scholarly journals Systemic mast cell disease in the Mongolian gerbil, Meriones unguiculatus: case report

1997 ◽  
Vol 31 (4) ◽  
pp. 373-378 ◽  
Author(s):  
Maria Angelica Guzman-Silva
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Case Report ◽  
Mongolian Gerbil ◽  
Skin Disease ◽  
Systemic Mastocytosis ◽  
Meriones Unguiculatus ◽  
Cell Disease ◽  
Mast Cell Disease ◽  
Spontaneous Occurrence

A case of spontaneous occurrence of systemic mast cell disease in a non-treated laboratory-reared gerbil is reported. This case corresponds to the form of systemic mastocytosis with bone marrow and visceral involvement associated with skin disease and displays clinically aggressive behaviour. The histopathological, histochemical and ultrastructural features are described.

The faces of mast cell disease: bone marrow infiltrates in 3 patients with systemic mastocytosis

2005 ◽  
Vol 9 (2) ◽  
pp. 81-85 ◽  
Author(s):  
Antonio E. Martinez ◽  
Ana M. Medina ◽  
Jonathan A. Hyde ◽  
Elisa Krill-Jackson ◽  
Arnold Blaustein ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Systemic Mastocytosis ◽  
Cell Disease ◽  
Mast Cell Disease

Bone Marrow Angiogenesis in Systemic Mast Cell Disease

2002 ◽  
Vol 11 (1) ◽  
pp. 139-146 ◽  
Author(s):  
Jin-Young Baek ◽  
Chin-Yang Li ◽  
Animesh Pardanani ◽  
Joseph H. Butterfield ◽  
Ayalew Tefferi
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Cell Disease ◽  
Mast Cell Disease

scholarly journals MDS with 5q deletion and rare cKIT positive mastocytosis: a diagnostic and therapeutic challenge

2019 ◽  
Vol 12 (4) ◽  
pp. e227768
Author(s):  
Daniel Steven Sanders ◽  
Thomas Fennell ◽  
Mohammad Muhsin Chisti
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Case Report ◽  
Systemic Mastocytosis ◽  
Molecular Testing ◽  
Bone Marrow Biopsies ◽  
Haematological Response ◽  
Clonal Origin ◽  
Mast Cell Population ◽  
5Q Deletion

A patient with a diagnosis of myelodysplastic syndrome (MDS) with isolated 5q deletion underwent repeat bone marrow biopsy to assess haematological response after 6 months of initial lenalidomide therapy. Subsequent bone marrow biopsies revealed persistent MDS with del(5q) in addition to a small atypical mast cell population with >25% of mast cells with spindle-shaped morphology and immunohistochemistry characteristics consistent with mastocytosis. Molecular testing on the bone marrow was positive for cKIT D816V and the patient was diagnosed with systemic mastocytosis (SM) with an associated haematological neoplasm. MDS with SM is well known to be associated; however, to the best of our knowledge, only one prior case report identifies MDS with del(5q) and associated cKIT D816V positive mastocytosis. While the exact clonal origin of both chromosomal aberrations is unclear, this case illustrates the therapeutic efficacy of lenalidomide in a patient with MDS with del(5q) and rarely associated cKIT positive SM.

Bone marrow mast cell disease associated with Felty's syndrome and liver cirrhosis

1998 ◽  
Vol 59 (2) ◽  
pp. 179-179 ◽  
Author(s):  
Ken-ichiro Inoue ◽  
Keiji Yoshioka ◽  
Yoshihiro Kasamatsu ◽  
Noriya Hiraoka ◽  
Yutaka Kawahito ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Liver Cirrhosis ◽  
Cell Disease ◽  
Felty's Syndrome ◽  
Felty’S Syndrome ◽  
Mast Cell Disease ◽  
Bone Marrow Mast Cell

Kit Signaling Regulates Mitf Expression in Mastocytosis.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3601-3601
Author(s):  
Youl-Nam Lee ◽  
Pierre Noel ◽  
Amir Shahlaee ◽  
Melody Carter ◽  
Reuben Kapur ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Mast Cells ◽  
Systemic Mastocytosis ◽  
Tyrosine Kinase Receptor ◽  
Bone Marrow Biopsies ◽  
Activating Mutations ◽  
Cell Assays ◽  
Mast Cell Disease ◽  
Mitf Expression

Abstract Mastocytosis is a heterogeneous disease arising from abnormal proliferation of mast cells. Activating mutations in codon D816 of the tyrosine kinase receptor, c-kit, are found in the majority of adult patients with systemic mastocytosis, an aggressive form of the disease. Constitutive activation of the Kit signaling pathway is critical to the transformed phenotype, and thus understanding how this pathway regulates downstream events is of great importance. A number of transcription factors are also essential to mast cell development, including the Microphthalmia-associated transcription factor (Mitf). We examined Mitf expression in bone marrow biopsies from nine patients with systemic mastocytosis by immunohistochemistry; we found that Mitf is highly expressed in all cases with the D816V mutation. In contrast, Mitf is not highly expressed in non-malignant mast cells in the bone marrow from patients with aplastic anemia and leukemia, suggesting thatMitf expression is regulated by Kit-dependent signalsMitf may play a role in the transformed phenotype of mastocytosis.We show that in normal mast cells, Kit signaling markedly upregulates Mitf expression. In both normal and malignant mast cells, pharmacologic inhibitors of Kit, and the downstream kinase, PI3K, block Mitf expression. To examine whether Mitf is required for transformed phenotype from constitutive Kit signaling in mast cells, we have used a shRNA-expressing lentivirus to knockdown Mitf expression in mastocytosis cell lines. We found that silencing of Mitf markedly impaired growth in proliferation and colony forming cell assays. This work demonstrates a link between two critical factors, Kit and Mitf, in the development of malignant mast cell disease.

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

1999 ◽  
Vol 106 (2) ◽  
pp. 400-405 ◽  
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

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

Cytometry ◽  
1998 ◽  
Vol 34 (5) ◽  
pp. 223-228 ◽  
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

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

Blood ◽  
1992 ◽  
Vol 79 (12) ◽  
pp. 3145-3158 ◽  
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.

Sign in / Sign up

Export Citation Format

Share Document