Differential expression of CD2 on neoplastic mast cells in patients with systemic mast cell disease with and without an associated clonal haematological disorder

An intense and reproducible peroxidase staining in the cutaneous mast cells of two patients with systemic mast cell disease and urticaria pigmentosa is demonstrated at the ultrastructural level. This enzyme activity was demonstrated by use of a cytochemical technique employing 3,3'- diaminobenzicine (DAB) as an oxidizable substrate, after fixation by a tannic acid-aldehyde mixture. Enzyme activity was localized in the perinuclear cisterna and strands of endoplasmic reticulum. Granules appeared unreactive. This peroxidase activity appears sensitive to fixation by aldehydes; it is inhibited by 3-amino-1,2,4-triazole (AMT) and by lack of H2O2 or DAB in the incubation medium. These characteristics are fundamentally different from the peroxidase activity of basophils, and the demonstration of this enzyme is therefore not a further argument for a common ontogenetic origin of both cells. On the other hand, the cytochemical characteristics of this enzyme are very similar to those of platelet peroxidase (P-PO), which has been connected to the synthesis by platelets of prostaglandins. Since the mast cell is known to generate prostaglandins, the relationship between the enzyme described and prostaglandin synthesis by mast cells is discussed.

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Flow Cytometric Analysis of Normal and Neoplastic Mast Cells: Role in Diagnosis and Follow-Up of Mast Cell Disease

Immunology and Allergy Clinics of North America ◽

10.1016/j.iac.2006.05.008 ◽

2006 ◽

Vol 26 (3) ◽

pp. 535-547 ◽

Cited By ~ 34

Author(s):

Luis Escribano ◽

Andres C. Garcia Montero ◽

Rosa Núñez ◽

Alberto Orfao

Keyword(s):

Mast Cell ◽

Mast Cells ◽

Flow Cytometric Analysis ◽

Cell Disease ◽

Flow Cytometric ◽

Mast Cell Disease

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The CD69 early activation molecule is overexpressed in human bone marrow mast cells from adults with indolent systemic mast cell disease

British Journal of Haematology ◽

10.1046/j.1365-2141.1999.01572.x ◽

1999 ◽

Vol 106 (2) ◽

pp. 400-405 ◽

Cited By ~ 32

Author(s):

Beatriz Díaz-Agustín ◽

Luis Escribano ◽

Pilar Bravo ◽

Sonia Herrero ◽

Rosa Nuñez ◽

...

Keyword(s):

Bone Marrow ◽

Mast Cell ◽

Mast Cells ◽

Human Bone ◽

Human Bone Marrow ◽

Cell Disease ◽

Early Activation ◽

Mast Cell Disease

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Human bone marrow mast cells from indolent systemic mast cell disease constitutively express increased amounts of the CD63 protein on their surface

Cytometry ◽

10.1002/(sici)1097-0320(19981015)34:5<223::aid-cyto3>3.0.co;2-b ◽

1998 ◽

Vol 34 (5) ◽

pp. 223-228 ◽

Cited By ~ 24

Author(s):

Luis Escribano ◽

Alberto Orfao ◽

Beatriz D�az Agust�n ◽

Carlos Cerver� ◽

Sonia Herrero ◽

...

Keyword(s):

Bone Marrow ◽

Mast Cell ◽

Mast Cells ◽

Human Bone ◽

Human Bone Marrow ◽

Cell Disease ◽

Mast Cell Disease

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Detection of phospho-STAT5 in mast cells: a reliable phenotypic marker of systemic mast cell disease that reflects constitutive tyrosine kinase activation

British Journal of Haematology ◽

10.1111/j.1365-2141.2007.06708.x ◽

2007 ◽

Vol 139 (1) ◽

pp. 31-40 ◽

Cited By ~ 15

Author(s):

Tania Zuluaga Toro ◽

Fred H. Hsieh ◽

Juraj Bodo ◽

Henry Y. Dong ◽

Eric D. Hsi

Keyword(s):

Mast Cell ◽

Mast Cells ◽

Tyrosine Kinase ◽

Cell Disease ◽

Phenotypic Marker ◽

Kinase Activation ◽

Mast Cell Disease

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Development of a lethal mast cell disease in mice reconstituted with bone marrow cells expressing the v-erbB oncogene

Blood ◽

10.1182/blood.v79.12.3145.3145 ◽

1992 ◽

Vol 79 (12) ◽

pp. 3145-3158 ◽

Cited By ~ 2

Author(s):

T von Ruden ◽

S Kandels ◽

T Radaszkiewicz ◽

A Ullrich ◽

EF Wagner

Keyword(s):

Bone Marrow ◽

Animal Model ◽

Mast Cell ◽

Mast Cells ◽

Bone Marrow Cells ◽

Myelogenous Leukemia ◽

Leukemic Cells ◽

Cell Disease ◽

Mast Cell Disease ◽

Marrow Cells

Abstract An animal model for malignant mastocytosis is described in mice reconstituted with bone marrow cells expressing the v-erbB oncogene. The lethal mast cell disease is characterized by massive infiltration of bone marrow, spleen, and several other visceral organs by connective tissue mast cells, which normally reside in the skin and the peritoneal cavity. As is frequently found in malignant mastocytosis, the v-erbB- induced mast cell disease was accompanied in some primary recipients by an acute myelogenous leukemia (AML) that killed all secondary recipients regardless of whether the AML was already evident in the primary host. The infiltrating mast cells stained strongly positive with berberine sulfate, suggesting that they were terminally differentiated and in vitro they showed only a weak proliferative capacity. The leukemias were clonal but apparently of different origin than the malignant mast cells, implying the transformation of two independent cell populations. Leukemic cells expressed various myeloid- specific markers as well as the B220 antigen, normally associated with the B-cell lineage. However, the Ig heavy chain genes were still in germ line configuration. In culture, these cells proliferated in the absence of exogenous growth factors and had the capacity to differentiate into mature myeloid cells. Preliminary experiments suggest that v-erbB may use parts of a signal transduction pathway normally coupled to the c-kit receptor. The v-erbB-induced malignant mast cell disease should provide a useful animal model for elucidating the cause for malignant mastocytosis in humans and to explore possible therapeutic strategies.

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