Cem Akin

Summary

Affiliation: University of Michigan
Country: USA

Publications

  1. pmc Tryptase haplotype in mastocytosis: relationship to disease variant and diagnostic utility of total tryptase levels
    Cem Akin
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892, USA
    Clin Immunol 123:268-71. 2007
  2. pmc Molecular diagnosis of mast cell disorders: a paper from the 2005 William Beaumont Hospital Symposium on Molecular Pathology
    Cem 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
  3. pmc Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis
    Cem 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
  4. ncbi request reprint Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatment
    Cem 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
  5. ncbi request reprint Clonality and molecular pathogenesis of mastocytosis
    Cem 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
  6. pmc Demonstration that mast cells, T cells, and B cells bearing the activating kit mutation D816V occur in clusters within the marrow of patients with mastocytosis
    Marcia 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
  7. ncbi request reprint KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities
    Irina 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
  8. ncbi request reprint Effects of tyrosine kinase inhibitor STI571 on human mast cells bearing wild-type or mutated c-kit
    Cem 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
  9. ncbi request reprint IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosis
    Knut 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
  10. ncbi request reprint A novel form of mastocytosis associated with a transmembrane c-kit mutation and response to imatinib
    Cem 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

Collaborators

Detail Information

Publications44

  1. pmc Tryptase haplotype in mastocytosis: relationship to disease variant and diagnostic utility of total tryptase levels
    Cem 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...
  2. pmc Molecular diagnosis of mast cell disorders: a paper from the 2005 William Beaumont Hospital Symposium on Molecular Pathology
    Cem 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...
  3. pmc Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis
    Cem 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...
  4. ncbi request reprint Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatment
    Cem 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...
  5. ncbi request reprint Clonality and molecular pathogenesis of mastocytosis
    Cem 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...
  6. pmc Demonstration that mast cells, T cells, and B cells bearing the activating kit mutation D816V occur in clusters within the marrow of patients with mastocytosis
    Marcia 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...
  7. ncbi request reprint KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities
    Irina 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...
  8. ncbi request reprint Effects of tyrosine kinase inhibitor STI571 on human mast cells bearing wild-type or mutated c-kit
    Cem 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...
  9. ncbi request reprint IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosis
    Knut 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...
  10. ncbi request reprint A novel form of mastocytosis associated with a transmembrane c-kit mutation and response to imatinib
    Cem 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
    ....
  11. ncbi request reprint Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome
    Amy 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...
  12. ncbi request reprint 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 cells
    Arnold 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)...
  13. ncbi request reprint 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 responsiveness
    Amy 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...
  14. ncbi request reprint Analysis of the lineage relationship between mast cells and basophils using the c-kit D816V mutation as a biologic signature
    Can 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
    ..Mast cells and basophils share similar morphologic and functional properties; however, it is not known whether they are derived from a bilineage (basophil/mast cell)-restricted progenitor...
  15. ncbi request reprint Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology
    Knut 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
    ..Cutaneous involvement occurs in most patients with systemic mastocytosis...
  16. ncbi request reprint An immunohistochemical study of the bone marrow lesions of systemic mastocytosis: expression of stem cell factor by lesional mast cells
    Cem 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
    ....
  17. ncbi request reprint Mastocytosis: advances in diagnosis and treatment
    Susan 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...
  18. ncbi request reprint Evidence for the involvement of a hematopoietic progenitor cell in systemic mastocytosis from single-cell analysis of mutations in the c-kit gene
    A 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
    ....
  19. ncbi request reprint 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 FcgammaRI
    Arnold 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...
  20. ncbi request reprint Surrogate markers of disease in mastocytosis
    Cem 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...
  21. ncbi request reprint Elevated tryptase levels are associated with greater bone density in a cohort of patients with mastocytosis
    Nataliya 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...
  22. ncbi request reprint Gene expression analysis in mastocytosis reveals a highly consistent profile with candidate molecular markers
    Claudio D'Ambrosio
    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
    ..Mastocytosis is a rare clonal disorder that might be accompanied by non-mast-cell clonal hematologic disorders, such as myeloproliferative or myelodysplastic syndromes...
  23. ncbi request reprint Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease
    Knut Brockow
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892 1881, USA
    Arch Dermatol 138:785-90. 2002
    ..To determine clinical correlates of urticaria pigmentosa (UP) regression in adult patients with systemic mastocytosis (SM)...
  24. pmc Mast cell activation syndrome: Proposed diagnostic criteria
    Cem Akin
    Department of Internal Medicine, Division of Allergy and Immunology, University of Michigan, Ann Arbor, MI, USA
    J Allergy Clin Immunol 126:1099-104.e4. 2010
    ..The proposed criteria will be discussed in the context of other disorders involving mast cells or with similar presentations and as a basis for further scientific study and validation...
  25. ncbi request reprint 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 tryptase
    Knut 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
    ..Mast-cell accumulation has been observed in the skin and other organs of patients with systemic indolent mastocytosis (SM). The basis for this pathologic increase is not fully understood...
  26. ncbi request reprint The biology of Kit in disease and the application of pharmacogenetics
    Cem 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...
  27. ncbi request reprint Generalized erythematous macules and plaques associated with flushing, repeated syncope, and refractory anemia
    Joanne 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
  28. ncbi request reprint Systemic mastocytosis
    Cem 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...
  29. ncbi request reprint Mastocytosis and allergy
    Matthew 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...
  30. ncbi request reprint 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 mutations
    Yongsheng 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...
  31. ncbi request reprint EXEL-0862, a novel tyrosine kinase inhibitor, induces apoptosis in vitro and ex vivo in human mast cells expressing the KIT D816V mutation
    Jingxuan 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...
  32. doi request reprint Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2-PDGFRB fusion
    Idoya 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)...
  33. doi request reprint The identification and characterisation of novel KIT transcripts in aggressive mast cell malignancies and normal CD34+ cells
    Ozden 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...
  34. ncbi request reprint Multilineage hematopoietic involvement in systemic mastocytosis
    Cem Akin
    Leuk Res 27:877-8. 2003
  35. ncbi request reprint Diagnosis and treatment of systemic mastocytosis: state of the art
    Peter Valent
    Department of Internal Medicine I, Division of Haematology, University of Vienna, Austria
    Br J Haematol 122:695-717. 2003
  36. ncbi request reprint Mast cell proliferative disorders: current view on variants recognized by the World Health Organization
    Peter 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...
  37. ncbi request reprint 17-Allylamino-17-demethoxygeldanamycin (17-AAG) is effective in down-regulating mutated, constitutively activated KIT protein in human mast cells
    Gerard 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...
  38. ncbi request reprint Effects of AMN107, a novel aminopyrimidine tyrosine kinase inhibitor, on human mast cells bearing wild-type or mutated codon 816 c-kit
    Srdan 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
    ....
  39. doi request reprint Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis
    Srdan Verstovsek
    Leukemia Department, M D Anderson Cancer Center, Houston, Texas 77030, USA
    Clin Cancer Res 14:3906-15. 2008
    ....
  40. ncbi request reprint Dasatinib (BMS-354825) inhibits KITD816V, an imatinib-resistant activating mutation that triggers neoplastic growth in most patients with systemic mastocytosis
    Neil 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...
  41. ncbi request reprint Current options in the treatment of mast cell mediator-related symptoms in mastocytosis
    Luis Escribano
    Unidad de Mastocitosis, Laboratorio K Frank Austen, Hospital Ramon y Cajal, Red EspaƱola de Mastocitosis, Madrid, Spain
    Inflamm Allergy Drug Targets 5:61-77. 2006
    ....
  42. ncbi request reprint Smouldering mastocytosis: a novel subtype of systemic mastocytosis with slow progression
    Peter 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...
  43. ncbi request reprint Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria
    Peter 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...
  44. ncbi request reprint Mastocytosis: pathology, genetics, and current options for therapy
    Peter 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...