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- W1967977237 abstract "RationaleSystemic mastocytosis (SM) patients with later onset of disease have a poorer prognosis than younger patients with this disorder.MethodsPatients 70 years of age and older at the time of the diagnosis of SM were sorted from the Mayo Clinic Database (1990 to the present). We examined associated hematologic disorders, genetic abnormalities, bone marrow cellularity, tryptase levels and other findings.Results43 patients (17 women, 26 men, average age 76.2 years) qualified for further evaluation. 10 patients (23%) had no associated hematologic disorder. Single or multiple chromosomal abnormalities, exclusive of the KIT Asp816Val mutation, were detected in 7 of 43 patients (16%); however there was no predominant alteration. Slight to marked bone marrow hypercellularity was found in 29 patients (67%) while hypocellularity was found in 2 patients. Hematologic disorders, many with an associated additional second hematologic abnormality included CMML5, AML 1, myelodysplastic syndrome 8, eosinophilia 8, myelofibrosis1, myeloproliferative disorder 1, multiple myeloma 1, B cell lymphoma 1, and thrombocytopenia 3. Some blood disorders predated the finding of mastocytosis: thrombasthenia (2 yr), anemia (6 mo), pancytopenia (4 yr), MDS (9 yrs), MDS with RARS (2 yrs), B-cell lymphoma (5 yrs), thrombocytopenia (> 20 yrs). Tryptase levels were available for 41 patients and were elevated in 40 with the highest average value found among those with associated thrombocytopenia.ConclusionsPatients whose diagnosis of SM is established at or above age 70 have a high frequency of secondary hematologic disorders, bone marrow hypercellularity and chromosomal abnormalities. RationaleSystemic mastocytosis (SM) patients with later onset of disease have a poorer prognosis than younger patients with this disorder. Systemic mastocytosis (SM) patients with later onset of disease have a poorer prognosis than younger patients with this disorder. MethodsPatients 70 years of age and older at the time of the diagnosis of SM were sorted from the Mayo Clinic Database (1990 to the present). We examined associated hematologic disorders, genetic abnormalities, bone marrow cellularity, tryptase levels and other findings. Patients 70 years of age and older at the time of the diagnosis of SM were sorted from the Mayo Clinic Database (1990 to the present). We examined associated hematologic disorders, genetic abnormalities, bone marrow cellularity, tryptase levels and other findings. Results43 patients (17 women, 26 men, average age 76.2 years) qualified for further evaluation. 10 patients (23%) had no associated hematologic disorder. Single or multiple chromosomal abnormalities, exclusive of the KIT Asp816Val mutation, were detected in 7 of 43 patients (16%); however there was no predominant alteration. Slight to marked bone marrow hypercellularity was found in 29 patients (67%) while hypocellularity was found in 2 patients. Hematologic disorders, many with an associated additional second hematologic abnormality included CMML5, AML 1, myelodysplastic syndrome 8, eosinophilia 8, myelofibrosis1, myeloproliferative disorder 1, multiple myeloma 1, B cell lymphoma 1, and thrombocytopenia 3. Some blood disorders predated the finding of mastocytosis: thrombasthenia (2 yr), anemia (6 mo), pancytopenia (4 yr), MDS (9 yrs), MDS with RARS (2 yrs), B-cell lymphoma (5 yrs), thrombocytopenia (> 20 yrs). Tryptase levels were available for 41 patients and were elevated in 40 with the highest average value found among those with associated thrombocytopenia. 43 patients (17 women, 26 men, average age 76.2 years) qualified for further evaluation. 10 patients (23%) had no associated hematologic disorder. Single or multiple chromosomal abnormalities, exclusive of the KIT Asp816Val mutation, were detected in 7 of 43 patients (16%); however there was no predominant alteration. Slight to marked bone marrow hypercellularity was found in 29 patients (67%) while hypocellularity was found in 2 patients. Hematologic disorders, many with an associated additional second hematologic abnormality included CMML5, AML 1, myelodysplastic syndrome 8, eosinophilia 8, myelofibrosis1, myeloproliferative disorder 1, multiple myeloma 1, B cell lymphoma 1, and thrombocytopenia 3. Some blood disorders predated the finding of mastocytosis: thrombasthenia (2 yr), anemia (6 mo), pancytopenia (4 yr), MDS (9 yrs), MDS with RARS (2 yrs), B-cell lymphoma (5 yrs), thrombocytopenia (> 20 yrs). Tryptase levels were available for 41 patients and were elevated in 40 with the highest average value found among those with associated thrombocytopenia. ConclusionsPatients whose diagnosis of SM is established at or above age 70 have a high frequency of secondary hematologic disorders, bone marrow hypercellularity and chromosomal abnormalities. Patients whose diagnosis of SM is established at or above age 70 have a high frequency of secondary hematologic disorders, bone marrow hypercellularity and chromosomal abnormalities." @default.
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- W1967977237 date "2013-02-01" @default.
- W1967977237 modified "2023-10-17" @default.
- W1967977237 title "Systemic Mastocytosis in the Elderly" @default.
- W1967977237 doi "https://doi.org/10.1016/j.jaci.2012.12.1101" @default.
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