Research Topics
Species | Cem AkinSummaryAffiliation: University of Michigan Country: USA Publications
| Collaborators
|
Detail Information
Publications
The biology of Kit in disease and the application of pharmacogeneticsCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, MD, USA
J Allergy Clin Immunol 114:13-9; quiz 20. 2004..This review will discuss the pathobiology of Kit in human disease, with a particular emphasis on implications for potential targeted treatment strategies in mast cell disease...
Demonstration that mast cells, T cells, and B cells bearing the activating kit mutation D816V occur in clusters within the marrow of patients with mastocytosisMarcia L Taylor
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 10 Center Drive, Room 11C 205, MSC1881, Bethesda, MD 20892 1881, USA
J Mol Diagn 6:335-42. 2004..Further, the B cell population is oligoclonal, suggesting that clonal proliferation is unlikely to be the basis of clustering...- KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entitiesIrina Maric
Department of Laboratory Medicine, Clinical Center, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
J Allergy Clin Immunol 120:680-7. 2007..It is of paramount importance, however, to distinguish between these 2 groups of patients because of differences in clinical sequelae, prognoses, and selection of treatment... - IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosisKnut Brockow
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892 1881, USA
Clin Immunol 115:216-23. 2005..There was an inverse correlation to hemoglobin. sIL-6R levels were not elevated. These observations demonstrate that IL-6 is a useful surrogate marker of severity of hematologic disease and suggest that IL-6 contributes to pathology... - Effects of tyrosine kinase inhibitor STI571 on human mast cells bearing wild-type or mutated c-kitCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
Exp Hematol 31:686-92. 2003..Because activating mutations of c-kit affecting codon 816 are associated with human mast cell neoplasms, we determined whether STI571 exerted a similar cytotoxic effect on neoplastic and normal human mast cells... - Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndromeAmy D Klion
Bldg 4, Rm 126, National Institutes of Health, Bethesda, MD 20892
Blood 103:473-8. 2004..The lack of reversal of cardiac abnormalities and persistence of the F/P mutation in some patients suggests that early intervention with higher doses of imatinib mesylate may be desirable in the treatment of patients with MHES... - Thrombopoietin alone or in the presence of stem cell factor supports the growth of KIT(CD117)low/ MPL(CD110)+ human mast cells from hematopoietic progenitor cellsArnold S Kirshenbaum
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
Exp Hematol 33:413-21. 2005..We explored the ability of TPO alone or in the presence of stem cell factor (SCF) to support human mast cells (HuMCs)... - A novel form of mastocytosis associated with a transmembrane c-kit mutation and response to imatinibCem Akin
Laboratory of Allergic Diseases, National Instititute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
Blood 103:3222-5. 2004.... - Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxisCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health NIH, Bethesda, MD, USA
Blood 110:2331-3. 2007..This intramural clinical trial was conducted in 2003 and 2004 and was registered at (http://clinicalcenter.nih.gov) with a study number 03-I-0010. Since the study is now closed, it is no longer available online... - Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsivenessAmy D Klion
Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
Blood 101:4660-6. 2003..In summary, elevated serum tryptase appears to be a sensitive marker of a myeloproliferative variant of HES that is characterized by tissue fibrosis, poor prognosis, and imatinib responsiveness... - Analysis of the lineage relationship between mast cells and basophils using the c-kit D816V mutation as a biologic signatureCan N Kocabas
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases/NIH, Bethesda, MD 20892, USA
J Allergy Clin Immunol 115:1155-61. 2005..These results argue against the presence of a bilineage-restricted committed progenitor for mast cells and basophils... - Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathologyKnut Brockow
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892-1881, USA
J Am Acad Dermatol 48:508-16. 2003..CONCLUSION: An examination of the extent and density of cutaneous lesions in adults helps identify those with more extensive extracutaneous disease and, thus, requiring a more thorough evaluation... - Tryptase haplotype in mastocytosis: relationship to disease variant and diagnostic utility of total tryptase levelsCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892, USA
Clin Immunol 123:268-71. 2007..Total and mature tryptase levels positively correlated with disease severity, as well as prothrombin time and partial thromboplastin time, and negatively correlated with the hemoglobin concentration... - Gene expression analysis in mastocytosis reveals a highly consistent profile with candidate molecular markersCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases (NIAID, Bethesda, MD 20892-1881, USA
J Allergy Clin Immunol 112:1162-70. 2003.... - Elevated tryptase levels are associated with greater bone density in a cohort of patients with mastocytosisNataliya M Kushnir-Sukhov
Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, MD 20892 1881, USA
Int Arch Allergy Immunol 139:265-70. 2006..Mastocytosis is associated with a pathological increase in tissue mast cells. Associated skeletal problems include a decrease in bone density and pathological fractures... - Characterization of novel stem cell factor responsive human mast cell lines LAD 1 and 2 established from a patient with mast cell sarcoma/leukemia; activation following aggregation of FcepsilonRI or FcgammaRIArnold S Kirshenbaum
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892-1881, USA
Leuk Res 27:677-82. 2003..Both LAD 1 and 2 release beta-hexosaminidase following FcepsilonRI or FcgammaRI aggregation. The availability of these cell lines offers an unparalleled circumstance to examine the biology of human mast cells... - An immunohistochemical study of the bone marrow lesions of systemic mastocytosis: expression of stem cell factor by lesional mast cellsCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892-1881, USA
Am J Clin Pathol 118:242-7. 2002.... - Surrogate markers of disease in mastocytosisCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
Int Arch Allergy Immunol 127:133-6. 2002..In addition, several novel markers including soluble CD117 and soluble CD25 have been identified in recent studies. The utility and the pitfalls of each of these measurements are discussed... - Generalized erythematous macules and plaques associated with flushing, repeated syncope, and refractory anemiaJoanne K Simpson
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases/NIH, Bethesda, MD 20892-1908, USA
J Am Acad Dermatol 46:588-90. 2002 - Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of diseaseKnut Brockow
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892-1881, USA
Arch Dermatol 138:785-90. 2002..In contrast, regression of UP in patients with indolent SM parallels a decrease in disease intensity, although bone marrow findings of indolent SM remain... - Clonality and molecular pathogenesis of mastocytosisCem Akin
University of Michigan, Department of Internal Medicine, Division of Allergy and Immunology, 1150 West Medical Center Drive, Ann Arbor, MI 48109 0638, USA
Acta Haematol 114:61-9. 2005..Improved knowledge of the mechanisms causing pathological mast cell growth will lead to the discovery of novel treatment options including drugs targeting the mutated Kit protein... - Molecular diagnosis of mast cell disorders: a paper from the 2005 William Beaumont Hospital Symposium on Molecular PathologyCem Akin
University of Michigan, 4220 D MSRB 3, Box 0638, 1150 West Medical Center Dr, Ann Arbor, MI 48109 0638, USA
J Mol Diagn 8:412-9. 2006..This article reviews diagnostic and therapeutic implications of c-kit mutations as well as other less common molecular abnormalities observed in mast cell disease... - Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatmentCem Akin
Department of Internal Medicine, University of Michigan, 4220 D MSRB 3, Box 0638, 1150 West Medical Center Drive, Ann Arbor, MI 48109 0638, USA
Curr Allergy Asthma Rep 6:282-8. 2006..Flow cytometric analysis of bone marrow mast cells is therefore a sensitive method of diagnosis of mast cell disease and is expected to find increasing use in determining response to emerging mast cell cytoreductive therapies... - Evidence for the involvement of a hematopoietic progenitor cell in systemic mastocytosis from single-cell analysis of mutations in the c-kit geneA Selim Yavuz
Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases NIAMS, and Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases NIAID, National Institutes of Health, Bethesda, MD 20892, USA
Blood 100:661-5. 2002.... - Systemic mastocytosisCem Akin
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
Annu Rev Med 55:419-32. 2004..These findings are being used in formulating diagnostic criteria as well as designing novel treatment approaches to the disease... - Levels of mast-cell growth factors in plasma and in suction skin blister fluid in adults with mastocytosis: correlation with dermal mast-cell numbers and mast-cell tryptaseKnut Brockow
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892-1881, USA
J Allergy Clin Immunol 109:82-8. 2002.... 
Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2-PDGFRB fusionIdoya Lahortiga
Human Genome Laboratory, Department of Molecular and Developmental Genetics, VIB, Leuven, Belgium
Haematologica 93:49-56. 2008..We report an unusual case of a patient presenting with peripheral basophilia and systemic mastocytosis in whom cytogenetic analysis revealed a t(4;5)(q21.1;q31.3)...- The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutationsYongsheng Ma
Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
Blood 99:1741-4. 2002..Furthermore, these results help establish a general paradigm whereby classification of mutations affecting oncogenic enzymes as RT or EST may be useful in predicting tumor sensitivity or resistance to inhibitory drugs... - Mastocytosis: advances in diagnosis and treatmentSusan I Hungness
Department of Internal Medicine, Division of Allergy and Clinical Immunology, University of Michigan, 5520 B, MSRB 1, Box 0600, 1150 W Medical Center Drive, Ann Arbor, MI 48109 0600, USA
Curr Allergy Asthma Rep 7:248-54. 2007..Efficacy of newer generation tyrosine inhibitors in mast cell disease is currently being evaluated... - EXEL-0862, a novel tyrosine kinase inhibitor, induces apoptosis in vitro and ex vivo in human mast cells expressing the KIT D816V mutationJingxuan Pan
The University of Texas M D Anderson Cancer Center, Unit 428, 1515 Holcombe Blvd, Houston, TX 77030, USA
Blood 109:315-22. 2007..We conclude that EXEL-0862 is active against KIT activation loop mutants and is a promising candidate for the treatment of patients with SM and other KIT-driven malignancies harboring active site mutations... - The identification and characterisation of novel KIT transcripts in aggressive mast cell malignancies and normal CD34+ cellsOzden Ozer
Section of Hematopathology, Department of Pathology, The University of Chicago, Chicago, IL 60637, USA
Leuk Lymphoma 49:1567-77. 2008..Our discovery of novel KIT transcripts underscores the importance of analysing entire protein encoding regions when studying genes of interest... - Effects of AMN107, a novel aminopyrimidine tyrosine kinase inhibitor, on human mast cells bearing wild-type or mutated codon 816 c-kitSrdan Verstovsek
Department of Leukemia, University of Texas M D Anderson Cancer Center, Unit 428, 1515 Holcombe Blvd, Houston, TX 77030, United States
Leuk Res 30:1365-70. 2006.... - 17-Allylamino-17-demethoxygeldanamycin (17-AAG) is effective in down-regulating mutated, constitutively activated KIT protein in human mast cellsGerard Fumo
National Cancer Institute, Cell and Cancer Biology Branch, 9610 Medical Center Dr, Ste 300, Rockville, MD 20850, USA
Blood 103:1078-84. 2004..These data provide compelling evidence that 17-AAG may be effective in the treatment of c-kit-related diseases including mastocytosis, GISTs, mast cell leukemia, subtypes of acute myelogenous leukemia, and testicular cancer... - Multilineage hematopoietic involvement in systemic mastocytosisCem Akin
Leuk Res 27:877-8. 2003 - Diagnosis and treatment of systemic mastocytosis: state of the artPeter Valent
Department of Internal Medicine I, Division of Haematology, University of Vienna, Austria
Br J Haematol 122:695-717. 2003 - Mast cell proliferative disorders: current view on variants recognized by the World Health OrganizationPeter Valent
Department of Internal Medicine 1, Division of Hematology and Hemostaseology, University of Vienna, Wahringer Gurtel 18 20, Vienna, Austria
Hematol Oncol Clin North Am 17:1227-41. 2003..In patients with SM-AHNMD, the SM should be treated as if no AHNMD is present, and the AHNMD should be treated as if no SM had been diagnosed... - Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosisSrdan Verstovsek
Leukemia Department, M D Anderson Cancer Center, Houston, Texas 77030, USA
Clin Cancer Res 14:3906-15. 2008.... - Mastocytosis: pathology, genetics, and current options for therapyPeter Valent
Department of Internal Medicine I, Medical Univeristy of Vienna, Austria
Leuk Lymphoma 46:35-48. 2005..Examples include the use of STI571 in patients with SM plus hypereosinophilic syndrome (SM-HES) and the FIPL1/PDGFRA fusion gene target, or chemotherapy for eradication of AML in patients with SM-AML... - Mastocytosis and allergyMatthew Greenhawt
Division of Allergy and Immunology, Department of Internal Medicine, University of Michigan, School of Medicine, Ann Arbor, Michigan 48109, USA
Curr Opin Allergy Clin Immunol 7:387-92. 2007..To illustrate features of allergy in mastocytosis... - Current options in the treatment of mast cell mediator-related symptoms in mastocytosisLuis Escribano
Unidad de Mastocitosis, Laboratorio K Frank Austen, Hospital Ramon y Cajal, Red Española de Mastocitosis, Madrid, Spain
Inflamm Allergy Drug Targets 5:61-77. 2006.... - Smouldering mastocytosis: a novel subtype of systemic mastocytosis with slow progressionPeter Valent
Department of Internal Medicine I, Division of Hematology, University of Vienna, Austria
Int Arch Allergy Immunol 127:137-9. 2002..In the present article, we discuss clinical and laboratory findings in smouldering SM and review the current literature. In addition, the pathophysiology of this novel subtype of SM is discussed... - Dasatinib (BMS-354825) inhibits KITD816V, an imatinib-resistant activating mutation that triggers neoplastic growth in most patients with systemic mastocytosisNeil P Shah
Division of Hematology/Oncology, The David Geffen School of Medicine at University of California-Los Angeles (UCLA, CA, USA
Blood 108:286-91. 2006..Moreover, dasatinib may be of clinical utility in other disease settings driven by activating KIT mutations... - Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteriaPeter Valent
Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Wahringer Gurtel 18 20, A 1090, Vienna, Austria
Leuk Res 27:635-41. 2003..This confirms clinical activity in some patients for this drug-combination, but also points to the need to search for more effective strategies in the treatment of patients with aggressive mast cell disorders...