{"title":"Pembrolizumab in MSI-high Pancreatic Sarcomatoid Carcinoma","authors":"Mayrhofer K","doi":"10.26420/annhematoloncol.2021.1327","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1327","url":null,"abstract":"We report the outcome of a patient with MSI-high metastatic pancreatic sarcomatoid carcinoma refractory to multiple lines of chemotherapy treated successfully with pembrolizumab. In November 2015, our patient presented with epigastric pain leading to radiologic workup. A lesion in the pancreas as well as liver metastasis were diagnosed; liver biopsy revealed a poorly differentiated sarcomatoid carcinoma. After having received every approved chemotherapy for pancreatic cancer his disease eventually progressed so that we reached out for novel therapeutic strategies. Because of the tumor being MSI high, treatment with pembrolizumab was commenced in May 2017. Clinical response with better overall quality of life was soon reported and repeated CT scans showed an ongoing partial response leading to a near complete remission in the latest scan obtained. Adverse events during the course of therapy included immune mediated arthralgia grade 1, colitis grade 2 and pneumonitis grade 1 which were managed by administration of glucocorticoids without interruption of immunotherapy. To our knowledge, this is the first case of a patient with MSI high metastatic sarcomatoid carcinoma of the pancreas successfully treated with immunotherapy for more than three years.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46893587","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}
Hernández Rivera JCH, Lizardi Gómez LF, Reyes Sánchez, Guerrero Soto J, R. Je, Muñoz Menjivar C, Venegas Vera AV, Camarillo Rosas C, Patiño Ortega R, Arenas Osuna J, Arenas Osuna J, Paniagua Sierra R
{"title":"Factors Related to the Presence of Anemia in Patients with Chronic Kidney Disease in Hemodialysis","authors":"Hernández Rivera JCH, Lizardi Gómez LF, Reyes Sánchez, Guerrero Soto J, R. Je, Muñoz Menjivar C, Venegas Vera AV, Camarillo Rosas C, Patiño Ortega R, Arenas Osuna J, Arenas Osuna J, Paniagua Sierra R","doi":"10.26420/annhematoloncol.2021.1326","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1326","url":null,"abstract":"Introduction: The prevalence of anemia in Chronic Kidney Disease (CKD) is high. However, little is known about the factors related to anemia in patients with chronic Hemodialysis (HD) in Mexico. Material and Methods: A cross-sectional study was conducted in adult patients with CKD undergoing HD in the northern area of Mexico City treated at the Mexican Institute of Social Security. Hemoglobin (Hb) and Hematocrit (Htc) levels, as well as clinical and biochemical factors associated with anemia, were evaluated. Results: Data was collected from 747 patients, obtaining a mean hemoglobin of 9.7 g/dl (IQR 8.4-10.9 g/dl). The group was divided into two using Hb <10.0 g/dl and >10.0 g/dl as cutoff limits. Fifty six percent of the patients had hemoglobin =10.0 g/dl. Hb level <10.0 g/dl were associated with DM (OR 1.49, IC 95% 1.06-2.10, p=0.001), hyperphosphatemia (OR 1.69, IC 95% 1.21-2.28, p=0.001), high calcium-phosphate product (OR 1.43, IC 95% 1.01-2.03, p=0.040) and iron deficiency (OR 1.95, IC 95% 1.38-2.75, p=0.001). Glomerulopathies (OR 0.44, IC 95% 0.22-0.90, p=0.026), female gender (OR 0.55, IC 95% 0.40-0.074, p=0.001) and erythropoietin administration (OR 0.57, IC 95% 0.39-0.82, p=0.002) were associated with hemoglobin ≥10 g/dl. Conclusion: The factors associated with Hb <10.0 g/dl were mineral-bone metabolism disorders and iron deficiency. The periodic evaluation of qualityof- care indicators of HD treatment, such anemia, are necessary to detect improvement opportunities.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48373487","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":"Takotsubo Cardiomyopathy a Rare but Serious Complication of Treatment of Primary Central Nervous System Diffuse Large B Cell Lymphoma","authors":"Zaucha Jm, S. A, Taszner M, Korzon Burakowska A","doi":"10.26420/annhematoloncol.2021.1324","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1324","url":null,"abstract":"Takotsubo Cardiomyopathy (TC) is a rare, temporary non-ischemic cardiomyopathy usually triggered by emotional stress or mechanical trauma of the brain, however, it might be associated with other Central Nervous System (CNS) diseases. Affected patients present with clinical symptoms of myocardial infarction, cardiac shock that may lead to cardiac arrest. Primary CNS lymphoma is also a rare disease affecting mainly elderly patients, with poor prognosis further aggravated by patient’s performance status, comorbidities, early and late treatment related complications. Here we present a 78-year-old patient with primary CNS lymphoma who unexpectedly developed TC episode following successful chemotherapy treatment.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41989653","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}
D. Shetty, E. Talker, H. Jain, S. Patil, P. Tembhare, N. Patkar, L. Nayak, M. Sengar
{"title":"Additional Cytogenetic Aberrations Indicative of Poor Prognosis in AML with RUNX1/RUNX1T1 Rearrangement: Karyotype a Chromosomal Blueprint","authors":"D. Shetty, E. Talker, H. Jain, S. Patil, P. Tembhare, N. Patkar, L. Nayak, M. Sengar","doi":"10.26420/ANNHEMATOLONCOL.2021.1325","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2021.1325","url":null,"abstract":"AML is a highly heterogeneous disease with t(8;21)(q22;q22) as a frequently occurring aberration. While most studies show these patients to demonstrate a good response to standard chemotherapy, a lot of Indian and Western studies suggest otherwise. Trisomy 4 is a rare but a specific chromosomal abnormality in certain subtypes of AML. Although the significance of t(8;21) with trisomy 4 in AML remains uncertain, patients with this abnormality are typically associated with poor prognosis. Similarly, KIT mutations are also common with t(8;21) AML suggesting poorer outcomes. We report a case of AML with duplicated derivative 21 due to t(8;21), trisomy 4 and KIT mutation at baseline and an additional t(2;12) and ASXL2 mutation at relapse.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46737175","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":"Severe Eosinophilia Associated with FIP1L1/PDGFRA Rearrangement Presenting with Yamaguchi Syndrome","authors":"Z. Aamir, F. Meraj, Hanif Hm","doi":"10.26420/ANNHEMATOLONCOL.2021.1323","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2021.1323","url":null,"abstract":"FIP1L1/PDGFRA is a rare genetic rearrangement, presenting most commonly as Chronic Eosinophilic Leukemia (CEL), but may also be associated with other myeloid and lymphoid neoplasms. The peripheral blood and bone marrow exhibit a striking eosinophilia, often associated with an increased number of mast cells on trephine biopsy. Tissue infiltration by eosinophils and release of cytokines from eosinophilic granules mediate multi-organ tissue damage. The tyrosine kinase inhibitor Imatinib has been shown to induce rapid and complete clinical and haematological responses in patients harboring the mutation. We present the case of a young patient with CEL associated with PDGFRA rearrangement, presenting with severe eosinophilia and evidence of multi-organ damage (cardiac, renal, endocrine and respiratory). The peripheral blood and bone marrow displayed a striking eosinophila, and FISH analysis for FIP1L1/PDGFRA revealed a positive fusion signal in 92% of the nuclei examined. Echocardiography showed left ventricular apical hypertrophy (Yamaguchi syndrome), which has previously not been reported in this subset of patients. He was managed with supportive care, along with low-dose imatinib (100mg/day initially), to which he achieved a rapid clinical and haematological response. Currently, five months from the initial diagnosis, he is doing well on low dose imatinib (100 mg) twice a week.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47814397","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}
D. Mehta, S. Deplano, E. Olavarría, P. Gaur, L. Bourantas
{"title":"A Fatal Case of Chronic Active EBV Infection","authors":"D. Mehta, S. Deplano, E. Olavarría, P. Gaur, L. Bourantas","doi":"10.26420/ANNHEMATOLONCOL.2021.1322","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2021.1322","url":null,"abstract":"Chronic active EBV infection is a rare condition with considerable diagnostic and treatment difficulties. We herein report the case of a 27-year old female presenting with headaches and diplopia who deteriorated rapidly following her diagnosis, with confirmed EBV-infected T-lymphocyte deposits in multiple tissue sites including temporalis muscle and liver.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49273182","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}
L. R, A. A, Tuluca A, B. B, Bonita R, B. S, Hamshari Ya, Robbins T, Mossayebi Mh, S. l.
{"title":"Off-Pump HeartMate 3® LVAD Implantation via Left Thoracotomy to Descending Aorta: Transition from Transaxillary Impella 5.0® LVAD","authors":"L. R, A. A, Tuluca A, B. B, Bonita R, B. S, Hamshari Ya, Robbins T, Mossayebi Mh, S. l.","doi":"10.26420/annhematoloncol.2021.1352","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1352","url":null,"abstract":"A 62-year-old man with a prior history of Coronary Artery Bypass Grafting (CABG) presented in cardiogenic shock. A percutaneous left femoral Impella CP® Left Ventricular Assist Device (LVAD) was placed with modest improvement in hemodynamics. The LVAD was upgraded to an open right transaxillary Impella 5.0® with hemodynamic stabilization. Cardiacfunction was assessed with serial echocardiography demonstrating persistent severe left ventricular dysfunction. In view of previous CABG with patent Left Internal Mammary Artery (LIMA) graft the decision was made to place a HeartMate 3® LVAD via left thoracotomy with LV apical inflow and descending aortic outflow. This approach was completed without the need for Cardiopulmonary Bypass (CPB). The postoperative course was uneventful and discharge to a rehabilitation center occurred on the ninth postoperative day.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69277611","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}
Steegmann Jl, Zamorano Jl, P. Ja, Hernández-Boluda Jc, P. R, García-Gutierrez V, G. Jr, D. J, Roa-Chamorro R, Pardo A, A. A, F. E, López-Fernández T
{"title":"Cardiovascular Risk Management Recommendations for Patients with Chronic Myeloid Leukaemia who are Candidates for Ponatinib: Multidisciplinary Delphi Analysis","authors":"Steegmann Jl, Zamorano Jl, P. Ja, Hernández-Boluda Jc, P. R, García-Gutierrez V, G. Jr, D. J, Roa-Chamorro R, Pardo A, A. A, F. E, López-Fernández T","doi":"10.26420/annhematoloncol.2021.1354","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1354","url":null,"abstract":"Progress in the treatment of Chronic Myeloid Leukaemia (CML) has significantly improved the survival rates and prognosis of these patients. As a result, there is a growing awareness of the adverse effects that the treatments used can have on the Cardiovascular (CV) system. A high percentage of patients develop sequential resistance to CML treatments and, in these cases, ponatinib represents a good therapeutic option that has been associated with cardiovascular events. This required the development of recommendations for its management. A Delphi analysis conducted by a multidisciplinary panel of experts developed and agreed on clinical practice recommendations to optimize cardiovascular risk control in CML patients requiring ponatinib treatment.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69277687","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}
Djibrilla Almoustapha A, Maman Brah M, Chefou M, O. M., A. B, Lawali Issa H, M. S, Bade Mr, Issoufou F, B. S., Malam Abdou B, Adehossi Eo
{"title":"Observation of Causes of Death from Sickle Cell Anemia in Niger","authors":"Djibrilla Almoustapha A, Maman Brah M, Chefou M, O. M., A. B, Lawali Issa H, M. S, Bade Mr, Issoufou F, B. S., Malam Abdou B, Adehossi Eo","doi":"10.26420/annhematoloncol.2021.1329","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1329","url":null,"abstract":"Purpose: Improve the management of Major Sickle Cell Syndromes (SDM) by studying the circumstances in which patients affected by this disease die in Niger. Methods: This was a retrospective study of descriptive and analytical type in sickle cell patients followed at the National Sickle Cell Reference Center (CNRD) in Niamey with a follow-up file, who died during our study period, which was 9 years (January 1, 2010 to December 31, 2018). Results: During the study period, 6,465 sickle cell patients were followed up at the CNRD, 249 deaths were recorded, representing a frequency of 3.8%. There is a male predominance with a sex ratio of 1:3. The 0-15 age group was the most represented with 34.5% with an average age of 9.25 years. 34.1% of the patients came from a consanguineous marriage. Of the 249 deaths, 94% (n=234) were SS, 5.6% (n=14) were SC and 0.4% (n=1) was S/β. The majority of patients died at the time of registration, i.e. 58.25% (n=145 (SS=137. SC=8.)). 90.4% had died in a health-care facility. The main cause of death was anemia in 73.1% of cases. Conclusion: The management of sickle cell disease requires adequate preventive action to reduce the rate of early mortality.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69277558","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":"Concise Review: How are Red Blood Cells Born, How do they Live and Die?","authors":"Vives Corrons J-L, Casafont Lb, Frasnedo Ef","doi":"10.26420/annhematoloncol.2021.1353","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1353","url":null,"abstract":"The average life cycle of a human RBC is approximately 120 days. Generally, by this point, the cell is worn out and damaged. RBCs pass through both the spleen and liver, where specialized immune cells called macrophages are found. Macrophages recognize when an RBC is spent, and undergo a process called phagocytosis where they digest the cell. In this process, the iron in hemoglobin is recycled for use in new blood cells and the hem molecule is degraded, conjugated to bilirubin, and eliminated from the body. All the other cellular proteins are either recycled or eliminated. Historically, this process was thought to occur exclusively in the spleen, but recent studies have shown that it occurs in the bone marrow .The RBC has been analyzed from many perspectives: cytological, hematological, and immunological, as well as from the focus of molecular biology, biophysics, and mathematics. Here we analyze how are red blood cells born and how they live and die in a brief overview of the whole process with special mention of the morphological aspects from bone marrow and spleen provided by Transmission and Scanning Electron Microscopy.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69277668","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}