mast cell leukemia

Summary

Summary: A form of systemic mastocytosis (MASTOCYTOSIS, SYSTEMIC) characterized by the presence of large numbers of tissue MAST CELLS in the peripheral blood without skin lesions. It is a high-grade LEUKEMIA disease with bone marrow smear of >20% MAST CELLS, multi-organ failure and a short survival.

Top Publications

  1. Valent P. Mast cell leukemia--a rare form of myeloid leukemia. Wien Klin Wochenschr. 2002;114:173-4 pubmed
  2. Aichberger K, Sperr W, Gleixner K, Kretschmer A, Valent P. Treatment responses to cladribine and dasatinib in rapidly progressing aggressive mastocytosis. Eur J Clin Invest. 2008;38:869-73 pubmed publisher
    ..Cladribine (2CdA) and dasatinib are two drugs that counteract the in vitro growth of neoplastic mast cells in SM. However, only little is known about the in vivo effects of these drugs in SM...
  3. Kitamura Y, Hirotab S. Kit as a human oncogenic tyrosine kinase. Cell Mol Life Sci. 2004;61:2924-31 pubmed
    ..The interrelationship between the type of Kit gain-of-function mutations and the therapeutic effect of imatinib mesylate has been well characterized in GISTs. Kit is interesting from both a biological and clinical view-point...
  4. Guo X, Schrader K, Xu Y, Schrader J. Expression of a constitutively active mutant of M-Ras in normal bone marrow is sufficient for induction of a malignant mastocytosis/mast cell leukemia, distinct from the histiocytosis/monocytic leukemia induced by expression of activated H-Ras. Oncogene. 2005;24:2330-42 pubmed
    ..Thus, expression of either activated M-Ras or H-Ras in normal hematopoietic cells was sufficient for transformation but each resulted in the generation of distinct lineages of cells...
  5. Wiesner S, Jones J, Hasz D, Largaespada D. Repressible transgenic model of NRAS oncogene-driven mast cell disease in the mouse. Blood. 2005;106:1054-62 pubmed
    ..transgenic animals uniformly develop a disease similar to human aggressive systemic mastocytosis (ASM) or mast cell leukemia (MCL) when they are between 2 and 4 months of age...
  6. Drexler H, MacLeod R. Malignant hematopoietic cell lines: in vitro models for the study of mast cell leukemia. Leuk Res. 2003;27:671-6 pubmed
    ..group of entities characterized by abnormal proliferation and accumulation of mast cells, including mast cell leukemia. Progress in understanding the (patho)biology of mast cells has been hindered by the lack of genuine model ..
  7. Furitsu T, Tsujimura T, Tono T, Ikeda H, Kitayama H, Koshimizu U, et al. Identification of mutations in the coding sequence of the proto-oncogene c-kit in a human mast cell leukemia cell line causing ligand-independent activation of c-kit product. J Clin Invest. 1993;92:1736-44 pubmed
    ..In a human mast cell leukemia cell line HMC-1, c-kitR was found to be constitutively phosphorylated on tyrosine, activated, and associated ..
  8. Chen T, Chen J, Huang W, Su W, Tsao C. Rapid engraftment of mast cells of donor origin in a case of acute myeloid leukemia with mast cell leukemia after allogeneic stem cell transplantation. Bone Marrow Transplant. 2003;32:111-4 pubmed
    ..We report a case of acute myeloid leukemia (AML) with t(8;21) and mast cell leukemia (MCL) in which the mastocytosis persisted after standard chemotherapy and allogeneic stem cell ..
  9. Kanakura Y, Furitsu T, Tsujimura T, Butterfield J, Ashman L, Ikeda H, et al. Activating mutations of the c-kit proto-oncogene in a human mast cell leukemia cell line. Leukemia. 1994;8 Suppl 1:S18-22 pubmed
    ..In a human mast cell leukemia cell line (HMC-1), KitR was found to be constitutively phosphorylated on tyrosine, activated and associated ..

More Information

Publications77

  1. Penack O, Sotlar K, Noack F, Horny H, Thiel E, Notter M. Cladribine therapy in a patient with an aleukemic subvariant of mast cell leukemia. Ann Hematol. 2005;84:692-3 pubmed
  2. Yang Y, Li L, Wong G, Krilis S, Madhusudhan M, Sali A, et al. RasGRP4, a new mast cell-restricted Ras guanine nucleotide-releasing protein with calcium- and diacylglycerol-binding motifs. Identification of defective variants of this signaling protein in asthma, mastocytosis, and mast cell leukemia patients and . J Biol Chem. 2002;277:25756-74 pubmed
    ..HMC-1 leukemia cells differentiated and underwent granule maturation when induced to express a normal form of RasGRP4. Thus, RasGRP4 plays an important role in the final stages of MC development...
  3. Gotlib J, Berube C, Growney J, Chen C, George T, Williams C, et al. Activity of the tyrosine kinase inhibitor PKC412 in a patient with mast cell leukemia with the D816V KIT mutation. Blood. 2005;106:2865-70 pubmed
    ..Limited treatment options exist for aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL)...
  4. Demehri S, Corbin A, Loriaux M, Druker B, Deininger M. Establishment of a murine model of aggressive systemic mastocytosis/mast cell leukemia. Exp Hematol. 2006;34:284-8 pubmed
    Aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL) are rare but devastating diseases, for which no effective specific therapy is currently available...
  5. Kimura N, Shiraishi S, Mizunashi K, Ohtsu H, Kimura I. Synaptotagmin I expression in mast cells of normal human tissues, systemic mast cell disease, and a human mast cell leukemia cell line. J Histochem Cytochem. 2001;49:341-6 pubmed
    ..The tumor cells of systemic mast cell disease (SMCD) and a human mast cell leukemia cell line (HMC-1) were also examined...
  6. Kajiguchi T, Lee S, Lee M, Trepel J, Neckers L. KIT regulates tyrosine phosphorylation and nuclear localization of beta-catenin in mast cell leukemia. Leuk Res. 2008;32:761-70 pubmed
    ..KIT protein, are characteristic of human mast cell disease and are believed to play a central role in mast cell leukemia oncogenesis, proliferation and survival...
  7. Valentini C, Rondoni M, Pogliani E, Van Lint M, Cattaneo C, Marbello L, et al. Mast cell leukemia: a report of ten cases. Ann Hematol. 2008;87:505-8 pubmed publisher
  8. Le Gall M, Crépin R, Neiveyans M, Auclair C, Fan Y, Zhou Y, et al. Neutralization of KIT Oncogenic Signaling in Leukemia with Antibodies Targeting KIT Membrane Proximal Domain 5. Mol Cancer Ther. 2015;14:2595-605 pubmed publisher
    ..KIT-activating mutations are major oncogenic drivers in subsets of acute myeloid leukemia (AML), in mast cell leukemia, and in gastrointestinal stromal tumors (GIST)...
  9. Zheng Y, Nong L, Liang L, Wang W, Li T. De novo mast cell leukemia without CD25 expression and KIT mutations: a rare case report in a 13-year-old child. Diagn Pathol. 2018;13:14 pubmed publisher
    b>Mast cell leukemia (MCL) is a very rare form of systemic mastocytosis (SM) and accounts for less than 0.5% of all mastocytosis...
  10. Azaña J, Torrelo A, Matito A. Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment. Actas Dermosifiliogr. 2016;107:15-22 pubmed publisher
    ..mastocytosis (SM), aggressive SM, SM with an associated clonal hematological non-mast cell lineage disease, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma. The two latest systemic variants are rare...
  11. Kirshenbaum A, Akin C, Wu Y, Rottem M, Goff J, Beaven M, et al. 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. Leuk Res. 2003;27:677-82 pubmed
    ..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...
  12. Yang M, Pan Z, Huang K, Büsche G, Feuerhake F, Chaturvedi A, et al. Activation of TRKA receptor elicits mastocytosis in mice and is involved in the development of resistance to KIT-targeted therapy. Oncotarget. 2017;8:73871-73883 pubmed publisher
    ..HMC-1 mast cell leukemia cells that are resistant to KIT inhibition induced by TRKA activation show reactivation of MAPK/ERK (..
  13. Wang R, Ward D, Dunn P, Chang C. Acute mast cell leukemia associated with t(4;5)(q21;q33). Hum Pathol. 2017;67:198-204 pubmed publisher
    ..best of our knowledge, this manuscript describes clinical and pathologic findings of the first case of acute mast cell leukemia harboring t(4;5)(q21;q33), compatible with fusion of the PDGFRB gene to a rare partner, PRKG2...
  14. Prasad P, Yanagihara A, Small Howard A, Turner H, Stokes A. Secretogranin III directs secretory vesicle biogenesis in mast cells in a manner dependent upon interaction with chromogranin A. J Immunol. 2008;181:5024-34 pubmed
    ..SgIII function in mast cells is unlikely to be limited to its partnership with CgA, as our interaction trap analysis suggests that SgIII has multiple binding partners, including the mast cell ion channel TRPA1. ..
  15. Jawhar M, Schwaab J, Meggendorfer M, Naumann N, Horny H, Sotlar K, et al. The clinical and molecular diversity of mast cell leukemia with or without associated hematologic neoplasm. Haematologica. 2017;102:1035-1043 pubmed publisher
    b>Mast cell leukemia is a rare variant of advanced systemic mastocytosis characterized by at least 20% of mast cells in a bone marrow smear...
  16. Krop M, Garrelds I, de Bruin R, van Gool J, Fisher N, Hollenberg N, et al. Aliskiren accumulates in Renin secretory granules and binds plasma prorenin. Hypertension. 2008;52:1076-83 pubmed publisher
    ..Aliskiren also binds to prorenin. This allows its detection as renin, and might explain, in part, the renin rise during renin inhibition. ..
  17. Sperr W, Escribano L, Jordan J, Schernthaner G, Kundi M, Horny H, et al. Morphologic properties of neoplastic mast cells: delineation of stages of maturation and implication for cytological grading of mastocytosis. Leuk Res. 2001;25:529-36 pubmed
    ..A percentage of MC>or=20% was invariably associated with the diagnosis 'mast cell leukemia'...
  18. Lukina E, Solov eva T. [Mast cell leukemia]. Ter Arkh. 2002;74:67-9 pubmed
  19. Ustun C, Arock M, Kluin Nelemans H, Reiter A, Sperr W, George T, et al. Advanced systemic mastocytosis: from molecular and genetic progress to clinical practice. Haematologica. 2016;101:1133-1143 pubmed
    ..systemic mastocytosis with an associated hematologic, clonal non mast cell-lineage disease, and mast cell leukemia. Whereas patients with indolent systemic mastocytosis have a near normal life expectancy, patients with ..
  20. Bibi S, Zhang Y, Hugonin C, Mangean M, He L, Wedeh G, et al. A new humanized in vivo model of KIT D816V+ advanced systemic mastocytosis monitored using a secreted luciferase. Oncotarget. 2016;7:82985-83000 pubmed publisher
    ..Patient with advanced forms of systemic mastocytosis, such as aggressive systemic mastocytosis or mast cell leukemia, are poorly treated to date...
  21. Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O, et al. Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future. Cancer Res. 2017;77:1261-1270 pubmed publisher
    ..mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and ..
  22. Carrasco Cubero C, Chamizo Carmona E. Multiple vertebral fractures in young man as first manifestation of systemic mastocytosis. Reumatol Clin. 2017;13:107-109 pubmed publisher
    ..clinical picture varies from asymptomatic forms (indolent) to a highly aggressive form with a very short (mast cell leukemia) survival...
  23. Andrade M, Hiragun T, Beaven M. Dexamethasone suppresses antigen-induced activation of phosphatidylinositol 3-kinase and downstream responses in mast cells. J Immunol. 2004;172:7254-62 pubmed
  24. Samorapoompichit P, Steiner M, Lucas T, Wachtler F, Schedled A, Sperr W, et al. Induction of apoptosis in the human mast cell leukemia cell line HMC-1 by various antineoplastic drugs. Leuk Lymphoma. 2003;44:509-15 pubmed
    b>Mast cell leukemia (MCL) is a rare disorder characterized by rapid disease progression, resistance against conventional cytoreductive drugs, and short survival...
  25. Lin J, Lachmann E, Nagler W. Low back pain and myalgias in acute and relapsed mast cell leukemia: a case report. Arch Phys Med Rehabil. 2002;83:860-3 pubmed
    b>Mast cell leukemia is a rare, severe disease that may manifest through an array of clinical presentations, including vasomotor flushing and hypotension...
  26. Ashman L, Ferrao P, Cole S, Cambareri A. Effects of mutant c-kit in early myeloid cells. Leuk Lymphoma. 2000;37:233-43 pubmed
    ..Thus the extent to which c-Kit mutations contribute to malignancies of early myeloid phenotype remains unknown, and resolution of this issue is complicated by the heterogeneity of this family of diseases...
  27. Fodinger M, Mannhalter C. Molecular genetics and development of mast cells: implications for molecular medicine. Mol Med Today. 1997;3:131-7 pubmed
  28. Horny H, Sotlar K, Valent P. Mastocytosis: state of the art. Pathobiology. 2007;74:121-32 pubmed
    ..particular those with CM and ISM, remain in an indolent stage over many years or even decades, while others, in particular those with aggressive SM, SM-AHNMD, or mast cell leukemia, show a progressive course, usually with a fatal outcome.
  29. Alexandrakis M, Letourneau R, Kempuraj D, Kandere Grzybowska K, Huang M, Christodoulou S, et al. Flavones inhibit proliferation and increase mediator content in human leukemic mast cells (HMC-1). Eur J Haematol. 2003;71:448-54 pubmed
    ..Some flavonoids, like quercetin, inhibit the growth of certain malignant cells in culture. Quercetin also inhibits histamine release and induces accumulation of secretory granules in rat basophilic leukemia cells...
  30. Pandey V, Mihara S, Fensome Green A, Bolsover S, Cockcroft S. Monomeric IgE stimulates NFAT translocation into the nucleus, a rise in cytosol Ca2+, degranulation, and membrane ruffling in the cultured rat basophilic leukemia-2H3 mast cell line. J Immunol. 2004;172:4048-58 pubmed
    ..The mechanism of activation is likely to be via aggregation of the FcepsilonR1 because activation by IgE can be inhibited with monovalent hapten...
  31. Spyridonidis A, Thomas A, Bertz H, Zeiser R, Schmitt Gräff A, Lindemann A, et al. Evidence for a graft-versus-mast-cell effect after allogeneic bone marrow transplantation. Bone Marrow Transplant. 2004;34:515-9 pubmed
    b>Mast cell leukemia (MCL) is a rare form of aggressive mastocytosis with a reported median survival below 6 months. Casuistic reports suggest the effectiveness of allogeneic bone marrow transplantation (BMT) for MCL...
  32. Escribano L, Diaz Agustin B, Bellas C, Navalón R, Nunez R, Sperr W, et al. Utility of flow cytometric analysis of mast cells in the diagnosis and classification of adult mastocytosis. Leuk Res. 2001;25:563-70 pubmed
    ..Such observations would support the need to include the immunophenotypic analysis of MC in the diagnosis of mastocytosis...
  33. Maslak P. Mast cell leukemia. Blood. 2003;101:789 pubmed
  34. Horny H, Sotlar K, Stellmacher F, Krokowski M, Agis H, Schwartz L, et al. The tryptase positive compact round cell infiltrate of the bone marrow (TROCI-BM): a novel histopathological finding requiring the application of lineage specific markers. J Clin Pathol. 2006;59:298-302 pubmed
  35. Jordan J, Walchshofer S, Jurecka W, Mosberger I, Sperr W, Wolff K, et al. Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: evidence for expression of CD2, CD117/Kit, and bcl-x(L). Hum Pathol. 2001;32:545-52 pubmed
    ..serial bone marrow (bm) sections of 22 patients with mastocytosis (systemic indolent mastocytosis, n = 19; mast cell leukemia [MCL], n = 1; isolated bm mastocytosis, n = 2) were analyzed by immunohistochemistry using antibodies ..
  36. Lin T, Bear M, Du Z, Foley K, Ying W, Barsoum J, et al. The novel HSP90 inhibitor STA-9090 exhibits activity against Kit-dependent and -independent malignant mast cell tumors. Exp Hematol. 2008;36:1266-77 pubmed publisher
    ..The purpose of this study was to evaluate a novel HSP90 inhibitor, STA-9090, against wild-type (WT) and mutant Kit in canine bone marrow-derived cultured mast cells (BMCMCs), malignant mast cell lines, and fresh malignant mast cells...
  37. Garton A, Crew A, Franklin M, Cooke A, Wynne G, Castaldo L, et al. OSI-930: a novel selective inhibitor of Kit and kinase insert domain receptor tyrosine kinases with antitumor activity in mouse xenograft models. Cancer Res. 2006;66:1015-24 pubmed
  38. Valent P, Horny H, Escribano L, Longley B, Li C, Schwartz L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25:603-25 pubmed
    ..clonal hematologic non-mast cell lineage disease (AHNMD), aggressive systemic mastocytosis (ASM), and mast cell leukemia (MCL). Patients with ISM usually have maculopapular skin lesions and a good prognosis...
  39. Penack O, Thiel E, Notter M. Mast cell leukaemia. Br J Haematol. 2005;131:416 pubmed
  40. Horny H, Krokowski M, Feller A, Hintze G, Sotlar K, Valent P. [Aleukemic mast cell leukemia (formerly: "malignant mastocytosis"): an extremely rare form of leukemia. A case report and simultaneously a contribution to revised classification of mastocytosis]. Wien Klin Wochenschr. 2002;114:222-8 pubmed
    ..While cutaneous mastocytosis is relatively frequent mast cell leukemia belongs to the rarest forms of human leukemia...
  41. Cai X, Shen G, Dat N, Kang O, Lee Y, Lee J, et al. Inhibitory effect of kaurane type diterpenoids from Acanthopanax koreanum on TNF-alpha secretion from trypsin-stimulated HMC-1 cells. Arch Pharm Res. 2003;26:731-4 pubmed
    ..Among the isolated compounds 16alphaH,17-isovaleryloxy-ent-kauran-19-oic acid (1) showed potent inhibitory activity (IC50 value, 16.2 uM) on TNF-alpha secretion from HMC-1, a trypsin-stimulated human leukemic mast cell line...
  42. Ranieri G, Passantino L, Patruno R, Passantino G, Jirillo F, Catino A, et al. The dog mast cell tumour as a model to study the relationship between angiogenesis, mast cell density and tumour malignancy. Oncol Rep. 2003;10:1189-93 pubmed
    ..These data indicate that angiogenesis and MCD are significantly correlated in MCTs progression...
  43. Kang O, Jeong H, Kim D, Choi S, Kim T, Nah Y, et al. Trypsin induces tumour necrosis factor-alpha secretion from a human leukemic mast cell line. Cell Biochem Funct. 2003;21:161-7 pubmed
    ..These results show that trypsin may induce TNF-alpha secretion following activation of ERK via both PAR2 and PAR4 on HMC-1 cells...
  44. Gleixner K, Mayerhofer M, Aichberger K, Derdak S, Sonneck K, Bohm A, et al. PKC412 inhibits in vitro growth of neoplastic human mast cells expressing the D816V-mutated variant of KIT: comparison with AMN107, imatinib, and cladribine (2CdA) and evaluation of cooperative drug effects. Blood. 2006;107:752-9 pubmed
    In most patients with systemic mastocytosis (SM), including aggressive SM and mast cell leukemia (MCL), neoplastic cells express the oncogenic KIT mutation D816V...
  45. Bocek P, Guthmann M, Pecht I. Analysis of the genes encoding the mast cell function-associated antigen and its alternatively spliced transcripts. J Immunol. 1997;158:3235-43 pubmed
    ..The transcription of the MAFA gene was detected in normal rat lungs, where both transmembranal and soluble MAFA appear to be expressed. Lung immunohistochemical analysis further suggests that MAFA expression is restricted to mast cells...
  46. Samorapoompichit P, Schernthaner G, Worda C, Wimazal F, Krauth M, Sperr W, et al. Evaluation of neoplastic human mast cells by tryptase-immunoelectron microscopy. Histopathology. 2006;48:247-57 pubmed
    ..To analyse and characterize the ultrastructural morphology of normal tissue mast cells (MC) and neoplastic bone marrow MC...
  47. Krauth M, Agis H, Aichberger K, Simonitsch Klupp I, Müllauer L, Mayerhofer M, et al. Immunohistochemical detection of histidine decarboxylase in neoplastic mast cells in patients with systemic mastocytosis. Hum Pathol. 2006;37:439-47 pubmed
    ..Histidine decarboxylase should therefore be considered as a new MC marker in the screen panel of antigens used to diagnose high-grade MC malignancies...
  48. Krishnaswamy G, Martin R, Walker E, Li C, Hossler F, Hall K, et al. Moraxella catarrhalis induces mast cell activation and nuclear factor kappa B-dependent cytokine synthesis. Front Biosci. 2003;8:a40-7 pubmed
    ..Human mast cell leukemia cells (HMC-1) were activated with either phorbol myristate acetate (PMA) and calcium ionophore or with ..
  49. Kang O, Choi Y, Park H, Lee J, Kim D, Choi S, et al. Inhibition of trypsin-induced mast cell activation by water fraction of Lonicera japonica. Arch Pharm Res. 2004;27:1141-6 pubmed
    ..However, LJ did not affect the trypsin activity even 1000 microg/mL. These results indicate that LJ may inhibit trypsin-induced mast cell activation through the inhibition of ERK phosphorylation than the inhibition of trypsin activity...
  50. Donadio A, Motzer R, Bajorin D, Kantoff P, Sheinfeld J, Houldsworth J, et al. Chemotherapy for teratoma with malignant transformation. J Clin Oncol. 2003;21:4285-91 pubmed
    ..We report that chemotherapy has a role in selected patients with MT, determined by cell type...
  51. Ozer O, Zhao Y, Ostler K, Akin C, Anastasi J, Vardiman J, et al. The identification and characterisation of novel KIT transcripts in aggressive mast cell malignancies and normal CD34+ cells. Leuk Lymphoma. 2008;49:1567-77 pubmed publisher
    ..b>Mast cell leukemia (MCL) is the most aggressive mast cell neoplasm, but has not been well studied due to its rarity...
  52. Ashman L, Ferrao P, Cole S, Cambareri A. Effects of mutant c-Kit in early myeloid cells. Leuk Lymphoma. 1999;34:451-61 pubmed
    ..Thus the extent to which c-Kit mutations contribute to malignancies of early myeloid phenotype remains unknown, and resolution of this issue is complicated by the heterogeneity of this family of diseases...
  53. Breuer W, Stahr K, Majzoub M, Hermanns W. Bone-marrow changes in infectious diseases and lymphohaemopoietic neoplasias in dogs and cats--a retrospective study. J Comp Pathol. 1998;119:57-66 pubmed
    ..To the extent that the bone marrow showed any changes in FIV and CDV infections, they were mostly reactive and not pathognomonic...
  54. Sperr W, Horny H, Lechner K, Valent P. Clinical and biologic diversity of leukemias occurring in patients with mastocytosis. Leuk Lymphoma. 2000;37:473-86 pubmed
    ..b>Mast cell leukemia (MCL) may also occur...
  55. Kitamura Y, Tsujimura T, Jippo T, Kasugai T, Kanakura Y. Regulation of development, survival and neoplastic growth of mast cells through the c-kit receptor. Int Arch Allergy Immunol. 1995;107:54-6 pubmed
    ..Signaling through the c-kit receptor tyrosine kinase (Kit) is essential for development and survival of mast cells but not of basophils. Moreover, we recently found an activation mutation of Kit in several tumor mast cell lines...
  56. Valent P, Sperr W, Samorapoompichit P, Geissler K, Lechner K, Horny H, et al. Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis. Leuk Res. 2001;25:595-602 pubmed
    ..In contrast to true mast cell leukemia (MCL), MC do not form multifocal dense infiltrates in the bone marrow...
  57. Petti F, Thelemann A, Kahler J, McCormack S, Castaldo L, Hunt T, et al. Temporal quantitation of mutant Kit tyrosine kinase signaling attenuated by a novel thiophene kinase inhibitor OSI-930. Mol Cancer Ther. 2005;4:1186-97 pubmed
    ..was used to selectively inhibit tyrosine phosphorylation downstream of juxtamembrane mutant Kit in the mast cell leukemia line HMC-1...
  58. Alexandrakis M, Kyriakou D, Seretakis D, Boucher W, Letourneau R, Kempuraj D, et al. Inhibitory effect of retinoic acid on proliferation, maturation and tryptase level in human leukemic mast cells (HMC-1). Int J Immunopathol Pharmacol. 2003;16:43-7 pubmed
    ..These results indicate that ATRA significantly inhibits both proliferation and growth as shown by the decreased intracellular tryptase levels in HMC-1. ATRA may be a useful agent in the treatment of mast cell proliferative disorders...
  59. Yoshida M, Nishikawa Y, Yamamoto Y, Doi Y, Tokairin T, Yoshioka T, et al. Mast cell leukemia with rapidly progressing portal hypertension. Pathol Int. 2009;59:817-22 pubmed publisher
    Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. A 52-year-old woman presented with urticaria-like skin symptoms, anemia, and thrombocytopenia...
  60. Lee S, Lee J, Kim D. Involvement of MITF-A, an alternative isoform of mi transcription factor, on the expression of tryptase gene in human mast cells. Exp Mol Med. 2010;42:366-75 pubmed
    ..These data suggest that MITF might play a role in regulating the transcription of tryptase gene in human mast cells...
  61. Duffy S, Leyland M, Conley E, Bradding P. Voltage-dependent and calcium-activated ion channels in the human mast cell line HMC-1. J Leukoc Biol. 2001;70:233-40 pubmed
    ..We suggest that this current may contribute to the malignant phenotype while the Ca(2+)-activated K(+) and Cl(-) currents may be involved in cell activation...
  62. Baek J, Tefferi A, Pardanani A, Li C. Immunohistochemical studies of c-kit, transforming growth factor-beta, and basic fibroblast growth factor in mast cell disease. Leuk Res. 2002;26:83-90 pubmed
    ..type I TGF-beta receptor (TGFbeta1R) was not expressed in 30% of SMCD-HD patients or in patients with mast cell leukemia, but the remaining cases of MCD showed near uniform expression...
  63. Worobec A. Treatment of systemic mast cell disorders. Hematol Oncol Clin North Am. 2000;14:659-87, vii pubmed
    ..This article presents a summary of treatment strategies for indolent and aggressive forms of mastocytosis along with a discussion of future therapeutic directions...
  64. Schernthaner G, Jordan J, Ghannadan M, Agis H, Bevec D, Nunez R, et al. Expression, epitope analysis, and functional role of the LFA-2 antigen detectable on neoplastic mast cells. Blood. 2001;98:3784-92 pubmed
    Recent data suggest that mast cells (MCs) in patients with systemic mastocytosis or mast cell leukemia express a CD2-reactive antigen...
  65. Hara M, Ono K, Wada H, Sasayama S, Matsumori A. Preformed angiotensin II is present in human mast cells. Cardiovasc Drugs Ther. 2004;18:415-20 pubmed
    ..However, their function remains unclear. In this study, preformed angiotensin II (ANG II), a potent growth factor, was found to be contained in, and released by, human mast cells...
  66. McArthur G. Splicing the way to leukemia with KIT. Leuk Lymphoma. 2008;49:1431-2 pubmed publisher
  67. Horny H, Valent P. Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings. Leuk Res. 2001;25:543-51 pubmed
  68. Guo X, Stratton L, Schrader J. Expression of activated M-Ras in hemopoietic stem cells initiates leukemogenic transformation, immortalization and preferential generation of mast cells. Oncogene. 2006;25:4241-4 pubmed