Annals of hematology & oncology最新文献

{"title":"MGMT Methylated High Grade Glioma with Distant Recurrence and Stable Original Tumor Site: Case Series.","authors":"Jonathan Lee, Rimas V Lukas, Ignacio Jusue-Torres, Anand V Germanwala, Ewa Borys, Abhishek A Solanki, Atul K Mallik, Kevin Barton, Jigisha P Thakkar","doi":"10.26420/annhematoloncol.2023.1421","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2023.1421","url":null,"abstract":"We present three cases of O6-Methylguanine-DNA Methyl-transferase (MGMT) methylated high grade gliomas with distant recurrence. All three patients had a radiographic stability of original tumor site at time of distant recurrence indicating impressive local control with Stupp protocol in patients with a MGMT methylated tumors. All patients had a poor outcome after distant recurrence. For one patient Next Generation Sequencing (NGS) was available for both original and recurrent tumor and did not reveal any difference other than high tumor mutational burden in the distant recurrent tumor. Understanding risk factors of distant recurrence in MGMT methylated tumors and investigating correlations between recurrences will help plan therapeutic strategies to prevent distant recurrence and improve survival of these patients.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259672/pdf/nihms-1902786.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9680509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Isolated Bone Marrow Mastocytosis may be Associated with Reduced Overall Survival","authors":"S. M, Damaj Gl, Jendoubi F, Negretto M, L. C, D. V., Evrard S, Apoil Pa, Mailhol C, Degboe Y, D. P, H. O, Paul C, Livideanu Cb","doi":"10.26420/annhematoloncol.2021.1380","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1380","url":null,"abstract":"Mastocytosis is characterized by accumulation/proliferation of abnormal Mast Cells (MCs) in tissues [1]. Depending on organ involvement, two main forms of mastocytosis are identified Isolated Cutaneous Mastocytosis (ICM), when the skin is the only tissue affected [2,3] and Systemic Mastocytosis (SM), characterized by MC infiltrates in internal organs, mostly the Bone Marrow (BM). SM can be or not associated with concomitant skin involvement. The most frequent form of SM is indolent SM [4]. Isolated Bone Marrow Mastocytosis (IBMM) constitutes a variant of indolent SM described in the 2008 WHO classification for mastocytosis [5]. There are limited information on the course and prognosis of IBMM in the literature. In the literature, it exist for patients with non-advanced and advanced mastocytosis a score who can be used to predict survival outcomes even if the predictive value of the International Prognostic Scoring system for Mastocytosis (IPSM) needs to be confirmed in forthcoming trials [6]. IBMM represents a diagnostic challenge for clinicians as clinical manifestations such as anaphylaxis; osteoporosis and digestive symptoms are not specific. The main objective of this study was to analyze the overall survival of patients with IBMM in adults with SM. The secondary objectives were to assess the frequency of IBMM in adults with SM, compare the clinical and laboratory characteristics of patients IBBM to those of patients with SM associated cutaneous mastocytosis (SMcm) and evaluate the IPSM prognostic score in our cohort.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47976623","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":"Combination Venetoclax and Rituximab: A Novel Approach to Gastrointestinal Hemorrhage from Acquired Von Willebrand Syndrome in the Setting of Relapsed Chronic Lymphocytic Leukemia","authors":"Heald Jt, Oleszewski Rt","doi":"10.26420/annhematoloncol.2021.1379","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1379","url":null,"abstract":"Congenital Von Willebrand Disease (VWD) is the most common bleeding diathesis in humans with a prevalence of one percent in the general population. However, acquired Von Willebrand Syndrome (aVWS) is a rare coagulopathy that has been reported to be associated with numerous conditions and occurs through a variety of mechanisms. In addition to causing bleeding diathesis due to coagulopathy, there is evidence that demonstrates Von Willebrand’s Factor (VWF) deficiency leads to aberrant blood vessel formation. Treatment for this disease is directed at stabilization of associated hemorrhage and correction of underlying etiology. We present the case of a patient with aVWS disease due to relapse of Chronic Lymphocytic Leukemia (CLL) complicated by gastrointestinal hemorrhage from gastric and small bowel angiodysplasia treated with rituximab and venetoclax, previously unreported in the medical literature.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44113246","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":"Basophils Unchained: A Rare Form of Leukemia","authors":"D. I, C. H., S. R., D. B, Demeester S, Jochmans K, D. A","doi":"10.26420/annhematoloncol.2021.1378","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1378","url":null,"abstract":"Primary Chronic Basophilic Leukemia (CBL) is an extremely rare disease and thus knowledge of this disease remains limited. There are no universal diagnostic criteria nor has the underlying pathogenetic mechanism been elucidated. In this peculiar case, we present a 93-year-old woman with an acute symptomatology of fatigue compounded by a manifest leukocytosis with 43% basophils in peripheral blood. The bone marrow aspirate also showed basophilia, 56% specifically. Interestingly, Next Generation Sequencing (NGS) on the bone marrow sample revealed three possible driver mutations, namely in the IDH2, DNMT3A, and BCOR gene. To date, NGS has never been performed on samples of patients with primary CBL. Our findings could be of great value in the search for the pathogenetic mechanism of this rare disease and to help find a successful treatment. Our patient was palliated with hydroxyurea to lower the white blood cells. Initially, this had a good effect, but unfortunately, she died two months after the diagnosis.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41430570","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":"Surge in Procalcitonin Levels Post ATG During Stem Cell Transplantation for Aplastic Anemia: A Diagnostic Dilemma with Sepsis?","authors":"Garg A, P. K, S. K, R. A., Shah S","doi":"10.26420/annhematoloncol.2021.1377","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1377","url":null,"abstract":"Allogenic Stem Cell Transplantation (AlloSCT) has paved way for curative therapy in numerous hematologic diseases such as Aplastic Anemia. Anti-Thymocyte Globulin (ATG), a polyclonal T-cell antibody is an integral part of the numerous conditioning regimens. Its purpose is the T-cell depletion of the recipient, which is of utmost importance for the prevention of graft rejection and successful engraftment. Febrile responses after ATG administration pose a diagnostic dilemma in these patients who are frequently neutropenic [1]. During the conditioning phase before AlloSCT, patients undergo substantial immunosuppression and immune-alteration under the effect of pharmacotherapy and total body irradiation. Conditioning with ATG can be associated with febrile episodes, circulatory instability and/or respiratory insufficiency. The severity of this reaction especially in a neutropenic patient can closely resemble sepsis. Since blood cultures can take a few days in reporting, biochemical markers of inflammation, such as C-Reactive Protein (CRP) and Procalcitonin (PCT) levels have a diagnostic value. In contrast to CRP, Procalcitonin is a sensitive and specific marker for systemic bacterial infection and fungal sepsis [2,3]. We observed a surge in procalcitonin levels after ATG administration in a patient suffering from severe aplastic anemia undergoing conditioning for allogenic bone marrow transplantation from a matched sibling donor. The patient weighing 72 kilograms, received rabbit ATG as GVHD prophylaxis at the rate 1.5mg/kg (5 vials of 25mg each) on Day - 3 of conditioning and developed high grade fever with chills, reaching a peak temperature of 103.6 degrees Fahrenheit. Microbiological cultures and procalcitonin levels were sent to rule out sepsis as part of the protocol. First line intravenous antibiotics were started and antipyretics were administered. Fever settled on its own once the ATG therapy was completed. On day -2 and -1, the same dose of ATG was given and the patient did not develop any febrile episodes. The median PCT elevation on day 1 after ATG infusion was 87.68mcg/L, nearly 70 times the baseline level. Patient was clinically stable with no features of sepsis. There was a steady decline in PCT levels over the next 4 days, 58.54, 24.79, 4.38 and 1.13 (mcg/L) respectively. No derangements in liver function tests or renal function tests were observed. Subsequently, the microbiological cultures were sterile ruling out sepsis. Several previous reports suggested limited diagnostic value of PCT and CRP in the presence of anti-T-lymphocyte antibodies. Dornbusch et al. compared 15 consecutive febrile episodes after T cell antibody infusion without clinical signs of infection with nine episodes of Gram-negative sepsis. After T-cell antibody infusion PCT and CRP serum levels increased to a similar extent as in Gramnegative sepsis. They concluded that during T-cell antibody treatment neither PCT nor CRP are adequate for differentiating between f","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43419306","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":"Weekly as Opposed to Bi-Weekly Bortezomib as Part of Induction Chemotherapy in Newly Diagnosed Multiple Myeloma is Better Tolerated and Equally Efficient in Terms of Initial Therapeutic Response: Real-World Data from a Retrospective Audit","authors":"Faulkner Lg, S. C, Sachedina S, Barton L, A. G., G. M.","doi":"10.26420/annhematoloncol.2021.1376","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1376","url":null,"abstract":"Bortezomib is a proteasome inhibitor that has shown efficacy in the treatment of newly diagnosed multiple myeloma. The VTD (Bortezomib, Thalidomide, Dexamethasone) triplet chemotherapy regime is frequently used as induction prior to autologous stem cell transplant, in line with national and international recommendations. The manufacturer’s protocol for Bortezomib recommend a twice weekly dosing schedule. Adverse effects are common, most notably peripheral and autonomic neuropathy. These adverse effects can be disabling, even at lower grades and often limit drug tolerance. We propose a once weekly Bortezomib treatment regime as an alternate modus operandi. Here we use real-world data to demonstrate that weekly compared to bi-weekly Bortezomib is better tolerated whilst achieving similar outcomes in terms of initial therapeutic response. We demonstrate a trend of lower incidence of neuropathy- both peripheral and autonomic- with the weekly regime. There was also a trend of fewer serious adverse events with the weekly regime with lower rates of hospital admissions due to infections. In addition, we show that this regime is associated with better Thalidomide tolerance. We believe that delivery of Bortezomib through a weekly regime facilitates patients being able to maintain on Bortezomib longer and receive higher cumulative doses.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49199325","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":"Pediatric Acute Promyelocytic Leukemia","authors":"C. E., Verdú Belmar J, Garzó Moreno A, D. F","doi":"10.26420/annhematoloncol.2021.1374","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1374","url":null,"abstract":"A previously healthy 4-year-old boy presented to the pediatric emergency department with high fever, headache, asthenia and neutropenia. The fever started two days prior along with the appearance of purple skin lesions. Laboratory results were as follows: White Blood Cell (WBC) count of 44.2 × 109 hemoglobin 62g/L; hematocrit 18.9%, platelet count 30 × 109/l, international normalized ratio (INR) 1.06, lactate dehydrogenase (LDH) 479u/l, creatinine 37μmol/l. Peripheral blood smear demonstrated: 80% of abnormal promyelocytes with bilobar nuclei and cytoplasmic granules; some contained multiple Auer rods. Immunophenotyping demonstrates CD13, CD33, CD117, and myeloperoxidase positivity with a high side-scatter. Fluorescence in situ hybridization revealed the t(15;17) (q22;q21.1) (PML-RARA) (Figure 1 and 2).","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49051142","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 Relationship between Use of Low Molecular Weight Heparin during Pregnancy and Risk of Peripartum Adverse Events: A Meta-Analysis","authors":"X. Y, S. L., Shengji S, T. S, Dongping L, Moli Z, J. L","doi":"10.26420/annhematoloncol.2021.1372","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1372","url":null,"abstract":"Introduction: To summarize the trials investigated on relationship between low molecular weight heparin use during pregnancy and peripartum adverse events. Meta-analysis was performed to evaluate the effect of Low Molecular Weight Heparin (LMWH) on maternal and fetal complications. Methods: Electronic research was performed in Cochrane Library, MEDLINE and EMBASE through October 2020. The primary outcome was the incidence of maternal and fetal complications during peripartum period. RevMan 5.3 was used for data analysis. Results: 11 articles were finally included. Meta-analysis showed there was no significant difference in abortion, premature delivery, stillbirth, preeclampsia and postpartum hemorrhage events between pregnant women who used LMWH and those who not. Conclusion: Using LMWH in pregnant women does not increase pregnancy related maternal and fetal complications.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46124725","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":"Pancreatic Origin Hepatoid Adenocarcinoma with Liver Metastasis","authors":"S. M, N. N., Grijalva V, Li Lz, Weinstein Pl","doi":"10.26420/annhematoloncol.2021.1371","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1371","url":null,"abstract":"Hepatoid Adenocarcinoma (HAC) is a rare form of aggressive extrahepatic neoplasm with similar morphologic features to Hepatocellular Carcinoma (HCC). HAC with pancreatic origin is rare and the exact incidence is unknown. Given shared morphological and Immunohistochemical (IHC) characteristics, it may be difficult to distinguish metastatic HAC with liver involvement from primary HCC. We present a rare case of ductal type pancreatic hepatoid adenocarcinoma involving the pancreatic head, ampulla of Vater, and liver, and illustrate strategies for diagnosis and treatment. A 65-year-old woman presented with epigastric pain, vomiting, melena, and weight loss. There was direct hyperbilirubinemia with elevated hepatic markers. Imaging displayed a pancreatic head mass with moderate biliary obstruction and a hepatic lobe lesion. Fine needle biopsy of the liver mass initially was consistent with HCC, and biopsies of the pancreatic mass and ampulla walls showed pancreatic adenocarcinoma. Due to the unusual finding of co-existing HCC and pancreatic adenocarcinoma, the hepatic mass biopsy was sent for external evaluation, which revealed poorly differentiated adenocarcinoma, consistent with HAC of pancreatic origin. The tumor was positive for mucicarmine stain, HepPar1, CEA, and CK7. It was negative for MOC-31, Arginase, ALBISH, MGB, ER, TTF-1, GCDFP15, CK-20 and CDX2, thus confirming HAC. The patient was referred to outpatient chemotherapy with gemcitabine and paclitaxel however demonstrated progression and expired following recurrent bilateral pulmonary emboli. HAC may present with non-specific symptoms, is highly aggressive, and may be difficult to distinguish from primary HCC from histopathologic characteristics alone. Accurate diagnosis requires clinicohistopathologic and IHC analysis.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47865712","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":"Study on Anti-Inflammatory Activity of Niloticin by Targeting MD-2 Based on Computer-Aided Drug Design and Biolayer Interferometry","authors":"C. G, L. Y, Z. M., X. Y","doi":"10.26420/annhematoloncol.2021.1370","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2021.1370","url":null,"abstract":"Niloticin is an active compound from Cortex phellodendri, but its antiinflammatory activity has not yet been explored. The aim of the present study was to assess the drug potential of niloticin and to study the MD-2-targeting mechanism of its anti-inflammatory activity. Niloticin’s drug potential was analyzed using the Traditional Chinese Medicine Systems Pharmacology Database. Molecular docking and biolayer interferometry technology were used to explore the anti-inflammatory mechanism of niloticin by targeting myeloid differentiation protein 2 (MD-2), which mediates a series of Toll-Like Receptor (TLR) 4-dependent inflammatory responses. The cytokines involved in the LPSTLR4/ MD-2-NF-κB pathway were evaluated by ELISA, RT-PCR, and western blot. The results showed that niloticin has drug potential and could bind to MD- 2. Niloticin had no impact on cell viability. Niloticin could significantly decrease the levels of NO, IL-6, TNF-a, and IL-1β (P<0.01) induced by LPS. IL-1β, IL-6, iNOS, TNF-a, and COX-2 mRNA expression levels were decreased by niloticin (all P<0.01). Compared with the control group, TLR4, p65, MyD88, p-p65, and iNOS expression levels induced by LPS were suppressed by niloticin (all P<0.01). In conclusion, niloticin is a potential MD-2 antagonist. It might interact with MD-2 to play an anti-inflammatory role by suppressing the activation of the LPS-TLR4/MD-2-NF-κB signaling pathway.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42169938","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}