Elsevier

Human Pathology

Volume 32, Issue 5, May 2001, Pages 545-552
Human Pathology

Original Contributions
Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: Evidence for expression of CD2, CD117/Kit, and bcl-xL*

https://doi.org/10.1053/hupa.2001.24319Get rights and content

Abstract

In an attempt to identify novel diagnostic markers for mast cell (MC)–proliferative disorders, serial bone marrow (bm) sections of 22 patients with mastocytosis (systemic indolent mastocytosis, n = 19; mast cell leukemia [MCL], n = 1; isolated bm mastocytosis, n = 2) were analyzed by immunohistochemistry using antibodies against CD2, CD15, CD29, CD30, CD31, CD34, CD45, CD51, CD56, CD68R, CD117, HLA-DR, bcl-2, bcl-xL, myeloperoxidase (MPO), and tryptase. Staining results revealed expression of bcl-xL, CD68R, and tryptase in neoplastic MCs (focal dense infiltrates) in all patients. Mastocytosis infiltrates were also immunoreactive for CD45, CD117 (Kit), and HLA-DR. In most cases, the CD2 antibody produced reactivity with bm MCs in mastocytosis, whereas in control cases (reactive bm, immunocytoma, myelodysplastic syndrome), MCs were consistently CD2 negative. Expression of bcl-2 was detectable in a subset of MCs in the patient with MCL, whereas no reactivity was seen in patients with SIM or bm mastocytosis. Mastocytosis infiltrates did not react with antibodies against CD15, CD30, CD31, CD34, or MPO. In summary, our data confirm the diagnostic value of staining for tryptase, Kit, and CD68R in mastocytosis. Apart from these, CD2 may be a novel useful marker because MCs in mastocytosis frequently express this antigen, whereas MCs in other pathologic conditions are CD2 negative. HUM PATHOL 32:545-552. Copyright © 2001 by W.B. Saunders Company

Section snippets

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.

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