{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:47:36Z","timestamp":1787028456623,"version":"build-2736575974"},"reference-count":77,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2021,8,20]],"date-time":"2021-08-20T00:00:00Z","timestamp":1629417600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Mast cell disease is an epigenetically and genetically determined disease entity with very diverse clinical manifestations in potentially every system and tissue due to inap pro priate release of variable subsets of mast cell mediators together with accumulation of either morphologically normal or altered mast cells. Easy bruising, excessive bleeding, and aberrancies of erythropoiesis can frequently be observed in patients with mast cell disease. A thorough history, including a family history, will guide the appropriate work-up, and laboratory evaluations may provide clues to diagnosis. In recent years, our understanding of the involvement of coagulation and anticoagulant pathways, the fibrinolytic system, and erythropoiesis in the pathophysiology of mast cell disease has increased considerably. This review summarizes current knowledge of the impact of the disturbed hemostatic and erythropoietic balance in patients with mast cell disease and describes options of treatment.<\/jats:p>","DOI":"10.3390\/ijms22168960","type":"journal-article","created":{"date-parts":[[2021,8,20]],"date-time":"2021-08-20T08:44:45Z","timestamp":1629449085000},"page":"8960","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Effects of Primary Mast Cell Disease on Hemostasis and Erythropoiesis"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5337-3797","authenticated-orcid":false,"given":"Holger","family":"Seidel","sequence":"first","affiliation":[{"name":"Center for Bleeding Disorders and Transfusion Medicine (CBT), Am Propsthof 3, D-53121 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans-J\u00f6rg","family":"Hertfelder","sequence":"additional","affiliation":[{"name":"Center for Bleeding Disorders and Transfusion Medicine (CBT), Am Propsthof 3, D-53121 Bonn, Germany"},{"name":"Institute of Experimental Haematology and Transfusion Medicine, University Hospital Bonn, Venusberg-Campus 1, D-53127 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1585-4100","authenticated-orcid":false,"given":"Johannes","family":"Oldenburg","sequence":"additional","affiliation":[{"name":"Institute of Experimental Haematology and Transfusion Medicine, University Hospital Bonn, Venusberg-Campus 1, D-53127 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes P.","family":"Kruppenbacher","sequence":"additional","affiliation":[{"name":"Center for Bleeding Disorders and Transfusion Medicine (CBT), Am Propsthof 3, D-53121 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3550-4244","authenticated-orcid":false,"given":"Lawrence B.","family":"Afrin","sequence":"additional","affiliation":[{"name":"Department of Mast Cell Studies, AIM Center for Personalized Medicine, 3010 Westchester Ave Suite 404, Purchase, NY 10577, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard J.","family":"Molderings","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, University Hospital of Bonn, Venusberg-Campus 1, D-53127 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"190","DOI":"10.3109\/07853890.2016.1161231","article-title":"Often seen, rarely recognized: Mast cell activation disease\u2014A guide to diagnosis and therapeutic options","volume":"48","author":"Afrin","year":"2016","journal-title":"Ann. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.imlet.2016.07.003","article-title":"Mast cells as effector cells of innate immunity and regulators of adaptive immunity","volume":"178","author":"Cardamone","year":"2016","journal-title":"Immunol. Lett."},{"key":"ref_3","unstructured":"Ibelgaufts, H. (2021, June 22). \u201cMast Cells\u201d in COPE: Cytokines and Cells Online Pathfinder Encyclopaedia. Available online: http:\/\/www.cells-talk.com\/."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Molderings, G.J., Haenisch, B., Bogdanow, M., Fimmers, R., and N\u00f6then, M.M. (2013). Familial occurrence of systemic mast cell activation disease. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0076241"},{"key":"ref_5","unstructured":"Maitland, A., Brock, I., and Reed, W. (2020, January 2\u20133). Immune dysfunction, both mast cell activation disorders and primary immune deficiency, is common among patients with hypermobile spectrum disorder (HSD) or hypermobile type Ehlers Danlos Syndrome (hEDS). Proceedings of the EDS ECHO Summit, Available online: https:\/\/www.ehlers-danlos.com\/eds-echo-summit-2020\/."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2235","DOI":"10.1016\/j.jaip.2021.03.005","article-title":"The genetic basis and clinical impact of hereditary alpha-tryptasemia","volume":"9","author":"Luskin","year":"2021","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Nedoszytko, B., Sobalska-Kwapis, M., Strapagiel, D., Lange, M., G\u00f3rska, A., Elberink, J.N.G.O., van Doormaal, J., S\u0142omka, M., Kalinowski, L., and Grucha\u0142a-Niedoszytko, M. (2020). Results from a genome-wide association study (GWAS) in Mastocytosis Reveal New Gene Polymorphisms Associated with WHO subgroups. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21155506"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.ajhg.2020.12.007","article-title":"Genome-wide association study identifies novel susceptibility loci for KIT D816V positive mastocytosis","volume":"108","author":"Kristensen","year":"2021","journal-title":"Am. J. Hum. Genet."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1007\/s00251-017-0981-y","article-title":"Mutational profiling in the peripheral blood leukocytes of patients with systemic mast cell activation syndrome using next-generation sequencing","volume":"69","author":"Haenisch","year":"2017","journal-title":"Immunogenetics"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1016\/j.amjms.2016.12.013","article-title":"Characterization of mast cell activation syndrome","volume":"353","author":"Afrin","year":"2017","journal-title":"Am. J. Med. Sci."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1097\/MAJ.0b013e31821d41dd","article-title":"Polycythemia from mast cell activation syndrome: Lessons learned","volume":"342","author":"Afrin","year":"2011","journal-title":"Am. J. Med. Sci."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1007\/s12185-010-0605-x","article-title":"Mast cell activation disorder masquerading as pure red cell aplasia","volume":"91","author":"Afrin","year":"2010","journal-title":"Int. J. Hematol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1055\/s-0029-1245707","article-title":"Evidence for mast cell activation in patients with therapy-resistant irritable bowel syndrome","volume":"49","author":"Frieling","year":"2011","journal-title":"Z. Gastroenterol."},{"key":"ref_14","first-page":"260","article-title":"Pathologic fracture in systemic mastocytosis: Radiographic spectrum and review of the literature","volume":"180","author":"Rafii","year":"1983","journal-title":"Clin. Orthop. Relat. Res."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1093\/rheumatology\/36.3.393","article-title":"The effect of pamidronate on lumbar spine bone density and pain in osteoporosis secondary to systemic mastocytosis","volume":"36","author":"Marshall","year":"1997","journal-title":"Br. J. Rheumatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"846","DOI":"10.3389\/fimmu.2017.00846","article-title":"The mast cell, contact, and coagulation system connection in anaphylaxis","volume":"8","author":"Guilarte","year":"2017","journal-title":"Front. Immunol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1160\/TH11-05-0351","article-title":"Bleeding diathesis in patients with mast cell activation disease","volume":"106","author":"Seidel","year":"2011","journal-title":"Thromb. Haemost."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"eaay6314","DOI":"10.1126\/sciadv.aay6314","article-title":"Platelets trigger perivascular mast cell degranulation to cause inflammatory responses and tissue injury","volume":"6","author":"Karhausen","year":"2020","journal-title":"Sci. Adv."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"e1414","DOI":"10.1097\/MD.0000000000001414","article-title":"A French national survey on clotting disorders in mastocytosis","volume":"94","author":"Carvalhosa","year":"2015","journal-title":"Medicine"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1177\/1076029607309173","article-title":"Fatal bleeding due to a heparin-like anticoagulant in a 37-year-old woman suffering from systemic mastocytosis","volume":"14","author":"Sucker","year":"2008","journal-title":"Clin. Appl. Thromb. Hemost."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1186\/1757-1626-1-100","article-title":"An unusual cause of spontaneous bleeding in the intensive care unit-mastocytosis: A case report","volume":"1","author":"Koenig","year":"2008","journal-title":"Cases J."},{"key":"ref_22","first-page":"517","article-title":"Bleeding and shock in a 44-year-old woman with systemic mastocytosis","volume":"23","year":"2013","journal-title":"J. Investig. Allergol. Clin. Immunol."},{"key":"ref_23","first-page":"629","article-title":"Systemic mastocytosis as an unconventional cause of variceal bleeding: Think outside the box","volume":"8","author":"Ahmed","year":"2016","journal-title":"Cureus"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.iac.2014.01.001","article-title":"Mast cell-restricted tetramer-forming tryptases and their beneficial roles in hemostasis and blood coagulation","volume":"34","author":"Castells","year":"2014","journal-title":"Immunol. Allergy Clin. N. Am."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1007\/BF02158526","article-title":"Occurrence and significance of endogenous heparin","volume":"10","author":"Studer","year":"1954","journal-title":"Experientia"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1055\/s-0028-1087139","article-title":"Endogenous heparin","volume":"4","author":"Jaques","year":"1978","journal-title":"Semin. Thromb. Hemost."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Vysniauskaite, M., Hertfelder, H.J., Oldenburg, J., Dre\u00dfen, P., Brettner, S., Homann, J., and Molderings, G.J. (2015). Determination of plasma heparin level improves identification of systemic mast cell activation disease. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0124912"},{"key":"ref_28","first-page":"491","article-title":"Acquired von Willebrand syndrome during systemic mastocytosis: An analysis of 21 cases","volume":"128","author":"Mital","year":"2018","journal-title":"Pol. Arch. Intern. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"151","DOI":"10.5603\/JTM.2020.0005","article-title":"Acquired von Willebrand syndrome associated with indolent systemic mastocytosis","volume":"13","author":"Majeranowski","year":"2020","journal-title":"J. Transf. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1055\/s-0038-1648232","article-title":"Hemostasis in allergy","volume":"44","author":"Vadasz","year":"2018","journal-title":"Semin. Thromb. Hemost."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.4049\/jimmunol.162.2.1032","article-title":"The mast cell as site of tissue-type plasminogen activator expression and fibrinolysis","volume":"162","author":"Sillaber","year":"1999","journal-title":"J. Immunol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"786","DOI":"10.1055\/s-0037-1613084","article-title":"New aspects in thrombosis research: Possible role of mast cells as profibrinolytic and antithrombotic cells","volume":"87","author":"Valent","year":"2002","journal-title":"Thromb. Haemost."},{"key":"ref_33","first-page":"469","article-title":"Higher susceptibility of mast-cell-deficient W\/WV mutant mice to brain thromboembolism and mortality caused by intravenous injection of India ink","volume":"122","author":"Kitamura","year":"1986","journal-title":"Am. J. Pathol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"878","DOI":"10.1038\/ni.3445","article-title":"Expression profiling of constitutive mast cells reveals a unique identity within the immune system","volume":"17","author":"Dwyer","year":"2016","journal-title":"Nat. Immunol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1042\/bj2760739","article-title":"Up-regulation of thrombomodulin by activation of histamine H1-receptors in human umbilical-vein endothelial cells in vitro","volume":"276","author":"Hirokawa","year":"1991","journal-title":"Biochem. J."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1962","DOI":"10.1111\/j.1538-7836.2008.03138.x","article-title":"Protein kinase C-delta mediates von Willebrand factor secretion from endothelial cells in response to vascular endothelial growth factor (VEGF) but not histamine","volume":"6","author":"Lorenzi","year":"2008","journal-title":"J. Thromb. Haemost."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"9360","DOI":"10.1016\/S0021-9258(19)76549-1","article-title":"Stimulation and desensitization of tissue plasminogen activator release from human endothelial cells","volume":"263","author":"Levin","year":"1988","journal-title":"J. Biol. Chem."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"684","DOI":"10.1182\/blood.V54.3.684.684","article-title":"Diminished platelet adenylate cyclase activation by prostaglandin D2 in acute thrombosis","volume":"54","author":"Cooper","year":"1979","journal-title":"Blood"},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Braune, S., K\u00fcpper, J.H., and Jung, F. (2020). Effect of prostanoids on human platelet function: An overview. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21239020"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"7834","DOI":"10.1074\/jbc.M111.325712","article-title":"Mast cell restricted mouse and human tryptase\u00b7heparin complexes hinder thrombin-induced coagulation of plasma and the generation of fibrin by proteolytically destroying fibrinogen","volume":"287","author":"Zheng","year":"2012","journal-title":"J. Biol. Chem."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"5727","DOI":"10.1182\/blood-2009-02-205237","article-title":"Systemic mastocytosis in 342 consecutive adults: Survival studies and prognostic factors","volume":"113","author":"Lim","year":"2009","journal-title":"Blood"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/S2215-0366(21)00084-5","article-title":"6-month neurological and psychiatric outcomes in 236 379 survivors of COVID-19: A retrospective cohort study using electronic health records","volume":"8","author":"Taquet","year":"2021","journal-title":"Lancet Psychiatry"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/j.ijid.2020.09.016","article-title":"Covid-19 hyperinflammation and post-Covid-19 illness may be rooted in mast cell activation syndrome","volume":"100","author":"Afrin","year":"2020","journal-title":"Int. J. Infect. Dis."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.tcm.2020.10.005","article-title":"COVID-19-A vascular disease","volume":"31","author":"Siddiqi","year":"2021","journal-title":"Trends Cardiovasc. Med."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1007\/s00104-019-0935-z","article-title":"Surgical interventions in patients with systemic mast cell activation disease: Recommendations for perioperative management","volume":"90","author":"Sido","year":"2019","journal-title":"Chirurg"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"2284","DOI":"10.1073\/pnas.85.7.2284","article-title":"Identification of chondroitin sulfate E proteoglycans and heparin proteoglycans in the secretory granules of human lung mast cells","volume":"85","author":"Stevens","year":"1988","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1016\/0049-3848(83)90337-7","article-title":"Heparin inactivation during blood storage: Its prevention by blood collection in citric acid, theophylline, adenosine, dipyridamole-C.T.A.D. mixture","volume":"31","author":"Contant","year":"1983","journal-title":"Thromb. Res."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1615\/JEnvironPatholToxicolOncol.v28.i3.30","article-title":"Effects of shear stress on intracellular calcium change and histamine release in rat basophilic leukemia (RBL-2H3) cells","volume":"28","author":"Yang","year":"2009","journal-title":"J. Environ. Pathol. Toxicol. Oncol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1016\/j.jbiomech.2012.11.014","article-title":"Mechanical loading promotes mast cell degranulation via RGD-integrin dependent pathways","volume":"46","author":"Fowlkes","year":"2013","journal-title":"J. Biomech."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1080\/01443615.2018.1550475","article-title":"Successful mast-cell-targeted treatment of chronic dyspareunia, vaginitis, and dysfunctional uterine bleeding","volume":"39","author":"Afrin","year":"2019","journal-title":"J. Obstet. Gynaecol."},{"key":"ref_51","unstructured":"Pfizer Canada Inc (2013). Cyklokapron\u00ae\u2014Product Monograph, Pfizer Canada Inc."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.2165\/00003495-199957060-00017","article-title":"Tranexamic acid: A review of its use in surgery and other indications","volume":"57","author":"Dunn","year":"1999","journal-title":"Drugs"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/cc6173","article-title":"Tranexamic acid attenuates inflammatory response in cardiopulmonary bypass surgery through blockade of fibrinolysis: A case control study followed by a randomized double-blind controlled trial","volume":"11","author":"Jimenez","year":"2007","journal-title":"Crit. Care"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1186\/1749-8090-6-138","article-title":"Safety and effectiveness of two treatment regimes with tranexamic acid to minimize inflammatory response in elective cardiopulmonary bypass patients: A randomized double-blind, dose-dependent, phase IV clinical trial","volume":"6","author":"Iribarren","year":"2011","journal-title":"J. Cardiothorac. Surg."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/0091-6749(77)90080-X","article-title":"Tranexamic acid: Preoperative prophylactic therapy for patients with hereditary angioneurotic edema","volume":"60","author":"Sheffer","year":"1977","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"585","DOI":"10.2165\/11209070-000000000-00000","article-title":"Tranexamic acid: A review of its use in the treatment of hyperfibrinolysis","volume":"72","author":"McCormack","year":"2012","journal-title":"Drugs"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1111\/j.1600-065X.2007.00515.x","article-title":"Mast cells: Multipotent local effector cells in atherothrombosis","volume":"217","author":"Kovanen","year":"2007","journal-title":"Immunol. Rev."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1160\/TH11-05-0291","article-title":"Mast cells in atherosclerosis","volume":"106","author":"Bot","year":"2011","journal-title":"Thromb. Haemost."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1046\/j.1523-1747.1999.00624.x","article-title":"Increased formation of thromboxane in vivo in humans with mastocytosis","volume":"113","author":"Morrow","year":"1999","journal-title":"J. Investig. Dermatol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1161\/CIRCRESAHA.117.311185","article-title":"Mast cells granular contents are crucial for deep vein thrombosis in mice","volume":"121","author":"Ponomaryov","year":"2017","journal-title":"Circ. Res."},{"key":"ref_61","first-page":"437","article-title":"Factor VIII related antigen and mast cells","volume":"90","author":"Kindblom","year":"1982","journal-title":"Acta Pathol. Microbiol. Immunol. Scand. A"},{"key":"ref_62","first-page":"344","article-title":"Immunohistochemical characterization of human cutaneous mast cells in urticaria pigmentosa (cutaneous mastocytosis)","volume":"41","author":"Akiyama","year":"1991","journal-title":"Acta Pathol. Jpn."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1111\/bjd.17534","article-title":"Mast cell chymase degrades fibrinogen and fibrin","volume":"181","author":"Siiskonen","year":"2019","journal-title":"Br. J. Dermatol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"S92","DOI":"10.1111\/jth.12896","article-title":"Polyphosphate as modulator of hemostasis, thrombosis, and inflammation","volume":"13","author":"Morrissey","year":"2015","journal-title":"J. Thromb. Haemost."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"76","DOI":"10.3389\/fmed.2019.00076","article-title":"Polyphosphate as a target for interference with inflammation and thrombosis","volume":"6","author":"Mailer","year":"2019","journal-title":"Front. Med."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1002\/path.5212","article-title":"Extracellular traps derived from macrophages, mast cells, eosinophils and neutrophils are generated in a time-dependent manner during atherothrombosis","volume":"247","author":"Pertiwi","year":"2019","journal-title":"J. Pathol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"3070","DOI":"10.1182\/blood-2007-07-104018","article-title":"Phagocytosis-independent antimicrobial activity of mast cells by means of extracellular trap formation","volume":"111","author":"Goldmann","year":"2008","journal-title":"Blood"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.thromres.2012.11.034","article-title":"Histamine reduces GPIb\u03b1-mediated adhesion of platelets to TNF-\u03b1-activated vascular endothelium","volume":"131","author":"Brown","year":"2013","journal-title":"Thromb. Res."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1159\/000083842","article-title":"New aspects in thrombotic research: Complement induced switch in mast cells from a profibrinolytic to a prothrombotic phenotype","volume":"33","author":"Wojta","year":"2003","journal-title":"Pathophysiol. Haemost. Thromb."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1007\/s00210-016-1247-1","article-title":"Pharmacological treatment options for mast cell activation disease","volume":"389","author":"Molderings","year":"2016","journal-title":"Naunyn Schmiedebergs Arch. Pharmacol."},{"key":"ref_71","first-page":"129","article-title":"Improved metastatic uterine papillary serous cancer outcome with treatment of mast cell activation syndrome","volume":"28","author":"Afrin","year":"2014","journal-title":"Oncology"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1007\/978-981-13-8871-2_18","article-title":"Inflammatory mediators and renal fibrosis","volume":"1165","author":"Meng","year":"2019","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_73","doi-asserted-by":"crossref","unstructured":"Vibhushan, S., Bratti, M., Montero-Hern\u00e1ndez, J.E., El Ghoneimi, A., Benhamou, M., Charles, N., Daugas, E., and Blank, U. (2020). Mast cell chymase and kidney disease. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22010302"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1182\/blood-2016-09-739003","article-title":"CCL2 is a KIT D816V-dependent modulator of the bone marrow microenvironment in systemic mastocytosis","volume":"129","author":"Greiner","year":"2017","journal-title":"Blood"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/j.humpath.2017.01.007","article-title":"Transforming growth factor \u03b2- and interleukin 13-producing mast cells are associated with fibrosis in bone marrow","volume":"62","author":"Nakayama","year":"2017","journal-title":"Hum. Pathol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.imlet.2018.04.002","article-title":"A new role for mast cells as scavengers for clearance of erythrocytes damaged due to oxidative stress","volume":"199","author":"Sharma","year":"2018","journal-title":"Immunol. Lett."},{"key":"ref_77","first-page":"6928571","article-title":"Aggressive systemic mastocytosis in association with pure red rell aplasia","volume":"2018","author":"Karam","year":"2018","journal-title":"Case Rep. 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