undergoing chemotherapy, 61.7% (29/47) received R-CHOP-like regimens, and 38.3% (18/47) used CHOP-like regimens. The 1-, 2-, 3-, and 5-year overall survival (OS) rates were 65.0% , 53.8% , 47.1% , and 43.5% , respectively. Univariate analysis revealed that age ≥ 60 years (P=0.012) , Eastern Cooperative Oncology Gruop Performance Status (ECOG-PS) score 2-4 points (P=0.043) , IPI score 3-5 points (P=0.001) , β(2)-MG elevation (≥5.5 mg/L) (P=0.007) , and systemic chemotherapy cycles less than four times (P<0.001) were the negative prognostic factors affecting the OS of patients. The Cox multivariate analysis depicted that age ≥60 years (HR=2.272, 95% CI 1.110-4.651, P=0.025) , IPI score 3-5 points (HR=3.562, 95% CI 1.794-7.074, P<0.001) , ECOG-PS score 2-4 points (HR=2.675, 95% CI 1.162-6.153, P=0.021) , and number of cycles of chemotherapy<4 (HR=0.290, 95% CI 0.176-0.479, P<0.001) were independent risk factors for adverse prognosis of OS. Conclusion: HIV-associated DLBCL is the most common HIV-related tumor, is most commonly seen in men, and has a high 1-year mortality rate. Chemotherapy combined with antiretroviral therapy can improve patient prognosis.
		                        		
		                        		
		                        		
		                        			Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
		                        			;
		                        		
		                        			Cyclophosphamide/therapeutic use*
		                        			;
		                        		
		                        			Doxorubicin/therapeutic use*
		                        			;
		                        		
		                        			HIV Infections
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Lymphoma, Large B-Cell, Diffuse/drug therapy*
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			Middle Aged
		                        			;
		                        		
		                        			Prednisone/therapeutic use*
		                        			;
		                        		
		                        			Prognosis
		                        			;
		                        		
		                        			Retrospective Studies
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		                        			Rituximab/therapeutic use*
		                        			;
		                        		
		                        			Survival Rate
		                        			;
		                        		
		                        			Vincristine/therapeutic use*
		                        			
		                        		
		                        	
            
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