Welcome, Guest. Please Login
MCD - Mast Cell Disorders
  Visit YaBB today Wink
  HomeHelpSearchLogin  
 
Page Index Toggle Pages: 1
Send Topic Print
Current state of biology and diagnosis of clonal mast cell diseases in adults (Read 4700 times)
peter
Mentor
****
Offline


I Love YaBB 2!
Posts: 294

Current state of biology and diagnosis of clonal mast cell diseases in adults
06/05/12 at 11:44:04
 
http://onlinelibrary.wiley.com/doi/10.1111/j.1751-553X.2012.01427.x/full
Mastocytosis comprises a heterogeneous group of disorders characterized by the presence of clonal mast cells (MC) in organs such as skin, bone marrow (BM), and gastrointestinal tract, among other tissues. The clonal nature of the disease can be established in most adult patients by the demonstration of activating KIT mutations in their BM MC. When highly sensitive techniques capable of identifying cells present at very low frequencies in a sample are applied, BM MC from virtually all systemic mastocytosis patients display unique immunophenotypical features, particularly the aberrant expression of CD25. By contrast, large, multifocal BM MC aggregates (the only World Health Organization major criterion for systemic mastocytosis) are absent in a significant proportion of patients fulfilling at least three minor criteria for systemic mastocytosis, particularly in subjects studied at early stages of the disease with very low MC burden. Moreover, recent molecular and immunophenotypical investigations of BM MC from patients with indolent systemic mastocytosis have revealed a close association of some biological features (e.g., multilineage involvement of hematopoiesis by the KIT mutation and an immature mast cell immunophenotype) with an increased risk for disease progression. These observations support the fact that, although the current consensus diagnostic criteria for systemic mastocytosis have been a major advance for the diagnosis and classification of the disease, rationale usage of the most sensitive diagnostic techniques available nowadays is needed to improve the diagnosis, refine the classification, and reach objective prognostic stratification of adult mastocytosis.

Back to top
 
 
IP Logged
 
peter
Mentor
****
Offline


I Love YaBB 2!
Posts: 294

Re: Current state of biology and diagnosis of clonal mast cell diseases in adults
Reply #1 - 06/05/12 at 17:14:20
 
Factors that can evoke the release of MC mediators in patients with mastocytosis  
– Heat (frequent): use mildly warm water for bath or shower. Do not rub briskly with towel to dry off. When drying hair, use hair dryer only on warm heat
– Cold (frequent)
– Pressure (frequent): traumas to scalp in patients with skin lesions in this area. Avoid Darier’s sign (friction of skin lesions), this procedure can evoke a massive mast cell mediators release, specially in large lesions (mastocytomas)
– Exercise (rare)
– Endoscopies (exceptional): panendoscopy, rectoscopy, colonscopy
– Manipulation of GI system/intestines during surgery (exceptional)
Emotional factors
– Stress (frequent)
– Anxiety (frequent)
Drugs and medication
– NSAIDs* (frequent) such as aspirin (acetylsalicylic acid), mefanamic acid, butibufen, diclofenac, fenbufen, fenilbutazone, flurbiprofen, ibuprofen, indometacine, ketoprofen, ketorolac, meclofenamate, metamizole, nabumetone, naproxen, propifenazone, among other drugs
– Opioids and morphine derivatives (rare) such as morphine, codeine, buprenorphine, meperidine, dextromethorphan, dimemorphan, fentanyl, tramadol, among other drugs
– Alcohol† (rare)
– Muscle relaxants and inductors used in general anesthesia∫ (rare)
– Local anesthetics‡ (exceptional if amide-derivatives are used)
– Contrast media (rare)
– Interferon-alpha 2b§ (exceptional)
– Clorodeoxiadenosine (2-Cda)¶ (exceptional)
– Hydroxyurea** (exceptional)
– Colloids (rare)
Insects
– Hymenoptera sting (frequent, particularly in ISMs-)
– Red ant (exceptional in Europe)
Hormonal changes
– Pregnancy (frequent)††
– Menstruation (frequent)
Miscellaneous
– Infections or fever of any etiology (frequent, particularly in children)
– Teething in children (frequent)
– Vaccination (rare, except in children with DCM)
Back to top
 
 
IP Logged
 
peter
Mentor
****
Offline


I Love YaBB 2!
Posts: 294

Re: Current state of biology and diagnosis of clonal mast cell diseases in adults
Reply #2 - 06/05/12 at 18:47:40
 
Abstract
The mast cells are multi-effector cells with wide distribution in the different body parts and traditionally their role has been well-defined in the development of IgE-mediated hypersensitivity reactions including bronchial asthma. Due to the availability of genetically modified mast cell-deficient mice, the broadened pathophysiological role of mast cells in diverse diseases has been revealed. Mast cells exert different physiological and pathophysiological roles by secreting their granular contents, including vasoactive amines, cytokines and chemokines, and various proteases, including tryptase and chymase. Furthermore, mast cells also synthesize plasma membrane-derived lipid mediators, including prostaglandins and leukotrienes, to produce diverse biological actions. The present review discusses the pathophysiological role of mast cells in different diseases, including atherosclerosis, pulmonary hypertension, ischemia-reperfusion injury, male infertility, autoimmune disorders such as rheumatoid arthritis and multiple sclerosis, bladder pain syndrome (interstitial cystitis), anxiety, Alzheimer’s disease, nociception, obesity and diabetes mellitus.
Back to top
 
 
IP Logged
 
Page Index Toggle Pages: 1
Send Topic Print