Abstract |
Within the general category of mastocytosis lies an array of clinical presentations with differing prognostic implications. We report 3 cases of systemic mastocytosis distinguished by novel aspects of the disease. Case 1 documents the first successful orthotopic liver transplantation in a patient with mastocytosis; case 2 depicts a potential hereditary component of mastocytosis; and case 3 documents the progression of mastocytosis with hematologic abnormality to mast cell leukemia. Future investigations, such as the early definition of c-kit receptor mutations, may provide additional insight as to the molecular basis for this heterogeneous disease and guidance for prognostic implications and targeted therapies.
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Authors | J D Pauls, J Brems, P J Pockros, A Saven, R L Wagner, R Weber, D Metcalfe, S C Christiansen |
Journal | Archives of internal medicine
(Arch Intern Med)
Vol. 159
Issue 4
Pg. 401-5
(Feb 22 1999)
ISSN: 0003-9926 [Print] United States |
PMID | 10030315
(Publication Type: Case Reports, Journal Article)
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Topics |
- Adult
- Female
- Humans
- Mastocytosis
(classification, diagnosis, therapy)
- Middle Aged
- Treatment Outcome
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