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Malignant Mastocytosis

MedGen UID:
281012
•Concept ID:
C1541840
•
Neoplastic Process
Synonyms: Malignant mastocytosis; Malignant systemic tissue mast cell disease
SNOMED CT: Malignant mastocytosis (307591004); Malignant systemic tissue mast cell disease (307591004)

Definition

A group of malignant mast cell disorders including aggressive systemic mastocytosis, mast cell leukemia, mast cell sarcoma, and systemic mastocytosis with an associated myeloid neoplasm. Individuals with advanced systemic mastocytosis have a reduced life expectancy, with median survival measured in months to years. [from NCI]

Professional guidelines

PubMed

Biology, classification and treatment of human mastocytosis.
Valent P
Wien Klin Wochenschr 1996;108(13):385-97. PMID: 8766423

Recent clinical studies

Etiology

Clinical and biologic diversity of leukemias occurring in patients with mastocytosis.
Sperr WR, Horny HP, Lechner K, Valent P
Leuk Lymphoma 2000 May;37(5-6):473-86. doi: 10.3109/10428190009058500. PMID: 11042508
Biology, classification and treatment of human mastocytosis.
Valent P
Wien Klin Wochenschr 1996;108(13):385-97. PMID: 8766423
Skeletal mastocytosis.
Andrew SM, Freemont AJ
J Clin Pathol 1993 Nov;46(11):1033-5. doi: 10.1136/jcp.46.11.1033. PMID: 8254091Free PMC Article
The spectrum of mastocytosis.
Tharp MD
Am J Med Sci 1985 Mar;289(3):119-32. doi: 10.1097/00000441-198503000-00007. PMID: 2579553
Benign and malignant mast cell proliferations. Diagnosis and separation using a pH-dependent toluidine blue stain in tissue section.
Klatt EC, Lukes RJ, Meyer PR
Cancer 1983 Mar 15;51(6):1119-24. doi: 10.1002/1097-0142(19830315)51:6<1119::aid-cncr2820510624>3.0.co;2-m. PMID: 6185213

Diagnosis

Aggressive systemic mastocytosis.
Inaoui R, Petit B, Jaccard A, Bertin P, Trèves R
Joint Bone Spine 2003 Feb;70(1):64-6. doi: 10.1016/s1297-319x(02)00015-5. PMID: 12639621
A near-haploid bone marrow karyotype in systemic mast cell disease: is it characteristic of the disease or an incidental finding?
Shekhter-Levin S, Ball E, Swerdlow SH, Li WV, Kapadia SB, Sherer ME, Wald N, Gollin SM
Cancer Genet Cytogenet 1998 Jun;103(2):124-9. doi: 10.1016/s0165-4608(97)00386-5. PMID: 9614910
Mast cell leukemia: report of a case and review of the literature.
Travis WD, Li CY, Hoagland HC, Travis LB, Banks PM
Mayo Clin Proc 1986 Dec;61(12):957-66. doi: 10.1016/s0025-6196(12)62636-6. PMID: 3095598
Functional asplenia in malignant mastocytosis.
Roth J, Brudler O, Henze E
J Nucl Med 1985 Oct;26(10):1149-52. PMID: 4045559
The spectrum of mastocytosis.
Tharp MD
Am J Med Sci 1985 Mar;289(3):119-32. doi: 10.1097/00000441-198503000-00007. PMID: 2579553

Therapy

Ultraviolet irradiation induces apoptosis in human immature, but not in skin mast cells.
Guhl S, Hartmann K, Tapkenhinrichs S, Smorodchenko A, Grützkau A, Henz BM, Zuberbier T
J Invest Dermatol 2003 Oct;121(4):837-44. doi: 10.1046/j.1523-1747.2003.12480.x. PMID: 14632203
Aggressive systemic mastocytosis.
Inaoui R, Petit B, Jaccard A, Bertin P, Trèves R
Joint Bone Spine 2003 Feb;70(1):64-6. doi: 10.1016/s1297-319x(02)00015-5. PMID: 12639621
Clinical and biologic diversity of leukemias occurring in patients with mastocytosis.
Sperr WR, Horny HP, Lechner K, Valent P
Leuk Lymphoma 2000 May;37(5-6):473-86. doi: 10.3109/10428190009058500. PMID: 11042508
The spectrum of mastocytosis.
Tharp MD
Am J Med Sci 1985 Mar;289(3):119-32. doi: 10.1097/00000441-198503000-00007. PMID: 2579553

Prognosis

Aggressive systemic mastocytosis.
Inaoui R, Petit B, Jaccard A, Bertin P, Trèves R
Joint Bone Spine 2003 Feb;70(1):64-6. doi: 10.1016/s1297-319x(02)00015-5. PMID: 12639621
A near-haploid bone marrow karyotype in systemic mast cell disease: is it characteristic of the disease or an incidental finding?
Shekhter-Levin S, Ball E, Swerdlow SH, Li WV, Kapadia SB, Sherer ME, Wald N, Gollin SM
Cancer Genet Cytogenet 1998 Jun;103(2):124-9. doi: 10.1016/s0165-4608(97)00386-5. PMID: 9614910
Skeletal mastocytosis.
Andrew SM, Freemont AJ
J Clin Pathol 1993 Nov;46(11):1033-5. doi: 10.1136/jcp.46.11.1033. PMID: 8254091Free PMC Article
Mast cell leukemia: report of a case and review of the literature.
Travis WD, Li CY, Hoagland HC, Travis LB, Banks PM
Mayo Clin Proc 1986 Dec;61(12):957-66. doi: 10.1016/s0025-6196(12)62636-6. PMID: 3095598
The spectrum of mastocytosis.
Tharp MD
Am J Med Sci 1985 Mar;289(3):119-32. doi: 10.1097/00000441-198503000-00007. PMID: 2579553

Clinical prediction guides

Cancer risk in patients with Noonan syndrome carrying a PTPN11 mutation.
Jongmans MC, van der Burgt I, Hoogerbrugge PM, Noordam K, Yntema HG, Nillesen WM, Kuiper RP, Ligtenberg MJ, van Kessel AG, van Krieken JH, Kiemeney LA, Hoogerbrugge N
Eur J Hum Genet 2011 Aug;19(8):870-4. Epub 2011 Mar 16 doi: 10.1038/ejhg.2011.37. PMID: 21407260Free PMC Article
Lymph node findings in generalized mastocytosis.
Horny HP, Kaiserling E, Parwaresch MR, Lennert K
Histopathology 1992 Nov;21(5):439-46. doi: 10.1111/j.1365-2559.1992.tb00428.x. PMID: 1452127
Immunoreactivity of normal and neoplastic human tissue mast cells.
Horny HP, Reimann O, Kaiserling E
Am J Clin Pathol 1988 Mar;89(3):335-40. doi: 10.1093/ajcp/89.3.335. PMID: 3126643
Mast cell leukemia: report of a case and review of the literature.
Travis WD, Li CY, Hoagland HC, Travis LB, Banks PM
Mayo Clin Proc 1986 Dec;61(12):957-66. doi: 10.1016/s0025-6196(12)62636-6. PMID: 3095598
Benign and malignant mast cell proliferations. Diagnosis and separation using a pH-dependent toluidine blue stain in tissue section.
Klatt EC, Lukes RJ, Meyer PR
Cancer 1983 Mar 15;51(6):1119-24. doi: 10.1002/1097-0142(19830315)51:6<1119::aid-cncr2820510624>3.0.co;2-m. PMID: 6185213

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