1.Rare metastatic primary intra-abdominal follicular dendritic cell sarcoma poorly responsive to chemotherapy: A case report
Leah Anne E. Legaspi ; Mary Ondinee-Igot ; Gracieux Fernando
Philippine Journal of Internal Medicine 2024;62(1):308-313
Background:
Follicular dendritic cell sarcoma (FDCS) accounts for about 0.4% of soft tissue sarcomas. Approximately onethird of cases occur in extranodal sites and about 28% of extranodal FDCS may metastasize. Intra-abdominal occurrence is
rare and there is limited published data to guide oncologists on how to best treat this malignancy.
Case Presentation:
This is a case of a 33-year-old female who came in due to incidental finding of a left supraclavicular
mass with 2-year history of early satiety. Neck node biopsy revealed a poorly differentiated malignant tumor with positive
staining for CD21, CD23, vimentin and S100 consistent with FDCS. PET-CT revealed an intensely FDG-avid large mass in
the left upper abdomen with signs of necrosis and mass effect. The patient was given three different chemotherapy
regimens that included (1) gemcitabine/docetaxel, (2) single agent doxorubicin and (3) ifosfamide/etoposide, but she
progressed on all these. Off-label use of bendamustine was then offered and after just the first cycle, the patient reportedly
regained strength and was able to get up from wheelchair with noted interval decrease in size of the cervical mass.
Unfortunately, the patient deteriorated and succumbed to infection and multiple pulmonary embolisms.
Conclusion
Intra-abdominal FDCS is a rare malignancy with heterogenous outcomes with no uniform treatment strategy
at present. Molecular tumor board discussion and multi-disciplinary approach in extranodal FDCS is important in the
diagnosis and management. Patients with multiple poor prognostic factors are at risk for tumor recurrence, metastasis, and
death.
Dendritic Cell Sarcoma, Follicular
;
Abdominal Neoplasms
;
Drug Therapy
;
Bendamustine Hydrochloride
;
Prognosis
2.Effects of Apis dorsata honey on the mRNA expression of selected CYP450, pro-apoptotic, and anti-apoptotic genes during induced cytotoxicity in cyclophosphamide-treated human lung carcinoma (A549) cells
Jay T. Dalet ; Jose Kenneth T. Narag ; Arnold V. Hallare ; Francisco T. Heralde
Acta Medica Philippina 2024;58(19):37-49
INTRODUCTION
One of the novel strategies in cancer treatment is the combination of conventional chemotherapeutic drugs and natural products. In a previous study, co-treatment of the anti-cancer drug cyclophosphamide (CP) with honey from giant honey bee (Apis dorsata) resulted to a dose-dependent increase in its cytotoxic effect in human lung carcinoma (A549) cells. However, the molecular mechanism of this combinatorial effect remains unknown.
OBJECTIVESIn this study, the effect of A. dorsata honey on the expression of selected CYP450 genes at the mRNA level, as well as the proapoptotic gene CASP8 and antiapoptotic gene BCL2 was investigated in CP-treated A549 cells.
METHODSMTT Assay was performed to determine the cell viability of A549 cells after treatment with CP with or without A. dorsata honey, as well as the EC50 of CP with honey thereafter. RT-qPCR was then performed to study the effect of A. dorsata honey on the expression of selected CYP450 genes as well as CASP8 and BCL2 genes in CPtreated A549 cells. LC-MS was carried out to screen for putative compounds in A. dorsata honey which may possibly have anti-cancer activity.
RESULTSHoney in the lowest concentration (0.6% v/v) most effectively enhanced the cytotoxic effect of CP. CYP2J2 and CYP1B1 indicated a 2.38-fold and 1.49-fold upregulation, respectively as compared to untreated cells. This cytotoxic effect is further enhanced by upregulation of CASP8 that is paralleled by a downregulation of BCL2. Phytosphingosine and sphinganine are honey constituents which may be linked to the increased cytotoxicity of CP observed in A549 cells.
CONCLUSIONThis study provides further knowledge on the molecular basis by which A. dorsata honey potentiates the cytotoxic effect of cyclophosphamide in A549 cells.
Cyclophosphamide ; Lc-ms ; Liquid Chromatography-mass Spectrometry ; Apoptosis
3.Anti-neutrophil cytoplasmic antibody-associated vasculitis with gastrointestinal bleeding as the main symptom: a case report and literature review.
Fei TIAN ; Zhaohui ZHANG ; Lingyun ZHANG ; Min LIU ; Jinglan LIU ; Xingguang QU ; Shengmin GUI ; Xiaoyun XU
Chinese Critical Care Medicine 2023;35(4):431-434
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) has a wide range of symptoms, and it is difficult for clinicians to make a quick and correct diagnosis. On November 11, 2021, a 36-year-old male patient with AAV was admitted to the emergency and critical care department of Yichang Central People's Hospital. He was admitted to the emergency intensive care unit (EICU) with gastrointestinal symptoms (abdominal pain, black stool) as the main physical signs, and was initially diagnosed as AAV with gastrointestinal hemorrhage (GIH). No bleeding point was found after repeated gastroscopy and colonoscopy. Abdominal emission CT (ECT) showed diffuse hemorrhage in the ileum, ascending colon and transverse colon. Multi-disciplinary consultation in the whole hospital considered the diffuse hemorrhage caused by small vascular lesions in the digestive tract caused by AAV. Pulse therapy with methylprednisolone 1 000 mg/d and immunosuppressive therapy with cyclophosphamide (CTX) 0.2 g/d were administered. The patient's symptoms quickly relieved and transferred out of the EICU. After 17 days of treatment, the patient finally died of massive gastrointestinal bleeding. A systematic review of relevant literatures combined with the case diagnosis and treatment process found that only a minority of AAV patients present with gastrointestinal symptoms as their first symptoms, and patients with GIH were very rare. Such patients had a poor prognosis. This patient delayed the use of induced remission and immunosuppressive agents due to the treatment of gastrointestinal bleeding, which may be the main cause of life-threatening GIH secondary to AAV. Gastrointestinal bleeding is a rare and fatal complication of vasculitis. Timely and effective induction and remission treatment is the key to survival. Whether patients should receive maintenance therapy, the duration of maintenance therapy, and the search for markers of disease diagnosis and treatment response are directions and challenges for further research.
Male
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Humans
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Adult
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Gastrointestinal Hemorrhage
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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
;
Critical Care
;
Cyclophosphamide
;
Death
4.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
5.Bendamustine plus rituximab as first-line treatment in patients with indolent B-cell non-Hodgkin lymphoma and mantle cell lymphoma : a real-world study in China.
Kai Xin DU ; Hao Rui SHEN ; Li WANG ; Jin Hua LIANG ; Jia Zhu WU ; Yue LI ; Yi XIA ; Hua YIN ; Jian Yong LI ; Wei XU
Chinese Journal of Hematology 2023;44(4):333-336
7.Efficacy and safety of VRD regimen of autologous hematopoietic stem cell transplantation in patients with newly diagnosed multiple myeloma.
Shuang YAN ; Song JIN ; Pan Feng WANG ; Ling Zhi YAN ; Jing Jing SHANG ; Xiao Lan SHI ; Xiao Jin WU ; Ying Ying ZHAI ; Wei Qin YAO ; Jing WANG ; Ying YAO ; Cheng Cheng FU
Chinese Journal of Internal Medicine 2023;62(7):819-825
Objective: To explore the stem cell collection rate and efficacy and safety of patients aged 70 and below with newly diagnosed multiple myeloma (MM) treated with the VRD (bortezomib, lenalidomide and dexamethasone) regimen followed by autologous stem cell transplantation (ASCT). Methods: Retrospective case series study. The clinical data of 123 patients with newly diagnosed MM from August 1, 2018, to June 30, 2020, at the First Affiliated Hospital of Soochow University and Suzhou Hopes Hematology Hospital, who were eligible for VRD regimen sequential ASCT, were collected. The clinical characteristics, efficacy after induction therapy, mobilization regimen of autologous stem cells, autologous stem cell collection rate, and side effects and efficacy of ASCT were retrospectively analyzed. Results: Of the 123 patients, 67 were males. The median patient age was 56 (range: 31-70) years. Patients with IgG, IgA, IgD, and light-chain types accounted for 47.2% (58/123), 23.6% (29/123), 3.2% (4/123), and 26.0% (32/123) of patients, respectively. In addition, 25.2% (31/123) of patients had renal insufficiency (creatinine clearance rate<40 ml/min). Patients with Revised-International Staging System (R-ISS) Ⅲ accounted for 18.2% (22/121) of patients. After induction therapy, the rates of partial response and above, very-good partial response (VGPR) and above, and complete response (CR)+stringent CR were 82.1% (101/123), 75.6% (93/123), and 45.5% (56/123), respectively. Overall, 90.3% (84/93) of patients were mobilized with cyclophosphamide+granulocyte colony-stimulating factor (G-CSF) and 8 patients with G-CSF or G-CSF+plerixafor due to creatinine clearance rate<30 ml/min and one of them was mobilized with DECP (cisplatin, etoposide, cyclophosphamide and dexamethasone)+G-CSF for progressive disease. The rate of autologous stem cell collection (CD34+cells≥2×106/kg) after four courses of VRD regimen was 89.1% (82/92), and the rate of collection (CD34+cells≥5×106/kg) was 56.5% (52/92). Seventy-seven patients treated with the VRD regimen sequential ASCT. All patients had grade 4 neutropenia and thrombocytopenia. Among the nonhematologic adverse events during ASCT, the highest incidence was observed for gastrointestinal reactions (76.6%, 59/77), followed by oral mucositis (46.8%, 36/77), elevated aminotransferases (44.2%, 34/77), fever (37.7%, 29/77), infection (16.9%, 13/77) and heart-related adverse events (11.7%, 9/77). Among the adverse events, grade 3 adverse events included nausea (6.5%, 5/77), oral mucositis (5.2%, 4/77), vomiting (3.9%, 3/77), infection (2.6%, 2/77), elevated blood pressure after infusion (2.6%, 2/77), elevated alanine transaminase (1.3%, 1/77), and perianal mucositis (1.3%, 1/77); there were no grade 4 or above nonhematologic adverse events. The proportion of patients who achieved VGPR and above after VRD sequential ASCT was 100% (75/75), and the proportion of patients who were minimal residual disease-negative (<10-4 level) was 82.7% (62/75). Conclusion: In patients aged 70 and below with newly diagnosed MM treated with VRD induction therapy, the collection rate of autologous stem cells was good, and good efficacy and tolerability were noted after follow-up ASCT.
Male
;
Humans
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Female
;
Multiple Myeloma/diagnosis*
;
Hematopoietic Stem Cell Transplantation/adverse effects*
;
Retrospective Studies
;
Creatinine
;
Hematopoietic Stem Cell Mobilization
;
Transplantation, Autologous
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Dexamethasone/therapeutic use*
;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
;
Heterocyclic Compounds/therapeutic use*
;
Bortezomib/therapeutic use*
;
Cyclophosphamide/therapeutic use*
;
Stomatitis/etiology*
8.Analysis of the prevalence and social security situation of pneumoconiosis in non-coal mine industry in Jiangsu Province.
Yuan ZHAO ; Lang ZHOU ; Li Zhuang XIE ; Meng YE ; Bao Li ZHU ; Lei HAN
Chinese Journal of Industrial Hygiene and Occupational Diseases 2023;41(5):350-353
Objective: To understand the social security situation of current cases of pneumoconiosis in non-coal mine industries in Jiangsu Province, and to provide reference for the treatment and security work of pneumoconiosis patients. Methods: From January to October 2020, a follow-up survey was conducted on 4038 cases of pneumoconiosis in non-coal mine industries of the province from October 1949 to December 2019. The age, type of pneumoconiosis, industry type, and social security status of the patients were collected. Namely, work-related injury insurance, employer compensation, basic medical insurance for urban and rural residents, major illness insurance, etc. SPSS 19.0 was used for statistical description and analysis. Results: The cases of pneumoconiosis in non-coal mine industries in Jiangsu Province ranged in age from 36 to 105 (70.78±8.43) years old, and had been exposed to dust for 1 to 55 (19.27±9.29) years. Silicosis was the main form (3875 cases, 95.96%), and non-metallic mining and dressing industry was the main form (2618 cases, 64.83%). A total of 3991 cases (98.84%) of pneumoconiosis patients enjoyed social security, most of them were urban and rural residents with basic medical insurance (3624 cases, 89.75%), but there were still 47 patients without any social security. 15 cases (0.37%) enjoyed the subsistence allowance, with the monthly allowance amount ranging from 104 to 3960 yuan, with the average amount of 954.87 yuan/month. Conclusion: In Jiangsu Province, the proportion of pneumoconiosis patients in non-coal mine industries enjoying social security is relatively high, but there are still patients who do not enjoy any social security, and the difference in the amount of subsistence allowance is slightly larger. It is necessary to further improve the medical security of pneumoconiosis patients and improve their quality of life.
Humans
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Adult
;
Middle Aged
;
Aged
;
Aged, 80 and over
;
Social Security
;
Prevalence
;
Quality of Life
;
Pneumoconiosis/epidemiology*
;
Silicosis/epidemiology*
;
Etoposide
;
Ifosfamide
;
Mesna
;
Coal Mining
;
China/epidemiology*
9.Efficacy and safety of BTK inhibitor combined with bendamustine and rituximab in the first-line treatment of chronic lymphocytic leukemia/small lymphocytic lymphoma.
Shu Chao QIN ; Rui JIANG ; Ye Qin SHA ; Jing Yan QIU ; Hong Ling MI ; Yi MIAO ; Wei WU ; Li WANG ; Lei FAN ; Wei XU ; Jian Yong LI ; Hua Yuan ZHU
Chinese Journal of Hematology 2023;44(2):158-161
10.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*


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