Mastocytosis: diverse presentations and outcomes.

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.

• 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)

Topics

• Adult
• Female
• Humans
• Mastocytosis (classification, diagnosis, therapy)
• Middle Aged
• Treatment Outcome