Spectrum of associated clonal hematologic non-mast cell lineage disorders occurring in patients with systemic mastocytosis.
Systemic mastocytosis (SM) is a myeloproliferative disease affecting multipotent and/or mast cell-committed hematopoietic progenitor cells. In a significant subgroup of patients (10-35%), an associated clonal hematologic non-mast cell lineage disorder (AHNMD) occurs. These AHNMDs can be classified according to recently established WHO criteria. Most AHNMDs resemble myeloid malignancies such as acute myeloid leukemia, myeloproliferative disorders or myelodysplastic syndromes. In only a few cases, lymphoproliferative disorders are diagnosed. Patients with SM-AHNMD have a less favorable prognosis concerning survival when compared to indolent SM. No general guidelines for the treatment of patients with SM-AHNMD have been established so far. A reasonable straightforward approach may be to treat the AHNMD in those patients in the same way as if no coexisting SM exists.
Sperr
Wolfgang R
WR
Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Austria. wolfgang.r.sperr@univie.ac.at
Horny
Hans-Peter
HP
Valent
Peter
P
D019070:Cell Lineage; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000209:etiology; D008415:Mastocytosis / Q000145:classification* / Q000150:complications; D009190:Myelodysplastic Syndromes / Q000209:etiology; D009196:Myeloproliferative Disorders / Q000209:etiology