Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The amount and distribution of tissue mast cells in the three subtypes of immunocytoma (IC) were studied in lymph nodes of 58 cases and compared with the findings on 34 cases of chronic lymphocytic leukemia (CLL). There were significantly more mast cells in the lymphoplasmacytic and lymphoplasmacytoid subtypes of IC than in CLL. The median mast cell count for the polymorphic subtype of IC was also greater than that for CLL; however, this difference was not statistically significant. Tissue mast cells were diffusely distributed in the lymph nodes in IC, whereas they were chiefly located in the sinus in CLL. Moreover, the cells themselves and their granules were generally larger in IC. Increase in the number and altered distribution of the tissue mast cells in histological sections are therefore diagnostic aids for distinguishing IC from CLL.
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PMID:[Tissue mast cell count in immunocytoma and chronic lymphocytic leukaemia (author's transl)]. 14 May 10

Systemic mastocytosis with associated clonal haematological non-mast cell lineage disease (SM-AHNMD) is a heterogeneous group of mast cell disorders with different clinical, pathologic and underlying molecular characteristics. While myelomonocytic/myeloid neoplasia overwhelmingly predominates the AHNMD component, lymphoproliferative disorders rarely occur as an AHNMD component of SM-AHNMD. Here we report two cases of SM-AHNMD, in which the AHNMD component is chronic lymphocytic leukemia in one case, and concurrent chronic lymphocytic leukemia as well as plasma cell myeloma in another case. To the best of our knowledge, this is the first case report of SM-AHNMD with chronic lymphocytic leukemia and plasma cell dyscrasia simultaneously.
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PMID:Systemic mastocytosis in association with chronic lymphocytic leukemia and plasma cell myeloma. 2049 Mar 36

This prospective study was planned to determine the intercourse between translationally controlled tumor protein (TCTP)/histamine releasing factor (HRF)/histamine pathway and angiogenesis in chronic lymphocytic leukemia (CLL). A total of 153 CLL patients were included. Serum histamine levels were higher in CLL patients. A positive correlation was found between microvessel density (MVD)-mast cell (MC) count; MVD-TCTP/HRF and MC count-TCTP/HRF. Microvessel density, MC and ZAP 70 were significantly higher in TCTP/HRF-positive group. Time to first treatment was shorter in patients with increased MVD and TCTP/HRF. Further data is essential to ascertain the role of TCTP/HRF pathway in tumor angiogenesis and CLL prognosis.
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PMID:TCTP/HRF pathway and angiogenesis: a feasible intercourse in chronic lymphocytic leukemia. 2343 29

Some patients with systemic mastocytosis have concurrent hematological neoplasms, designated in the World Health Organization (WHO) classification as systemic mastocytosis with associated clonal hematological non-mast cell lineage disease (SM-AHNMD). In this study, we analyzed 29 patients with SM-AHNMD and compared them to 40 patients with pure SM. The AHNMDs were classified as chronic myelomonocytic leukemia (CMML) (n = 10), myelodysplastic syndrome (MDS) (n = 7), myeloproliferative neoplasms (n = 4), B-cell lymphoma/leukemia/plasma cell neoplasms (n = 7), and acute myeloid leukemia (n = 1). Patients with SM-AHNMD were older, more frequently had constitutional symptoms and hematological abnormalities, less often had skin lesions, and had an inferior overall survival compared with pure SM patients (48 months vs. not-reached, P < 0.001). Karyotypic abnormalities were detected in 9/28 (32%) patients with SM-AHNMD but not in pure SM patients (P < 0.001). Combined imaging/ fluorescence-in-situ hybridization performed in four SM-AHNMD cases revealed shared abnormal signals in mast cells and myeloid cells in two patients with SM-CMML and one patient with SM-MDS, but not in the mast cells of a case SM-associated with chronic lymphocytic leukemia with ATM-deletion. Quantitative mutation analysis showed higher levels of mutant KIT D816V in SM-CMML and SM-MDS than in pure SM (P < 0.001). Our data indicate that the SM-AHNMD category in the WHO classification is heterogeneous, including clonally related and unrelated forms of AHNMD. The presentation, treatment, and outcome of patients with SM-AHNMD is often dictated by the type of AHNMD.
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PMID:Systemic mastocytosis with associated clonal hematological non-mast cell lineage disease: clinical significance and comparison of chomosomal abnormalities in SM and AHNMD components. 2344 Jun 62

BACKGROUND Systemic mastocytosis with an associated hematologic non-mast cell lineage disease is a rare entity, and the majority of systemic mastocytosis cases are associated with myeloid neoplasm. Lymphoproliferative disorders are less commonly associated with systemic mastocytosis and a few cases of systemic mastocytosis associated with chronic lymphocytic leukemia have been described in the literature. CASE REPORT We present a case of indolent systemic mastocytosis associated with small lymphocytic lymphoma. The bone marrow biopsy demonstrated mast cells in the form of clusters and perivascular distribution on immunohistochemistry for tryptase, CD2, and CD25 markers. In addition, 30% involvement by small lymphocytic lymphoma was discovered in the form of interstitial lymphoid aggregates composed of small lymphocytes. Flow cytometry showed B-cells positively stained for CD19, CD20, CD5, CD23, and kappa light chains, and the CD38 expression was <5%. CONCLUSIONS In systemic mastocytosis with an associated hematologic non-mast cell lineage disease, the combination of systemic mastocytosis associated with small lymphocytic lymphoma is rare and the management strategy follows the principle of treating the two entities individually as if they are not related. Clinical surveillance is indicated for indolent systemic mastocytosis and low-risk small lymphocytic lymphoma to monitor for disease progression.
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PMID:Systemic Mastocytosis in Association with Small Lymphocytic Lymphoma. 2897 Apr 67

Angioedema is a clinical entity defined as self-limiting edema localized in the deeper layers of the skin and mucosa and lasting for several days. Angioedema can be provoked by bradykinin and/or mast cell mediators, including histamine. Four types of acquired and three types of hereditary angioedema have been identified. The most obvious form of angioedema associated with other systemic disease is acquired angioedema due to C1-inhibitor deficiency. It is characterized by acquired consumption of C1 inhibitor and various underlying disorders, such as multiple myeloma, chronic lymphocytic leukemia, rectal carcinoma, and non-Hodgkin lymphoma. Suspected cases need an accurate differential diagnosis to exclude all other types of acquired and hereditary angioedema.
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PMID:Angioedema as a systemic disease. 3186 42