Original ContributionsImmunohistochemical properties of bone marrow mast cells in systemic mastocytosis: Evidence for expression of CD2, CD117/Kit, and bcl-xL*
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Patients
A total number of 22 patients (13 female, 9 male) with mastocytosis were analyzed. The patients' characteristics are summarized in Table 1.The median age at onset of disease was 33.5 years (range, 13 to 72 years). At the time of bm investigation, the median age was 46.5 years. Staging included physical examination, complete blood picture, blood chemistry, serum tryptase measurement, ultrasound of liver and spleen, and bm biopsy (histology, cytology). Serum total tryptase (normal range, 0.1 to
Clinical course
The clinical course of the patient with MCL (patient 8) was characterized by rapid multiorgan failure and short survival.18 Two of 3 AML patients (patients 4 and 11) received polychemotherapy and entered complete remission.21 However, relapses occurred, and both died of leukemic progression. Patient 17 (AML-M5) received chemotherapy and a bm transplant (in 1996) from her HLA-identical sister. This patient is still in remission. In all 3 AML patients, the AML blasts did not react with
Discussion
The histologic detection of focal dense MC infiltrates in the bm is of major diagnostic importance in (suspected) systemic mastocytosis. However, it may sometimes be difficult to discriminate between true mastocytosis, a reactive MC hyperplasia, and a myeloid neoplasm with increase in (clonal) bm MCs. In an attempt to identify novel diagnostic markers for mastocytosis, we analyzed paraffin-embedded bm sections using a panel of antibodies known to react with MC-related (and possibly
Acknowledgements
The authors thank Michaela Fuchs and Hans Semper for excellent technical assistance.
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Supported by Fonds zur Förderung der Wissenschaftlichen, Forschung in Österreich (FWF) grant P-12517.