Mastocytosis: A heterogenous group of disorders characterized by the abnormal increase of MAST CELLS in only the skin (MASTOCYTOSIS, CUTANEOUS), in extracutaneous tissues involving multiple organs (MASTOCYTOSIS, SYSTEMIC), or in solid tumors (MASTOCYTOMA).Mastocytosis, Systemic: A group of disorders caused by the abnormal proliferation of MAST CELLS in a variety of extracutaneous tissues including bone marrow, liver, spleen, lymph nodes, and gastrointestinal tract. Systemic mastocytosis is commonly seen in adults. These diseases are categorized on the basis of clinical features, pathologic findings, and prognosis.Mastocytosis, Cutaneous: Skin lesions due to abnormal infiltration of MAST CELLS. Cutaneous mastocytosis is confined to the skin without the involvement of other tissues or organs, and is mostly found in children. The three major variants are: URTICARIA PIGMENTOSA; diffuse cutaneous mastocytosis; and SOLITARY MASTOCYTOMA OF SKIN.Urticaria Pigmentosa: The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules.Tryptases: A family of neutral serine proteases with TRYPSIN-like activity. Tryptases are primarily found in the SECRETORY GRANULES of MAST CELLS and are released during mast cell degranulation.Proto-Oncogene Proteins c-kit: A protein-tyrosine kinase receptor that is specific for STEM CELL FACTOR. This interaction is crucial for the development of hematopoietic, gonadal, and pigment stem cells. Genetic mutations that disrupt the expression of PROTO-ONCOGENE PROTEINS C-KIT are associated with PIEBALDISM, while overexpression or constitutive activation of the c-kit protein-tyrosine kinase is associated with tumorigenesis.Mast Cells: Granulated cells that are found in almost all tissues, most abundantly in the skin and the gastrointestinal tract. Like the BASOPHILS, mast cells contain large amounts of HISTAMINE and HEPARIN. Unlike basophils, mast cells normally remain in the tissues and do not circulate in the blood. Mast cells, derived from the bone marrow stem cells, are regulated by the STEM CELL FACTOR.Leukemia, Mast-Cell: A form of systemic mastocytosis (MASTOCYTOSIS, SYSTEMIC) characterized by the presence of large numbers of tissue MAST CELLS in the peripheral blood without skin lesions. It is a high-grade LEUKEMIA disease with bone marrow smear of >20% MAST CELLS, multi-organ failure and a short survival.Bone Marrow: The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.Methylhistamines: Histamine substituted in any position with one or more methyl groups. Many of these are agonists for the H1, H2, or both histamine receptors.Chymases: A family of neutral serine proteases with CHYMOTRYPSIN-like activity. Chymases are primarily found in the SECRETORY GRANULES of MAST CELLS and are released during mast cell degranulation.Bone Marrow Examination: Removal of bone marrow and evaluation of its histologic picture.Trichinella spiralis: A parasite of carnivorous mammals that causes TRICHINELLOSIS. It is especially common in rats and in swine fed uncooked garbage. Human infection is initiated by the consumption of raw or insufficiently cooked pork or other meat containing the encysted larvae.Anaphylaxis: An acute hypersensitivity reaction due to exposure to a previously encountered ANTIGEN. The reaction may include rapidly progressing URTICARIA, respiratory distress, vascular collapse, systemic SHOCK, and death.Mastocytoma: A solid tumor consisting of a dense infiltration of MAST CELLS. It is generally benign.Trichinellosis: An infection with TRICHINELLA. It is caused by eating raw or undercooked meat that is infected with larvae of nematode worms TRICHINELLA genus. All members of the TRICHINELLA genus can infect human in addition to TRICHINELLA SPIRALIS, the traditional etiological agent. It is distributed throughout much of the world and is re-emerging in some parts as a public health hazard and a food safety problem.Interleukin-9: A multifunctional cytokine secreted by primarily by activated TH2 CELLS that may play a role as a regulator of allergic INFLAMMATION. It has been shown to enhance the growth and CELL DIFFERENTIATION of MAST CELLS, and can act on a variety of other immune cells.Intestinal Diseases, Parasitic: Infections of the INTESTINES with PARASITES, commonly involving PARASITIC WORMS. Infections with roundworms (NEMATODE INFECTIONS) and tapeworms (CESTODE INFECTIONS) are also known as HELMINTHIASIS.Hydroxyzine: A histamine H1 receptor antagonist that is effective in the treatment of chronic urticaria, dermatitis, and histamine-mediated pruritus. Unlike its major metabolite CETIRIZINE, it does cause drowsiness. It is also effective as an antiemetic, for relief of anxiety and tension, and as a sedative.Prostaglandins D: Physiologically active prostaglandins found in many tissues and organs. They show pressor activity, are mediators of inflammation, and have potential antithrombotic effects.Trematode Infections: Infections caused by infestation with worms of the class Trematoda.Trematoda: Class of parasitic flukes consisting of three subclasses, Monogenea, Aspidogastrea, and Digenea. The digenetic trematodes are the only ones found in man. They are endoparasites and require two hosts to complete their life cycle.Stem Cell Factor: A hematopoietic growth factor and the ligand of the cell surface c-kit protein (PROTO-ONCOGENE PROTEINS C-KIT). It is expressed during embryogenesis and is a growth factor for a number of cell types including the MAST CELLS and the MELANOCYTES in addition to the HEMATOPOIETIC STEM CELLS.Eosinophilia: Abnormal increase of EOSINOPHILS in the blood, tissues or organs.Interleukin-5 Receptor alpha Subunit: A low affinity interleukin-5 receptor subunit that combines with the CYTOKINE RECEPTOR COMMON BETA SUBUNIT to form a high affinity receptor for INTERLEUKIN-5. Several isoforms of the interleukin-5 receptor alpha subunit exist due to multiple ALTERNATIVE SPLICING.Arthropod Venoms: Venoms from animals of the phylum Arthropoda. Those most investigated are from scorpions and spiders of the class Arachnidae and from ant, bee, and wasp families of the Insecta order Hymenoptera. The venoms contain protein toxins, enzymes, and other bioactive substances and may be lethal to man.Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.Vulvar Diseases: Pathological processes of the VULVA.

*  Single Vaccines: A Safer Option for Children with Mastocytosis - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
Single Vaccines: A Safer Option for Children with Mastocytosis. Single Vaccines: A Safer Option for Children with Mastocytosis ... 2) Children with mastocytosis should receive their first dose of vaccine in a controlled clinical setting. Furthermore, they ... Matito A, Carter M. Cutaneous and systemic mastocytosis in children: A risk factor for anaphylaxis? Curr Allergy Asthma Rep. ... Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and ...
  https://tmsforacure.org/single-vaccines-safer-option-children-mastocytosis/
*  prognosis
aggressive systemic mastocytosis, indolent systemic mastocytosis, prognosis, progression, smoldering systemic mastocytosis, ... The subtypes of systemic mastocytosis are indolent systemic mastocytosis (ISM), smoldering systemic mastocytosis (SSM), ... indolent systemic mastocytosis, MastAttack 107, prognosis, smoldering systemic mastocytosis, systemic mastocytosis Progression ... Is systemic mastocytosis a form of cancer? Why do some papers say the life expectancy for systemic mastocytosis patients is ...
  http://www.mastattack.org/tag/prognosis/
*  Interferon-alpha in combination with corticosteroids improves systemic mast cell disease. - Semantic Scholar
Mastocytosis: pathology, genetics, and current options for therapy.. *Peter Valent. , Cem Akin. , +8 authors. Dean D Metcalfe ... Cladribine therapy for systemic mastocytosis.. *Hanneke C Kluin-Nelemans. , J Marja Oldhoff. , +6 authors. Rob Fijnheer ... Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis.. *Jason Gotlib. , Hanneke C Kluin-Nelemans. , +14 authors ... Systemic mastocytosis in adults: 2012 Update on diagnosis, risk stratification, and management.. *Animesh Pardanani ...
  https://www.semanticscholar.org/paper/Interferon-alpha-in-combination-with-corticosteroi-Delaporte-Pi%C3%A9rard/6e1b2699a73ad57895dfd54a02672a264e00c5d4
*  Home - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
The Mastocytosis Society is a non-profit organization dedicated to supporting patients affected by Mast Cell Disorders via ... The Mastocytosis Society. is a non-profit organization dedicated to supporting patients affected by Mastocytosis and Mast Cell ... The Mastocytosis Society, Inc. will hold its next large scientific and support conference for patients, families and health ... ICD-10-CM Codes for Mast Cell Activation Syndrome and Mastocytosis- December 2017. ICD-10-CM codes for Mast Cell Activation ...
  http://tmsforacure.org
*  Mast Cell Disorders Forum - MTHFR defect? undermethylation associated with not clearing histamine
... such as mastocytosis (They have included MCAS). This offering has been listed on the TMS website for months, and several of us ...
  http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1346571085
*  A Note from the Research Chair - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
Our TMS RESEARCH FUND supports the amazing work done by leading mast cell disorder researchers. Our TMS GENERAL FUND supports all other TMS initiatives and activities, including EDUCATION and SUPPORT of patients, caregivers and physicians, our ADVOCACY on Capitol Hill and our RESEARCH Committee work. Please consider donating to one or both of these vital funds.. DONATE TO RESEARCH FUND , DONATE TO GENERAL FUND ,. Hello Everyone! Welcome to the Research section of our new TMS website. I am thrilled to now have such an easy platform to share information with you. Throughout the whole TMS website, we have tried to make information as helpful and easy to find as possible. In this section, we will keep you up to date on our various Research activities and hope to provide you with the resources you need to do your own research and share valuable information with your physicians, as an informed patient or caregiver. Or, maybe you are a physician trying to find information to help your patients or ...
  https://tmsforacure.org/research/note-research-chair/
*  George Carlo Exposes Mobile Phone Wireless Industry Part 11 | Cell Phone Radiation News
The manner in which primary mastocytosis can be distinguished from secondary mastocytosis and other conditions should also be ... It is important to remember that mastocytosis - a n abnormal accumulation of mast cells in one or more organ system - can occur ... The increase in EHS being described here is more accurately thought of as "primary" mastocytosis, meaning that the increased ... Howeve r, because of the increased number of mast cells in primary mastocytosis, conditions such as osteoporosis and ...
  http://www.emfnews.org/articles/george-carlo-exposes-mobile-phone-wireless-industry-part-11/
*  The MastAttack 107: The Layperson's Guide to Understanding Mast Cell Diseases, Part 66 - Mast Attack
For mastocytosis patients, chemotherapy is used for patients with systemic mastocytosis in whom the disease is malignant ( ... There is no described use for chemo in cutaneous mastocytosis.. *There are situations where patients with other disease ... aggressive systemic mastocytosis, chemo, mast cell activation syndrome, mast cell leukemia, MastAttack 107, medication, ... aggressive systemic mastocytosis allergies anaphylaxis angioedema antihistamines asthma autoimmune disease cardiovascular ...
  http://www.mastattack.org/2017/10/mastattack-107-laypersons-guide-understanding-mast-cell-diseases-part-66/
*  What is Mastocytosis? Symptoms and Treatments - Facty Health
Systemic mastocytosis, which affects other parts of the body, and is more common in adults. This type of mastocytosis is a ... Mastocytosis is a rare condition in which too many mast cells swell in the body's tissues. Mast cells are a type of white blood ... Since mastocytosis can affect different parts of the body, signs, and symptoms may vary. The following symptoms may appear in a ... What is Mastocytosis? Symptoms and Treatments. Share on Facebook Share on Twitter ...
  http://health.facty.com/ailments/body/what-is-mastocytosis-symptoms-and-treatments/
*  Mast Cell Disorder Patient Registries - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
Below are links to information on two mastocytosis patient registries. The European Competence Network on Mastocytosis (ECNM) ... Neither of these registries is owned or managed by The Mastocytosis Society, Inc. ...
  https://tmsforacure.org/research/mast-cell-disorder-patient-regisitries/
*  The Poet's Corner - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
by. Kathy Bell, 2016. My skin, it itches, my eyebrows, they twitch.. My feet burn like they were hit with a switch.. I thirst and I drink and yet I thirst the more.. Puffed up like a toad until I am sore,. I can't bend my fingers and can't see my toes,. And my belly it swells and swells-. That's where all the fluid goes!. My gut, oh my gut did- I mention it swells?. Like a mighty fortress it closes up tight,. On guard for any intruders that might put up a fight.. But the flaw in the system is that food is not my rival.. Food, yes, food is what I need for survival.. Backing up, freezing up, fermenting my fodder.. Eventually it stalls and sputters its way open,. The pain I endure is truly unbearable,. But somehow, lamenting, bear it I do.. My mind is confused, my words have all …gone. What used to be easy is now a great feat.. My thinker won't think, brain fog they call it,. But when I'm in a flare it's more like pea soup!. My thoughts, they just go round and round in a loop.. Sometimes it's so ...
  https://tmsforacure.org/the-poets-corner/
*  TMS Research Grant Awards: 2010 - 2017 - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
Title: Occult Mastocytosis and KIT D816V in CBF AML. 2014 TMS/ARTrustTM Research Award in Mastocytosis and/or Mast Cell ... The Mastocytosis Society, Inc. (TMS) awards grant funding for mastocytosis and mast cell activation syndrome (MCAS) research. ... 2015 TMS/ARTrustTM Research Award in Mastocytosis and/or Mast Cell Activation Syndrome. This grant award was a 1 year joint ... 2017 TMS/AAAAI Research Award in Mastocytosis and/or Mast Cell Activation Syndrome. This grant award is a 1 year joint offering ...
  https://tmsforacure.org/tms-research-grant-awards-2010-2017/
*  Neuropsychiatric features of mast cell disease: Part 1 of 2 - Mast Attack
One study found that in a group of 88 patients with indolent systemic mastocytosis (ISM) and cutaneous mastocytosis (CM), 75% ... I had never read or heard of this being part of the cognitive part of Mastocytosis. Even in a conversation at times I have a ... One mastocytosis cohort reported 38.6% had cognitive impairment of some kind. Inability to focus and pay attention is the ... 35% of mastocytosis patients in one study reported 35% had either acute or chronic headaches. 37.5% had migraines, while 17.2% ...
  http://www.mastattack.org/2016/04/neuropsychiatric-features-of-mast-cell-disease-part-1-of-2/
*  Mastocytosis and Mast Cell Disorders | Patient
Mastocytosis is a condition in which there are too many mast cells in the body, Another disorder involves mast cells that are ... Cutaneous mastocytosis. Cutaneous means skin. One of the most common skin problems caused by mastocytosis is urticaria ... Maculopapular cutaneous mastocytosis; DermNet NZ, 2014. *Pardanani A; Systemic mastocytosis in adults: 2017 update on diagnosis ... Mastocytosis is a condition in which there are too many mast cells in the body, Another disorder involves a normal number of ...
  https://patient.info/health/mastocytosis-and-mast-cell-disorders-leaflet?utm_source=GPWEB&utm_medium=site&utm_campaign=gpwebsite
*  Advocacy - TMS - The Mastocytosis Society :TMS - The Mastocytosis Society
This past October, NIAID researchers announced promising results from an NIH sponsored clinical trial on the efficacy and value of an intervention for treating children and young adults with peanut allergies. This January, an NIAID-sponsored expert panel issued clinical guidelines to help healthcare providers give parents and caregivers important information on early introduction of peanut-containing foods to infants to prevent the development of peanut allergy. These are the type of results the American people can expect from supporting NIH (NIAID, NHLBI and NIEHS) and their medical research mission ...
  https://tmsforacure.org/community/advocacy/
*  Characteristics of Mast Cells in Mastocytosis - Full Text View - ClinicalTrials.gov
Characteristics of Mast Cells in Mastocytosis. The safety and scientific validity of this study is the responsibility of the ... Mastocytosis. Neoplasms, Connective Tissue. Neoplasms, Connective and Soft Tissue. Neoplasms by Histologic Type. Neoplasms. ... The blood and bone marrow samples will be used for clinical care and for research to determine if mastocytosis is due to mast ... Patients with mastocytosis now followed at the NIH on protocol 88-I-0190 will be continued on this study. ...
  https://clinicaltrials.gov/ct2/show/NCT00001356?cond=%22Cutaneous+mastocytosis%22&rank=19
*  Masitinib in Patients With Mastocytosis With Handicap and Bearing the D816V Mutation - Full Text View - ClinicalTrials.gov
Indolent Systemic Mastocytosis having 2 infiltrated organs (skin and bone-marrow). *Any mastocytosis with in the last 6 months ... Patients with one of the following mastocytosis:. *Systemic Mastocytosis with an Associated clonal Hematologic Non Mast cell ... Patients With Mastocytosis With Handicap and Bearing Mutations Asp-816-Val (D816V) Drug: masitinib Phase 2 ... Masitinib in Patients With Mastocytosis With Handicap and Bearing the D816V Mutation. The safety and scientific validity of ...
  https://clinicaltrials.gov/ct2/show/NCT01266369
*  Mastocytosis - Wikipedia
Cutaneous mastocytosis (CM) *The most common cutaneous mastocytosis is maculopapular cutaneous mastocytosis, previously named ... Systemic mastocytosis (SM) Systemic mastocytosis involves the bone marrow in the majority of cases and in some cases other ... "Mastocytosis & mast cell disorders". Mastocytosis Society Canada. Archived from the original on 2013-12-28. Retrieved 2017-11- ... The true incidence and prevalence of mastocytosis is unknown, but mastocytosis generally has been considered to be an "orphan ...
  https://en.wikipedia.org/wiki/Mastocytosis
*  Mastocytosis - familydoctor.org
Mastocytosis is the abnormal growth of mast cells in the body. Mast cells are part of the immune system, which helps protect ... The symptoms of mastocytosis can be similar to the symptoms of many other health problems. Your doctor may do a skin biopsy to ... Mastocytosis can cause a severe allergic reaction in some people. It's a good idea to keep an emergency kit with you at all ... Mastocytosis is the abnormal growth of mast cells in the body. Mast cells are part of the immune system, which helps protect ...
  https://familydoctor.org/condition/mastocytosis/
*  Symptoms of mastocytosis
Also, mastocytosis is so rare, that many doctors do not know how to diagnose or treat mastocytosis, so they focus on other, ... Mastocytosis concerns the mast cells in the body. People suffering with mastocytosis have too many mast cells and they do not ... Mastocytosis can be just a skin disease, Cutaneous Mastocytosis or Urticaria Pigmentosa, which means that the mast cells ... This form is the one most commonly found in childhood or pediatric mastocytosis. Children who develop cutaneous mastocytosis ...
  http://www.empowher.com/groups/mastocytosis/posts/symptoms-mastocytosis
*  Mastocytosis: Symptoms and Signs | Cancer.Net
Sometimes, people with mastocytosis do not have any of these changes. Or, the cause of a symptom may be a different medical ... Use the menu to see other pages.People with mastocytosis may experience the following symptoms or signs. ... condition that is not mastocytosis.General symptoms of mastocytosisHivesRed, itchy rashDiarrheaAbdominal painFainting ... People with mastocytosis may experience the following symptoms or signs. Sometimes, people with mastocytosis do not have any of ...
  https://www.cancer.net/cancer-types/mastocytosis/symptoms-and-signs
*  Cromoglicate in Mastocytosis - Full Text View - ClinicalTrials.gov
Chronic stable symptomatic maculopapulous cutaneous mastocytosis or indolent systemic mastocytosis with skin involvement and a ... Cromoglicate in Mastocytosis. The safety and scientific validity of this study is the responsibility of the study sponsor and ... Mastocytosis. Neoplasms, Connective Tissue. Neoplasms, Connective and Soft Tissue. Neoplasms by Histologic Type. Neoplasms. ... The trial will be performed as a left/right comparison study in male/female subjects with mastocytosis, in a prospective, ...
  https://clinicaltrials.gov/ct2/show/study/NCT01701843?view=record
*  Search of: 'Systemic mastocytosis' - Modify Search - ClinicalTrials.gov
Search of: 'Systemic mastocytosis' - Modify Search. Fill in any or all of the fields below. Click on the label to the left of ...
  https://clinicaltrials.gov/ct2/results/refine?cond=%22Systemic+mastocytosis%22
*  Use of Tamoxifen in Systemic Mastocytosis
Treatment of Systemic Mastocytosis With Tamoxifen. Trial Phase:. N/A. Minimum Age:. 21 Years. Maximum Age:. N/A ... Systemic Mastocytosis. Exclusion Criteria:. - Current treatment with Imatinib mesylate, cladribine or interferon alpha. ... Stability or reduction of the percent bone marrow involvement by mastocytosis, including stable mast cell morphology and ... medications regardless of the percentage bone marrow involvement by mastocytosis. The dose of Tamoxifen will be 20 mg/day and ...
  http://www.knowcancer.com/cancer-trials/NCT01334996/
*  How Anxiety Relates to Mastocytosis
Causes of Anxiety From Mastocytosis. Most anxiety from mastocytosis is related to either attacks of systemic mastocytosis or ... How Anxiety Relates to Mastocytosis. Living with a chronic health condition can be a challenging. In the case of mastocytosis, ... Systemic Mastocytosis and Anxiety. Most mastocytosis is cutaneous, and the cutaneous version doesn't necessarily create any ... since mastocytosis is far less common. Less than 200,000 cases of mastocytosis are believed to exist in the United States ( ...
  https://www.calmclinic.com/anxiety/types/mastocystosis

Xanthelasmoidal mastocytosis: Xanthelasmoidal mastocytosis is a cutaneous condition characterized by numerous, confluent, yellow–tan papules.Diffuse cutaneous mastocytosis: Diffuse cutaneous mastocytosis has diffuse involvement in which the entire integument may be thickened and infiltrated with mast cells to produce a peculiar orange color, giving rise to the term "homme orange."James, William; Berger, Timothy; Elston, Dirk (2005).Urticaria pigmentosaTryptase: Tryptase (, ) is the most abundant secretory granule-derived serine proteinase contained in mast cells and has been used as a marker for mast cell activation. The biological function of tryptase is unclear.MotesanibDegranulationMast cell leukemiaBone marrow suppression: Bone marrow suppression or myelotoxicity (adjective myelotoxic) or myelosuppression is the decrease in production of cells responsible for providing immunity (leukocytes), carrying oxygen (erythrocytes), and/or those responsible for normal blood clotting (thrombocytes). Bone marrow suppression is a serious side effect of chemotherapy and certain drugs affecting the immune system such as azathioprine.Bone marrow examination: Bone marrow examination refers to the pathologic analysis of samples of bone marrow obtained by bone marrow biopsy (often called a trephine biopsy) and bone marrow aspiration. Bone marrow examination is used in the diagnosis of a number of conditions, including leukemia, multiple myeloma, lymphoma, anemia, and pancytopenia.Anaphylaxis Campaign: The Anaphylaxis Campaign is the only UK charity to exclusively meet the needs of the growing numbers of people at risk from severe allergic reactions by providing information and support relating to foods and other triggers such as latex, drugs and insect stings.The Anaphylaxis CampaignInternational Conference on Trichinellosis: The International Commission on Trichinellosis (ICT) was created in 1958 in Budapest and is aiming to exchange information on the biology, the physiopathology, the epidemiology, the immunology, and the clinical aspects of trichinellosis in humans and animals. Prevention is a primary goal (see ICTweb pages).Intestinal parasiteHydroxyzineBithynia fuchsiana: Bithynia fuchsiana is a species of small freshwater snail with a gill and an operculum, an aquatic gastropod mollusk in the family Bithyniidae.Philophthalmus gralliAncestimEosinophilic gastroenteritisScolopendra: Scolopendra (through Latin from Greek "skolopendra") is a genus of centipedes of the family Scolopendridae.Brain biopsyLabial fusion

(1/170) Cutaneous and systemic manifestations of mastocytosis.

Mastocytosis is characterized by an excessive number of apparently normal mast cells in the skin and, occasionally, in other organs. Characteristic skin lesions, called urticaria pigmentosa, are present in most patients, but clinical presentation can vary from a pruritic rash to unexplained collapse and sudden death. These lesions are typically tan to red-brown macules that appear on the trunk and spread symmetrically. Patients with mastocytosis often have a long history of chronic and acute symptoms that were unrecognized as mastocytosis. Skin lesions may or may not accompany systemic mastocytosis. Systemic disease may involve the gastrointestinal tract, the bone marrow or other organs. Even when the disease is considered as a possibility by the physician, the diagnosis can be difficult because of special technical requirements necessary for biopsy and because of the problems with biochemical testing. Drug therapy is initiated to stabilize mast cell membranes, to reduce the severity of the attacks and to block the action of inflammatory mediators. The mainstay of therapy is histamine H1 and H2 blockers and the avoidance of triggering factors.  (+info)

(2/170) Stem cell factor is localized in, released from, and cleaved by human mast cells.

Stem cell factor (SCF) is the most important cytokine regulating human mast cell growth and functions. The immunogold technique showed SCF in the secretory granules of skin mast cells and in lung parenchymal mast cells (HLMC). Immunoreactive SCF (iSCF) was detected in cell lysates of HLMC, but not in basophils; iSCF and histamine were detected in supernatants of HLMC 3 min after challenge with anti-FcepsilonRI or anti-IgE, and iSCF in supernatants rapidly declined after 30 min, whereas histamine remained unchanged for 120 min. HPLC and electrospray mass spectrometry (ES/MS) analysis of recombinant human SCF1-166 (18,656. 9 +/- 0.9 Da) treated with chymase showed a polypeptide of 17,977.1 +/- 0.6 Da and a minor component of 697.4 +/- 0.1 Da generated by specific cleavage at Phe159. SCF1-166 and SCF1-159 similarly activated HLMC, potentiated anti-IgE-induced activation of these cells, and stimulated HLMC chemotaxis. SCF159-166 had no effect on mast cells. Western blot analysis of supernatants of anti-IgE-activated HLMC incubated with recombinant human SCF1-166 showed that SCF1-166 was rapidly cleaved to SCF1-159 and SCF1-144. Experiments with supernatants of anti-IgE-activated HLMC incubated with SCF1-166 yielded similar results. In conclusion, SCF is stored in mast cell secretory granules and is immunologically released by human mast cells. SCF1-166 is rapidly and specifically cleaved to SCF1-159 by chymase, which retains its biological effect on mast cells. SCF is also cleaved by other proteases to several SCF species whose possible biological activities remain to be established.  (+info)

(3/170) An unusual presentation of systemic mastocytosis.

A 47 year old man presented with mastocytosis, a disease process characterised by proliferation of mast cells. The clinical features and outcome are discussed.  (+info)

(4/170) Cutaneous mucinosis and mastocytosis in a shar-pei.

A 7-year-old shar-pei was presented because of a recurrent dermatologic condition. Skin biopsies revealed an idiopathic (primary) cutaneous mucinosis that initially responded to corticosteroids. The condition reappeared 2 years later and subsequent biopsies revealed a mast cell tumor in some of the skin sites previously diagnosed with mucinosis.  (+info)

(5/170) c-Kit and c-kit mutations in mastocytosis and other hematological diseases.

Mast cells (MC) are tissue elements derived from hematopoietic stem cells. Their differentiation and proliferation processes are under the influence of cytokines, including one of utmost importance known as stem cell factor (SCF). SCF receptor is encoded by the protooncogene c-kit, belongs to the type III receptor tyrosine kinase subfamily, and is also expressed on other hematopoietic or non-hematopoietic cells. Ligation of c-kit receptor by SCF induces its dimerization, followed by induction of multiple intracellular signaling pathways leading to cell proliferation and activation. Mastocytosis, a relatively rare group of diseases characterized by accumulation of MC in various tissues, are found isolated or sometimes associated with other hematological malignancies in humans. Although the initial events leading to mastocytosis are not yet unraveled, alterations of the c-kit gene have been described. Particularly interesting are acquired mutations resulting in a constitutively activated receptor, possibly involved in the increased numbers of MC in tissues. For this reason, future strategies might be envisaged to target specifically the mutated c-kit and/or its intracellular signaling.  (+info)

(6/170) A role of mast cell glycosaminoglycans for the immunological expulsion of intestinal nematode, Strongyloides venezuelensis.

We examined effects of mast cell glycosaminoglycans on the establishment of the intestinal nematode, Strongyloides venezuelensis, in the mouse small intestine. When intestinal mastocytosis occurred, surgically implanted adult worms could not invade and establish in the intestinal mucosa. In mast cell-deficient W/Wv mice, inhibition of adult worm invasion was not evident as compared with littermate +/+ control mice. Mucosal mastocytosis and inhibition of S. venezuelensis adult worm mucosal invasion was tightly correlated. To determine effector molecules for the invasion inhibition, adult worms were implanted with various sulfated carbohydrates including mast cell glycosaminoglycans. Among sulfated carbohydrates tested, chondroitin sulfate (ChS)-A, ChS-E, heparin, and dextran sulfate inhibited invasion of adult worms into intestinal mucosa in vivo. No significant inhibition was observed with ChS-C, desulfated chondroitin, and dextran. ChS-E, heparin, and dextran sulfate inhibited adhesion of S. venezuelensis adult worms to plastic surfaces in vitro. Furthermore, binding of intestinal epithelial cells to adhesion substances of S. venezuelensis, which have been implicated in mucosal invasion, was inhibited by ChS-E, heparin, and dextran sulfate. Because adult worms of S. venezuelensis were actively moving in the intestinal mucosa, probably exiting and reentering during infection, the possible expulsion mechanism for S. venezuelensis is inhibition by mast cell glycosaminoglycans of attachment and subsequent invasion of adult worms into intestinal epithelium.  (+info)

(7/170) Nitric oxide mediates intestinal pathology but not immune expulsion during Trichinella spiralis infection in mice.

The relationship between intestinal pathology and immune expulsion of gastrointestinal (GI) nematodes remains controversial. Although immune expulsion of GI helminth parasites is usually associated with Th2 responses, the effector mechanisms directly responsible for parasite loss have not been identified. We have previously shown that while the intestinal pathology accompanying the expulsion of the GI parasite Trichinella spiralis may be dependent on IL-4 and mediated by TNF, parasite loss is independent of TNF. In contrast, intestinal pathology in other disease models has been attributed to Th1 cytokines, although it closely resembles that seen in helminth infections. Whereas production of inducible NO synthase (iNOS) in the gut is important for both homeostasis of the epithelial layer and in protection against pathogenic microorganisms, overproduction of NO has been implicated in the pathogenesis of a number of inflammatory conditions. We therefore investigated the role of NO in T. spiralis infection using iNOS-deficient mice. iNOS-/- and iNOS-/+ mice were infected with T. spiralis, and parasite expulsion and intestinal pathology were followed. Parasite expulsion proceeded similarly in both groups of animals, but significant intestinal pathology was only observed in the heterozygous mice. Thus it appears that, although the protective effects of Th2 responses in GI helminth infection do not require NO, this mediator contributes substantially to the associated enteropathy. NO may therefore be an important mediator of enteropathy in both Th1- and Th2-inducing conditions.  (+info)

(8/170) Pulmonary manifestation of systemic mast cell disease.

Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon alpha-2a over 6 months, the patient's condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.  (+info)