Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.21.4 (trypsin)
 42,187 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mast cells with bilobed or multilobed nuclei have only rarely been observed in the bone marrow of patients with systemic mastocytosis and in a case of subdural mast cell sarcoma. To our knowledge, they have not been reported in cutaneous mast cell disease. We report a rare occurrence of mast cells with bilobed or multilobed nuclei (atypical mast cell type II) in a nodular lesion of a 24-year-old woman with urticaria pigmentosa. The typical and atypical mast cells were confirmed by Giemsa and Leder's naphthol-AS-D-chloroacetate esterase stains and by immunohistochemical staining for tryptase and KIT protein (CD117). Although the nodular lesion with atypical mast cells did not appear to be cytologically malignant, the occurrence of atypical mast cells in a nodular lesion but not in a papular lesion might denote progression of the disease as suggested by the emergence of cells positive for p53.
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PMID:Mast cells with bilobed or multilobed nuclei in a nodular lesion of a patient with urticaria pigmentosa. 1245 1

Despite maturation arrest, blast cells in acute myeloid leukemia (AML) are often capable of expressing lineage-restricted (granulomonocytic or myelomastocytic) differentiation antigens. Tryptases are lineage-associated serine proteases primarily expressed in mast cells, and less abundantly in blood basophils. We have recently shown that myeloblasts in a group of patients with AML (approximately 40%) produce significant amounts of tryptase(s). In these patients, serum tryptase levels are elevated (> 15 ng/ml) and reflect the total burden of leukemic cells. In most cases, myeloblasts express alpha-tryptase mRNA in excess over beta-tryptase mRNA, and secrete the respective protein (= pro-alpha-tryptase) in a constitutive manner. It was also found that these AML blasts frequentlyco-express tryptase with additional mast cell lineage- and/or basophil-related differentiation antigens including KIT (CD117), histamine, and 2D7. We hypothesize that tryptase-positive AMLs arise from a leukemic progenitor that exhibits a limited potential to differentiate into mast cells and/or basophils.
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PMID:Tryptase a novel biochemical marker of acute myeloid leukemia. 1261 10

Mast cell sarcoma is an extremely rare and aggressive type of mast cell disease. Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intracranial mast cell sarcoma. In Wright-Giemsa-stained cytospin preparations, the morphology of dispersed cells appeared to be highly atypical with a considerable percentage of metachromatic blasts and mast cells with bilobed or multilobed nuclei. Combined toluidine blue/immunofluorescence staining revealed expression of CD13, CD45, CD88, CD116, and CD117 (c-KIT) on neoplastic mast cells. As assessed by immunohistochemistry, mast cells were immunoreactive for tryptase and CD68R, In contrast, the CD2 antigen that is expressed in mast cells in patients with indolent systemic mastocytosis was not detectable. Mast cells also failed to display the c-KIT mutation Asp-816-Val, which is typically found in systemic mast cell disorders. Together, neoplastic mast cells in a case of mast cell sarcoma were found to exhibit unique morphologic, phenotypical, and molecular features when compared with mast cells in indolent mastocytosis or normal tissue mast cells.
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PMID:Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma. 1282 96

A solitary mastocytoma was encountered involving the left labium majus of a 6-year-old girl. Tumor cells contained numerous cytoplasmic metachromatic granules that were immunoreactive for tryptase and CD117. As mast cells are difficult to recognize on routine stains, identification of mast cell lesions requires a high degree of suspicion. This is probably the first reported case of mastocytoma occurring in the vulva.
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PMID:Solitary mastocytoma of the vulva: report of a case. 1450 24

SERPINB6 (PI6) is a member of the intracellular serine protease inhibitors (serpins). Previous studies showed that SERPINB6 is localized mainly in the cytoplasm of endothelial cells, some epithelial cells, monocytes, and neutrophils. In these cells SERPINB6 is thought to prevent cellular damage by scavenging leaking lysosomal proteases. We show here, using novel, well-defined monoclonal antibodies, that SERPINB6 is abundantly expressed by mast cells in all organs and by the human mast cell line HMC-1. Gel filtration experiments revealed that the latter cells contain a high-molecular-weight form of SERPINB6, which consists of sodium dodecyl sulfate (SDS)-stable complexes of this inhibitor with monomeric beta-tryptase. Expression of SERPINB6 by mast cells was compared with those of tryptase and CD117 (c-kit) in biopsies from patients with different forms of mast cell disease. In all cases the lesional mast cells expressed SERPINB6, and, in diffuse cutaneous mastocytosis and mastocytoma, SERPINB6 was expressed by a substantially higher number of mast cells when compared with tryptase. In conclusion, SERPINB6 is abundantly expressed by normal mast cells and by mast cells in mastocytoma lesions. We suggest that in mast cells, SERPINB6 serves to regulate the activity of endogenous beta-tryptase in the cytoplasm.
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PMID:Intracellular serpin SERPINB6 (PI6) is abundantly expressed by human mast cells and forms complexes with beta-tryptase monomers. 1467 Sep 19

End-stage renal disease is characterized by interstitial infiltrate of inflammatory cells in association with tubular atrophy and interstitial fibrosis. Mast cells (MCs) secrete a large number of fibrogenic factors and have been implicated in chronic inflammatory processes with fibrous tissue deposition. The aim of this study was to investigate the distribution of MCs in kidneys with reflux nephropathy (RN) and to determine the relationship between MCs and the interstitial fibrotic process in RN. Kidney specimens from 12 patients (aged 2-13 years) with severe RN secondary to primary high-grade vesicoureteral reflux, obtained at the time of nephrectomy, and 5 controls were examined. Sections were investigated histochemically by acid toluidine blue (TB) and immunohistochemically with antibodies for anti MC-tryptase, MC-chymase, c- kit (CD117), and fibronectin. Double staining for fibronectin and MC-tryptase was performed and examined using confocal scanning microscopy. TB histochemistry showed a marked increase of MCs in RN specimens compared with controls. MC-tryptase, chymase, and c- kit immunopositive MC infiltration was significantly higher in RN samples (14.2+/-9.6) than controls (1.3+/-0.8), ( P<0.05). In all the sections there were more MC-tryptase-positive cells than MC-chymase-positive MCs. Double staining showed increased immunoreactivity of MCs and fibrosis in the renal interstitium of kidneys with RN. The number of infiltrating tryptase-positive MCs was correlated with the degree of interstitial renal scarring. This study demonstrates for the first time the increased expression of MCs in RN, suggesting that MCs may be involved in the development of scarring in RN.
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PMID:Increased expression of mast cells in reflux nephropathy. 1467 55

Systemic mastocytosis is a disease defined by an abnormal infiltration of mast cells involving several extra-cutaneous organs. Hepatic involvement is frequent, however it rarely reveals the disease. We report two cases of systemic mastocytosis revealed by hepatic symptoms: liver failure in one case and jaundice in the second case. The diagnosis is often difficult. Mast cell tissular infiltration can be identified on paraffin sections by tryptase or CD117 (c-kit) immuno-staining.
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PMID:[Liver involvement revealing systemic mastocytosis: report of two cases]. 1504 7

Massive hepatic necrosis (MHN) is a condition that offers an opportunity to study the remarkable ability of the liver to become repopulated with hepatocytes. A maximal regenerative stimulus is expected in cases of MHN (Roskams et al. APMIS Suppl 1991;23:32-39). Sequential chronological observations, after a severe degree of liver cell loss, permit study of the human equivalent of the situation in animal models in which circulating and bone marrow-derived stem and liver progenitor cells are recruited to the hepatopoietic process. To date, the bone marrow and circulating precursors have not been identified morphologically in human material. We present data that suggest that the circulating liver progenitor could have a lymphoblastoid morphological appearance. Similar cells are seen among the cellular infiltrate of MHN. We have found that combinations of markers, such as CD117/CD133 positive CD45/tryptase negative are useful to isolate these cells using cell-sorting technology. This may facilitate their expansion in vitro and the development of their use for therapeutic purposes. In MHN, the residual portal tracts and ductular reaction with the associated lymphoid infiltrate (some of which are probably liver cell progenitors derived from the circulation) constitute the fundamental regenerative community unit in which hepatopoiesis takes place. Defining the hepatopoietic process is hindered by the lack of morphological transitional forms in the period between the progenitors within the circulation and when they assume recognizable hepatocytic form as "metaplastic" hepatocytes associated with the ductular reaction. By achieving a better comprehension of these processes of liver cell restoration, we will be better placed to accelerate liver recovery in MHN, for example by the administration of granulocyte colony stimulating factor (GCSF). Thus, more patients will be able to restore their own livers and avoid liver transplantation.
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PMID:The histopathology of regeneration in massive hepatic necrosis. 1508 86

An association between mastocytosis and monoclonal gammopathy is a relatively rare but well recognized clinical finding. In the majority of cases, however, overt myeloma or lymphoma is not detectable morphologically. Here we describe the case of a 51 year-old male patient first presenting with paresis of the right facial nerve and the serological finding of IgM kappa paraproteinemia. The patient did not have organomegaly, lytic bone lesions, or urticaria pigmentosa-type skin lesions. Histological examination of a trephine biopsy specimen revealed the unusual coexistence of plasma cell myeloma and mastocytosis. Immunohistochemically, plasma cells were found to exhibit a monotypic staining for Ig heavy chain mu and Ig light chain kappa, thus confirming their neoplastic nature. Mast cells showed prominent spindling and formed dense multifocal infiltrates, thus enabling the diagnosis of bone marrow mastocytosis. Immunohistochemically, mast cells expressed tryptase, chymase, and KIT (CD117). In addition, aberrant expression of CD25 on mast cells was detected, confirming the coexistence of a neoplastic mast cell-proliferative disorder. According to the WHO proposal for classification of hematopoietic malignancies, this unique case, showing the association of two very rare haematologic neoplasms, can therefore best be referred to as bone marrow mastocytosis associated with IgM kappa plasma cell myeloma (SM-AHNMD).
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PMID:Bone marrow mastocytosis associated with IgM kappa plasma cell myeloma. 1516 Sep 59

IL-4 is a mast cell and T cell produced immune cytokine that is important in the regulation of macrophage function. IL-4 has also been implicated in the induction of foreign body giant cell formation. In patients with long-term joint prostheses, a localized granulomatous inflammation develops in periarticular tissues and other organs where phagocytosis of particulate material from various prosthetic components takes place. In this study we used the inflammatory lesions of the bone-implant interface as a model to investigate the possible production, the frequency and the cellular source of IL-4. 40 samples of the interface membrane obtained from 25 patients undergoing revision of clinically failed implants were analyzed by immunohistochemistry. Cryostat sections were labeled with specific monoclonal antibodies to mast cell products: IL-4, tryptase and the receptor c-kit (CD117). The study has identified a significant level of production of IL-4 by mast cells in all the cases analyzed. There was an apparent difference in the number of mast cells in relation to the histological variants of the interface. The increase in the number of mast cells and IL-4 production was more pronounced in cases with heavy macrophage infiltrate than those exhibiting a predominance of giant cells. The findings imply that the recruitment of mast cell and IL-4 expression precede the granulomatous reaction and may have a role in the induction of a number of immunopathological changes related to mast cell activation by biomaterial particles.
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PMID:Direct activation of mast cells by prosthetic biomaterial particles. 1534 52


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