TY - JOUR
T1 - Systemic mastocytosis in the elderly
AU - Butterfield, Joseph H.
AU - Weiler, Catherine R.
PY - 2013/5
Y1 - 2013/5
N2 - "Later onset" of systemic mastocytosis (SM) has been associated with a poorer prognosis. We examined clinical and laboratory findings, associated disorders, and survival in an older mastocytosis population. After receiving Mayo Clinic Institutional Review Board approval, we identified 42 patients aged 70 years and older at the time of diagnosis of SM. Associated disorders, cytogenetic abnormalities, laboratory findings, and survival were recorded. Only 10 patients had no associated hematologic disorder. Single or multiple chromosomal abnormalities, exclusive of the KIT Asp816Val mutation, were detected in eight patients (19%). KIT Asp816Val mutation was present in 14 patients, negative in three, and not tested in 25. Slight to marked bone marrow hypercellularity was observed in 33 patients (79%). Concurrent hematologic abnormalities included chronic myelomonocytic leukemia (n=7), acute myelocytic leukemia (n=1), myelodysplastic syndrome (MDS; n=7), eosinophilia (n=7), myelofibrosis (n=1), myeloproliferative disorder (n=1), multiple myeloma (n=1), B-cell lymphoma (n=1), and thrombocytopenia (n=4). Eight patients had a hematologic disorder that preceded the diagnosis of SM. Tryptase levels were elevated in 38 of 39 patients tested. Survival from the diagnosis of SM was poor for patients with associated thrombocytopenia, leukemias, and MDS. In conclusion, patients with SM diagnosed at age 70 or older have an increased risk of secondary hematologic disorders and abnormal laboratory findings. Cytogenetic abnormalities are common, and survival is short in many SM patients with associated leukemias, MDS, or eosinophilia.
AB - "Later onset" of systemic mastocytosis (SM) has been associated with a poorer prognosis. We examined clinical and laboratory findings, associated disorders, and survival in an older mastocytosis population. After receiving Mayo Clinic Institutional Review Board approval, we identified 42 patients aged 70 years and older at the time of diagnosis of SM. Associated disorders, cytogenetic abnormalities, laboratory findings, and survival were recorded. Only 10 patients had no associated hematologic disorder. Single or multiple chromosomal abnormalities, exclusive of the KIT Asp816Val mutation, were detected in eight patients (19%). KIT Asp816Val mutation was present in 14 patients, negative in three, and not tested in 25. Slight to marked bone marrow hypercellularity was observed in 33 patients (79%). Concurrent hematologic abnormalities included chronic myelomonocytic leukemia (n=7), acute myelocytic leukemia (n=1), myelodysplastic syndrome (MDS; n=7), eosinophilia (n=7), myelofibrosis (n=1), myeloproliferative disorder (n=1), multiple myeloma (n=1), B-cell lymphoma (n=1), and thrombocytopenia (n=4). Eight patients had a hematologic disorder that preceded the diagnosis of SM. Tryptase levels were elevated in 38 of 39 patients tested. Survival from the diagnosis of SM was poor for patients with associated thrombocytopenia, leukemias, and MDS. In conclusion, patients with SM diagnosed at age 70 or older have an increased risk of secondary hematologic disorders and abnormal laboratory findings. Cytogenetic abnormalities are common, and survival is short in many SM patients with associated leukemias, MDS, or eosinophilia.
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U2 - 10.1002/ajh.23426
DO - 10.1002/ajh.23426
M3 - Article
C2 - 23436294
AN - SCOPUS:84876742896
SN - 0361-8609
VL - 88
SP - 406
EP - 408
JO - American journal of hematology
JF - American journal of hematology
IS - 5
ER -