Annals of hematology & oncology最新文献

{"title":"The Usefulness of Diagnostic Vitrectomy in Neoplastic and Non-Neoplastic Masquerade Syndrome: An Observational Study","authors":"Morawski K","doi":"10.26420/annhematoloncol.2021.1369","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1369","url":null,"abstract":"Introduction: Masquerade intraocular inflammation may be considered neoplastic or non- neoplastic masquerades such as primary intraocular lymphoma, leukemia, infectious and inflammatory diseases. These pathologies require a definitive diagnosis, as the treatment modalities are different. The aim of our study was to investigate the safety and usefulness of diagnostic vitrectomy with vitreous humor flow cytometry in eyes with intraocular inflammation of unknown etiology. Methods: A retrospective observational study included 35 eyes of 29 patients with atypical intraocular inflammation unresponsive to corticosteroid therapy. In all cases diagnostic vitrectomy with flow cytometry analysis of the vitreous specimen was performed. Results: Among 35 eyes, the result of diagnostic vitrectomy analysis showed unspecific inflammatory response in 7 (20.0%) eyes, confirmed neoplastic diseases in 5 (14.3%) eyes. All of them it was intraocular lymphoma but one of the eyes with primarily diagnosed lymphoma and one of the eyes with primarily diagnosed unspecific inflammatory response in flow cytometry has been diagnosed finally as a choroidal melanoma after enucleation of the eyeball. Diagnostic vitrectomy excluded neoplastic disease in 7 eyes (20.0%). In 3 eyes (8.6%) bacterial infection, in 4 eyes (11.4%) viral infection. In 2 eyes (5.7%) we excluded bacterial infection, in 7 cases (20.0%) no conclusive results were obtained. The most common adverse event was cataract in patients (12 eyes, 34.3%). Conclusion: Diagnostic vitrectomy with flow cytometry of vitreous humor is helpful in confirming the clinical suspected diagnosis of posterior segment inflammation. Flow cytometry need to be complemented with other diagnostic test including cytopathology, especially in cases suspected of intraocular lymphoma. Flow cytometry of the vitreous humor in choroidal melanoma is not a useful diagnostic tool.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43626686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"AML with Renal Infiltration Manifesting as Acute Renal Failure, Diagnosed with FDG-PET CT Scan: Case Report","authors":"C. P., Gupta A, O. M, A. S, N. S","doi":"10.26420/annhematoloncol.2021.1368","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1368","url":null,"abstract":"Extramedullary disease in acute myeloid leukemia is a known phenomenon with reported incidence of 2.5-9.1 %. However, acute kidney injury due to direct infiltration of malignant cells is reported in only 1% cases of acute leukemia. We report a case of acute myeloid leukemia who developed acute kidney failure at presentation, was diagnosed with renal and pancreatic infiltration by FDG-PET scan and was treated successfully with hypomethylating agent and venetoclax. PET-CT scan can be a non-invasive modality for diagnosing extramedullary disease in acute myeloid leukemia and monitoring of response to therapy in these cases. Early initiation of anti-leukemia therapy in our case lead to complete metabolic response with normalization of the renal functions.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41324871","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vanishing Bile Duct Syndrome and Hodgkin’s Lymphoma: Case Report and Thorough Review of the Literature","authors":"Keramidas V, Tastanis C, Tsirogianni K, H. P., P. M.","doi":"10.26420/annhematoloncol.2021.1365","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1365","url":null,"abstract":"Vanishing Bile Duct Syndrome (VBDS) is a rare, acquired disorder, characterized by progressive destruction and loss of intrahepatic bile ducts. The main clinical manifestations are jaundice and pruritus, caused by intralobular cholestasis. Although the pathogenic mechanism is poorly understood, VBDS has been associated with numerous etiologies such as medications, malignancies, infections and autoimmune diseases. This syndrome can appear as a paraneoplastic phenomenon in patients with Hodgkin’s Lymphoma (HL). Diagnosis is based on clinical evaluation and confirmed via liver biopsy, while treating the underlying cause is the main therapeutic target. If bile duct regeneration does not occur, possible outcomes include cirrhosis, hepatic failure and death, with liver transplantation being the only curative option. In this paper, we describe a case of HL-related VBDS in a 31-year-old female patient, who presented with jaundice, pruritus and cervical lymphadenopathy. The stage of HL was determined as IIA and a liver biopsy was performed, which confirmed the degeneration of bile ducts. The patient was treated with the ABVD regimen and dexamethasone. Follow-up tests were normal and supported the full remission hypothesis. We conducted an analytical literature review and collected the available data from 38 confirmed cases, regarding the epidemiology, viral infections, clinical findings, therapeutic options and outcome.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46912686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and Side Effects in HER2-Positive Advanced Breast Cancer Patients Treated with Pyrrotinib: A Real-World Study in China","authors":"Xiaolei Wang, Chenxu Meng, Jing-jing Li, Yun Su, Fanfan Li, Jun Zhao","doi":"10.26420/annhematoloncol.2021.1366","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1366","url":null,"abstract":"Background: Pyrotinib is a molecular and irreversible tyrosine kinase inhibitor independently developed in China, and its efficacy against HER2- positive breast cancer in the real world is not clear. In this study, we evaluated the efficacy and safety of pyrotinib in the treatment of HER2-positive advanced breast cancer based on real-world evidence. Materials and Methods: We designed a prospective observational study. Thirty-six patients with HER2-positive advanced breast cancer from a single medical center were included in the study from December 2018 to February 2021. All patients received the oral HER2 receptor inhibitor pyrotinib and received concurrent chemotherapy or endocrinotherapy. The follow-up endpoint is set as April 1, 2021. The primary endpoint is Objective Response Rate (ORR) and Disease Control Rate (DCR), and the secondary endpoint is Progression- Free Survival (PFS) and related side effects. Results: By the end point of follow-up, a total of 17 patients had progressed (including 6 deaths), and the progression-free survival rate was 52.78%. The median PFS was 13months (PFS range: 3-22 months). As the best response, 4 patients achieved CR, 20 patients achieved PR, 9 patients achieved SD, and 3 patient developed PD. The ORR was 66.67% and DCR was 91.67%. In the analysis, first-line pyrotinib treatment appeared to have higher ORR (88.88% vs 59.26%), but there was no significant difference. In addition, pyrotinib showed significant efficacy in patients with brain metastases, with an ORR of 42.85%. In terms of safety, the incidence of diarrhea was 80.55%, but only 4 patients had grade 3 diarrhea, which was tolerable after the drug dose was reduced; 1 patient had grade 4 neutropenia and grade 3 and thrombocytopenia, which were considered to be related to the chemotherapy drugs. The incidence of other adverse reactions was low, and all were grade 1 to 2. Conclusion: Pyrotinib combined with chemotherapy has a significant effect on HER2-positive breast cancer, and there is still a high ORR in patients who fail multiple lines of treatment. Side effects are overall controllable and safe.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48271400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Challenge of Diagnosing Heparin-Induced Thrombocytopenia with Negative Immunologic and Functional Assays","authors":"Glosser Ld, Knauss Hm, W. Jodeh, D. Craig","doi":"10.26420/annhematoloncol.2021.1362","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1362","url":null,"abstract":"Heparin-Induced Thrombocytopenia (HIT) is a prothrombotic and potentially fatal immune complication of heparin therapy. HIT is challenging to diagnose, particularly in critically ill patients where multiple causes of thrombocytopenia must be considered. Diagnostic algorithms for HIT begin with a clinical assessment, followed by laboratory testing when indicated. If Platelet Factor-4 (PF4)/heparin immunoassay and Serotonin Release Assays (SRA) are negative, HIT is deemed unlikely and heparin therapy may be resumed. Current recommendations have excluded the next step in work up for thrombocytopenia after immunoassay and functional assays result negative despite worsening thrombocytopenia following heparin re-initiation. We present the case of an 85-year-old male with multiple comorbidities, found to have a clinical course consistent with HIT despite negative serologic and functional assay results. Our case highlights the challenge in diagnosing heparin-induced thrombocytopenia in a medically complex patient and demonstrates the need for standardized recommendations following negative laboratory results despite high clinical suspicion.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44410534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Review of Hereditary Angioedema: Diagnosis and Management","authors":"Weis M","doi":"10.26420/annhematoloncol.2021.1361","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1361","url":null,"abstract":"Hereditary Angioedema (HAE) is caused by a deficiency in C1 esterase inhibitor and is characterized by sudden attacks of edema associated with discomfort and pain. The disease places patients at risk for disability and death if left untreated. Symptom severity and frequency can be extremely variable even among affected members of the same family. Attacks are not associated with inflammation or allergy, with most occurring secondary to trauma or stress. Swelling can affect any part of the body or multiple sites at once. Commonly affected areas include the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx. Swelling typically worsens over 24 to 36 hours and resolves within 48 hours in less severe cases. Attacks result in 15 000 to 30 000 emergency department visits each year. Many of these emergency cases will undergo unnecessary surgeries or medical procedures due to misdiagnosis. The hallmarks of HAE recurrent episodes of swelling without urticaria, a family history of HAE, first attack in childhood, and worsening at puberty can be identified by a thorough family history, and the diagnosis can be confirmed by laboratory studies. Nevertheless, diagnosis may be delayed by 2 decades. We review available therapies and clinical characteristics that will both help clinicians diagnose HAE and distinguish among emergencies and nonemergency cases.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43515291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nitric Oxide Increases Mitomycin-C Cytotoxicity by Generation of Reactive Oxygen Species and Induces Cell Death by Apoptosis in FancC-/- Cells","authors":"B. P, Patra B, Verma Rs","doi":"10.26420/annhematoloncol.2021.1360","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1360","url":null,"abstract":"Fanconi Anemia (FA) is a rare genetic disorder and patients of FA exhibit progressive bone marrow failure, developmental defects and cancer predisposition. Mitochondrial dysfunction due to oxidative stress and higher levels of Reactive Oxygen Species (ROS) has been well documented in FA. Increase in the levels of Reactive Nitrogen Species (RNS) and Nitric Oxide Synthase (NOS) activity is often correlated with OS- related disorders which have altered mitochondrial function. Biological effects of Nitric Oxide (NO) and nitrosative stress depend on the cell type, concentration of NO, the time of incubation and cellular homeostasis. NO is known to have both prooncogenic as well as tumour -suppressing effects which are a result of regulation of S- nitrosylation, genome wide epigenetic changes and posttranslational modification of protein. NO plays strategic roles in metabolic and signalling pathways, making NO metabolism a key mediator in pathways that are responsible for supporting tumorigenesis. NO is also known to have important regulatory roles in the process of autophagy, which is an important mediator in the cross-talk with ROS and RNS. In the present study, we examined the interaction between ROS and NO, induction of apoptosis by NO and the possible role of NO in the pathogenesis of FA. Investigating the involvement of NO and NS and their interactions with the known hallmarks of FA will give a more comprehensive understanding of pathogenesis of FA.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48456133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Successful Treatment of Diffuse Large B-cell Lymphoma Presenting as Acute Liver Failure","authors":"Rao Rl, N. Hornstein, K. Britten, Ghafour Sn, J. Pilley, M. Rettig","doi":"10.26420/annhematoloncol.2021.1359","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1359","url":null,"abstract":"Diffuse Large B-Cell Lymphoma (DLBCL) is the most common histologic subtype of lymphoma. Affected patients most commonly present with rapid lymph node enlargement in the neck, abdomen, or chest, with extranodal disease occurring in up to 40% of all cases. Generally, DLBCL is a clinically aggressive malignancy and the presence of extranodal disease is a poor prognostic indicator. Acute Liver Failure (ALF) is a rare presentation of extranodal DLBCL that is typically associated with a bleak prognosis; however, the impact of prompt diagnosis followed by initiation of appropriate chemotherapy in this setting is not well understood. Further complicating treatment, first-line chemotherapy regimens for DLBCL are typically contraindicated in the setting of liver failure. In this case report, we describe the successful treatment of DLBCL originally presenting as ALF through bridging therapy with a non-standard bendamustinebased approach.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49523645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Myelodysplastic Syndrome/Myeloproliferative Neoplasms with Ringed Sideroblasts and Thrombocytosis (MDS/MPN RS-T)–A Case Report with Literature Review on Diagnosis and Management","authors":"Niazi Mrk, Yousaf F, Asti D, Skaradinskiy Y, Xue W","doi":"10.26420/annhematoloncol.2021.1358","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1358","url":null,"abstract":"Myelodysplastic syndrome/Myeloproliferative neoplasms with ringed sideroblasts and thrombocytosis (MDS/MPN RS-T) is a rare disorder with mixed features of dysplasia and myeloproliferation. This is a relatively new independent entity included in the 2016 WHO classification as MDS/MPN with RS-T. The diagnostic criteria include erythroid lineage dysplasia, ≥15% Ringed Sideroblasts (RS), <1% blast cells in peripheral blood, <5% blast cells in the bone marrow, persistent thrombocytosis with platelet count ≥450×109/L, presence of SF3B1 mutation, absence of BCR-ABL1 gene fusion and rearrangement of PDGFRA, PDGFRB or FGFR1 or PCM1-JAK2. In MDS/MPN RS-T, two mutations are commonly seen JAK 2, which promotes myeloid proliferation, and SF3B1 gene, which causes myelodysplasia with ringed sideroblasts commonly observed in this syndrome. We present a relatively young 59-year-old woman diagnosed with MDS/ MPN RS-T based on the above guideline criteria. She has low-risk MDS, which favors a good prognosis; however, the presence of Essential Thrombocythemia (ET) favors a poor prognosis. Currently, there is no consensus on the specific management of this entity, given its rarity. She was referred to an allogeneic hematopoietic stem cell transplant center for curative treatment since she had become transfusion dependent. Before curative treatment, the patient was initiated on ruxolitinib as a bridging therapy to bone marrow transplant.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46077065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subcutaneous Tranexamic Acid: A Novel Approach to Managing Bleeding","authors":"A. Sutherland, M. Carey, M. Miller","doi":"10.26420/annhematoloncol.2021.1356","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1356","url":null,"abstract":"We describe the case of a 68 year old with a transglottic squamous cell carcinoma, a tracheostomy and persistent blood stained tracheal secretions. Oral and intravenous Tranexamic Acid (TA) effectively controlled the bleeding. On losing both routes, we administered 2g of TA (20ml) by continuous subcutaneous infusion over 24 hours. Control of bleeding was maintained over 18 days until death. No site reactions were observed. A literature review was undertaken, however, none of the studies looked at the use of TA in an end of life or palliative care population. We identified 3 clinical palliative care guidelines relating to continuous subcutaneous administration of TA. Further use should be reported in the literature to build the evidence base surrounding this novel practice.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42852138","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}