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 records of 26 patients with systemic mast cell disease (SMCD) treated during the past decade at the National Institutes of Health were reviewed to determine the role of splenectomy in the management of SMCD. Seventeen (65%) patients had indolent SMCD, manifested primarily by urticaria pigmentosa and mast cell infiltration of the skin, bone marrow, or gastrointestinal tract. None of these patients underwent splenectomy. These patients required only symptomatic therapy. Nine (35%) patients, including those with associated hematologic disorders and those with a lymphoma-like illness termed lymphadenopathic mastocytosis with eosinophilia, had aggressive SMCD. Five of nine patients with aggressive SMCD underwent splenectomy. Of the five patients with splenectomy, three were alive at the time of this report, whereas none of the four who did not have a splenectomy was still alive. Length of survival without splenectomy was 26 months. With splenectomy, length of survival at the time of this report was 34 months. Patients without splenectomy died of bleeding caused by severe thrombocytopenia. Patients with splenectomy appeared better able to tolerate chemotherapy. We thus conclude that while splenectomy is of no value in the management of indolent mastocytosis, it should be considered in patients with aggressive SMCD.
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PMID:Splenectomy in the management of systemic mast cell disease. 240 52

Sixteen bone marrow aspirates and 15 trephine core biopsies from 17 children with cutaneous mastocytosis, of which 15 exhibited urticaria pigmentosa and 2 exhibited diffuse cutaneous mastocytosis, were evaluated for the presence of bone marrow-associated pathologic conditions. Eight bone marrow aspirates from 8 children and 23 trephine core bone marrow biopsies from 16 children who had had evaluation for hematologic abnormalities not associated with cutaneous mastocytosis served as a control population. Eosinophilia was a prominent finding in bone marrows of 9 of 17 patients who had cutaneous mastocytosis. Increased mast cell numbers in bone marrow aspirates were observed in 5 children with cutaneous mastocytosis (5 of 16) and in 2 of the control children (2 of 8). Examination of the trephine core bone marrow biopsies obtained from patients with cutaneous mastocytosis demonstrated focal perivascular and paratrabecular aggregates consisting of mast cells, eosinophils, and early myeloid cells in 10 of 15 individuals. Similar lesions were observed in trephine core bone marrow biopsies of 3 of 16 control patients. The focal mast cell lesions characteristic of adult systemic mastocytosis were not observed. The authors conclude that cutaneous mastocytosis in the pediatric age group is rarely associated with definitive bone marrow findings suggestive of systemic mast cell disease and that this observation is consistent with previous reports that cutaneous mastocytosis in the majority of pediatric cases resolves by adulthood.
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PMID:Hematopathology of the bone marrow in pediatric cutaneous mastocytosis. A study of 17 patients. 247 Feb 48

We have developed a model of asthma in conscious guinea-pigs in which inhalation challenge with specific allergen induces both early and late phase reductions in specific airways conductance. Analysis of cells removed from the airways by bronchoalveolar lavage showed the presence of a neutrophilia, which reached a maximum at 17 hours, and an eosinophilia which developed more slowly, still increasing at 72 hours. Nedocromil sodium inhaled before challenge inhibited both the early and late phase responses. In contrast, the beta-adrenoceptor stimulant, salbutamol, inhibited only the early phase. When inhaled 6 hours after challenge, i.e. between the early and late phase responses, the late phase bronchoconstriction was prevented by nedocromil sodium but only partially by salbutamol. Evidence that the neutrophilia was not functionally associated with the late response was gained from the observations that it was inhibited by both nedocromil sodium and salbutamol, whereas only nedocromil sodium blocked the late phase airways response. When administered 6 hours after challenge, nedocromil sodium reduced eosinophil accumulation at 72 hours in parallel with inhibiting a secondary late response at this time. These results demonstrate that nedocromil sodium is able to prevent both early and late phase reductions in airways function in an animal model of allergic asthma. Whereas inhibition of the early response may reflect an effect on mast cell mediator release, the effects of nedocromil sodium on the late response and on eosinophil accumulation are strongly suggestive of an anti-inflammatory effect within the lung.
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PMID:Effect of nedocromil sodium on early and late phase responses to allergen challenge in the guinea-pig. 254 59

A rare case of thrombocytopenia associated with ranitidine is described. The thrombocytopenia was accompanied by eosinophilia and slightly elevated serum IgE. The platelet and eosinophilic counts returned to normal as soon as the drug was stopped. Cell-mediated immunity (CMI) determined in vitro by the leukocyte migration inhibition factor test was found against ranitidine and cimetidine. IgE antibody response against both drugs was also found by the mast cell degranulation test. These data suggest an association between the ranitidine-induced thrombocytopenia and both humoral antibody response and CMI. Cross-reactivity between the two H2-receptor antagonists is suggested, as well.
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PMID:Thrombocytopenia associated with hypersensitivity to ranitidine: possible cross-reactivity with cimetidine. 271 14

Seven adults with acquired platelet dysfunction with eosinophilia presented with histories of spontaneous bruising and three also had moderate thrombocytopenia. Six patients had platelet function tests performed and all showed variable storage pool defects. When assayed, IgE concentrations were raised. Although only two patients had parasites isolated in their stools, all seven responded to antihelminth treatment. It is speculated that the IgE response to parasites mediates mast cell degranulation, which leads to in vivo platelet activation.
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PMID:Acquired platelet dysfunction with eosinophilia: review of seven adult cases. 279 84

A twelve year-old boy who developed, after a period of strenuous physical work, an illness characterized by thickened skin over his right thigh and hemiabdomen, flexion contractures in right wrist and elbow and in right metacarpophalangeal joints without Raynaud's phenomenon or other visceral symptoms is presented. Pertinent laboratory studies showed hypereosinophilia and hypergammaglobulinemia. Deep-fascia biopsy showed typical findings of fasciitis with eosinophilia. Prednisone therapy resulted in sustained improvement. Periarticular osteopenia of the right hand and mast cell infiltration in fascial biopsy are remarkable features. Authors stress striking differences between fasciitis with eosinophilia or Shulman's syndrome and scleroderma. Clinical picture, laboratory changes, typical histology and a usually rapid response to corticosteroids, as well as some autoimmune diseases possibly associated, suggest a different disease and an immunological pathogenesis. From literature review authors conclude that cases of Shulman's syndrome reported in children are very limited and that this disease should by ruled out in every child with thickened skin changes and articular flexion contractures.
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PMID:[Fasciitis with eosinophilia: Shulman syndrome. Report of a case and review of the literature]. 305 3

A diagrammatic representation of the interactions between mediators of hypersensitivity and leucocytes in early-, late-phase, and ongoing asthma is shown in Fig. 1. Early-phase or immediate reactions are largely the result of bronchoconstriction consequent to the release of mediators such as histamine, PGD2, LTC4/D4 and PAF. The principal mediator cell (MC) is the mast cell (although other IgE receptor-bearing cells such as the macrophage, eosinophil and platelet might also be involved in this immediate response). The stimulus for mediator cell activation may be either immunologic (IgE-dependent) or non-immunologic (i.e. changes in osmolarity as a result of the respiratory water loss associated with exercise-induced asthma). Late-phase reactions appear to be a consequence of infiltration with neutrophils (N), eosinophils (E) and macrophages (MO). These cells are recruited and activated either by mast cell-associated chemotactic factors [such as LTB4, PAF, the eosinophil chemotactic factor of anaphylaxis (ECF-A) or high molecular weight neutrophil chemotactic activity (NCA (HMW]] and/or "lymphokines" derived from T helper cells (TH) which have been stimulated by antigen processed by the antigen processing cells (APC). These mononuclear cell interactions are under the control of regulatory T cells [T suppressor (TS) cells] and it is speculated that the availability of these subsets may determine the magnitude of the late-phase response. Lymphokines and monokines which selectively activate neutrophils, eosinophils and monocytes include LIF, EAF and INF-gamma respectively. Macrophage-derived tumour necrosis factor (TNF) also amplifies the inflammatory response by its capacity to enhance eosinophil cytotoxicity. Eosinophil-derived agents such as PAF, LTC4, MBP and ECP might be responsible for submucosal oedema and non-specific bronchial hyperreactivity which are characteristic features of late-phase reactions. T cell-derived lymphokines such as EDF (IL-5), together with GM-CSF, might lead to eosinophilopoiesis and account for the prolonged eosinophilia of ongoing chronic asthma. The T cell is prominent in the pathology of chronic asthma and is possibly "chronically activated". Thus lymphocytes, driven by as yet undetermined "antigens" (possibly viral), may perpetuate the inflammatory response in and around the bronchi. IL-5-like products from these putative activated lymphocytes might perpetuate (a) eosinophil production by the bone marrow, (b) its release into the circulation, (c) its migration into bronchial tissue and (d) activation to release PAF, LTC4, MBP, etc.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Leucocytes in asthma. 306 26

In order to better assess the prognosis of adult patients with urticaria pigmentosa, 35 patients with generalized maculopapular skin lesions and biopsy-proved cutaneous mast cell increase had light and electron microscopic examinations of their bone marrow; 46% had an increased mast cell content that was focal in all but four patients. Additional frequent findings in this group were an increased cellularity, nuclear-cytoplasmic dissociation of maturation, eosinophilia, and macrophages that contained ingested nuclei. Similar changes were found in far fewer patients without increased marrow mast cells. Only one patient with massive cutaneous mastocytosis had mast cells with relatively large nuclei and immature granules in the extensively involved marrow. Repeated biopsies in this and in six other patients over 3 years showed no worsening of the marrow findings, and the clinical course of all patients was stable over up to 10 years of follow-up. The variable findings in the patients suggest that mastocytosis with cutaneous involvement is a heterogeneous disease where diverse stimuli may cause an increase of mast cells. The clinical course is, however, relatively stable.
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PMID:Bone marrow findings in adult patients with urticaria pigmentosa. 316 49

The development of granulomatous reactions against moulting nymphal pentastomids (Porocephalus crotali) in the tissues of rat and mouse intermediate hosts is described. Adipose tissue and lungs are favoured sites for encystment accounting for 70% of larvae. Six moults separate the primary larva from the final infective stage which first appears about 80 days post-infection (p.i.) and is fully infective by day 120. Larvae, and particularly their cast cuticles, are the foci of granulomatous reactions characterized by an intense eosinophilia. During ecdysis, large numbers of eosinophils permeate the entire lesion but, significantly, degranulation is limited to the underside of cast cuticles where the resultant debris is endocytosed by macrophage/epithelioid cells. A pronounced asymmetry in the granulomatous lesion, evident even in the earliest cysts, results from the accumulation of individual epithelioid granulomas associated with cuticle fragments close to the ventral side of the developing parasite; each is circumscribed by fibrosis. External to this region are extensive tracts of tissue composed of mature plasma cells. Particularly in rats, large numbers of partially degranulated mast cells (= globule leucocytes) also surround cuticle granulomas, and mast cell granules can accumulate within macrophages and fibroblasts. Inflammation slowly subsides once the infective stage is attained. This 1 cm-long larva resides in a thin, fibrotic, C-shaped cyst and can remain viable for years: uniquely this instar retains its last moulted cuticle as a protective sheath. Nymphal instars II-VI feed predominantly upon eosinophils but we do not yet know whether this requirement is obligate.
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PMID:Light microscope observations of granulomatous reactions against developing Porocephalus crotali (Pentastomida: Porocephalida) in mouse and rat. 317 36

The spleen and lymph node are two of the most common organs involved in systemic mast cell disease (SMCD). However, SMCD infiltrates in the spleen and lymph node have a broad spectrum of morphological patterns which can make it difficult to recognize the diagnosis, especially when specimens are examined from patients in whom SMCD is not suspected. We reviewed the pathological features of 16 spleen and 23 lymph node specimens from 19 patients which represented all available material from a series of 58 Mayo Clinic patients with SMCD. The purpose of this study was to investigate the pathological manifestations of SMCD involvement in the spleen and lymph node and to address difficulties in differential diagnosis. All compartments of the spleen and lymph node were found to be affected by SMCD. SMCD lesions in the spleen were found in a paratrabecular (92%), parafollicular (69%), follicular (15%), and a diffuse red pulp (8%) distribution. In the lymph node, mast cell infiltrates affected the paracortex (88%), the parafollicular region (50%), the follicles (25%), the medullary cords (13%), and the sinuses (6%). Mast cells were frequently found in a perivascular location, and associated eosinophilia was common. Because of the broad spectrum of histological manifestations of SMCD in the spleen and lymph node, a wide range of differential diagnoses is discussed including follicular lymphoma, T-cell lymphoma, monocytoid B-cell hyperplasia and lymphoma, Kaposi's sarcoma, and Langerhans' cell granulomatosis.
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PMID:Pathology of the lymph node and spleen in systemic mast cell disease. 323 90


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