Cases reported "Mastocytosis"

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1/33. 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.
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2/33. Clinical stable systemic mastocytosis with interferon alpha-2b therapy.

    Systemic mastocytosis (SM) with progressive infiltration of various organs by mast cells is a rare disease with a mean survival of 2 years. So far there is no established therapy. We describe a patient with progressive mastocytosis who had a clinical response to interferon alpha-2b 3 x 5 Mio U s.c. three times a week. The response is stable 3 years after initiation of therapy and includes reduction of abdominal lymphadenopathy, ascites, decrease of serum and urinary histamine levels as well as pulmonary reticulonodular markings.
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3/33. Diffuse cutaneous mastocytosis with bone marrow infiltration in a child: a case report.

    mastocytosis encompasses a range of disorders characterized by overproliferation and accumulation of tissue mast cells. Mast cell disease is most commonly seen in the skin, but the skeleton, gastrointestinal tract, bone marrow, and central nervous system may also be involved. We present a 10-year-old boy with diffuse cutaneous mastocytosis characterized by disseminated papular, nodular, and infiltrated leathery lesions. The patient presented with chronic diarrhea and malnutrition. Laboratory studies were normal except for an elevated urinary 1-methylhistamine level. The bone marrow aspirate showed a dense mast cell infiltrate confirming systemic involvement.
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4/33. A solitary manifestation of mastocytosis in the head and neck.

    Solitary mastocytosis in adulthood is a rare finding. Only two such lesions have been reported in the head and neck. We describe a 27-year-old woman who had a 10-year history of a forehead swelling that had fluctuated in size. light trauma or pressure on the lesion resulted in an increase in its size. A mass was found to be situated just below the galea and was successfully removed surgically using a high forehead lift. Histologically, the specimen contained predominantly mast cells. A systemic mastocytosis was excluded by a multidisciplinary diagnostic approach and measurement of the 24-h urinary excretion of histamine metabolites. After 36 months of follow-up there has been no recurrence.
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5/33. Effects of interferon-alpha2b treatment on ex vivo differentiation of mast cells from circulating progenitor cells in a patient with systemic mastocytosis.

    Interferon (IFN)-alpha, a known inhibitor of myelopoiesis, is increasingly used to treat patients with systemic mastocytosis (SM). However, the mechanisms of IFN-alpha effects on mast cell (MC) growth remain unknown, and the treatment responses may be variable. In the present study, factor-dependent ex-vivo differentiation of MCs from peripheral blood mononuclear cells (PBMNCs) was analyzed in a patient with SM treated with IFN-alpha2b (3 million U/day). The patient exhibited an extensive MC infiltration in his bone marrow (BM) and increasing serum total tryptase levels (spiking to > 1,400 ng/ml). PBMNCs were collected before and during IFN-alpha2b treatment and cultured in the presence or absence of stem cell factor (SCF, 100 ng/ml) for 42 days. In the absence of SCF, no MC growth was detectable. However, in the presence of SCF, MC containing tryptase appeared in the cultures. Treatment with IFN-alpha2b resulted in a time-dependent decrease in SCF-inducible formation of MCs from PB progenitor cells in vitro. Also, during IFN-alpha2b treatment, blood histamine concentrations decreased. serum total tryptase levels initially increased despite IFN-alpha2b treatment. However, after a latency period of a few months, tryptase concentrations declined and then reached a plateau. In healthy individuals, the SCF-induced in vitro growth of MCs from their progenitor cells was also inhibitable by the addition of IFN-alpha2b. In summary, our data show that IFN-alpha2b can exhibit inhibitory effects on factor-dependent growth of MC progenitor cells. However, it still remains open which of the patients with mastocytosis can benefit from long-term IFN-alpha treatment.
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6/33. Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: case report.

    Severe generalized nodular and bullous mastocytosis of the xanthelasmoid type is described in a 7-month-old boy. Reddish to yellowish-brown xanthelasmoid papules and nodules first developed in the inguinal region a few weeks after birth and then progressively spread to cover nearly the entire body surface. There was severe pruritus and recurrent episodes of blistering. The diagnosis of cutaneous mastocytosis of the xanthelasmoid type with subepidermal bullae was confirmed by skin biopsies showing solid and deeply penetrating infiltrates of metachromatic mast cells under light and electron microscopy. Systemic involvement of other organs, however, was excluded by bone scintigraphy, abdominal ultrasound, bone marrow aspiration and echocardiography. The extensive skin involvement was reflected in highly elevated urinary levels of histamine (263.4 microg L(-1)) and its metabolite N-methylimidazole acetic acid (20.8 mg L(-1)). The patient was systematically well and received only symptomatic treatment. Over a period of 1 year, the condition gradually improved, and the skin lesions began to flatten and regress.
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keywords = histamine
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7/33. Dental management of a pediatric patient with mastocytosis: a case report.

    mastocytosis is a heterogeneous group of clinical disorders characterized by an excessive number of normal mast cells in a variety of tissues (skin, bone marrow, liver, spleen and lymph nodes). It is most often seen in the skin in pediatric-onset mastocytosis presenting as urticaria pigmentosa. Children with this disorder are on a strict avoidance protocol of triggering factors to decrease the likelihood of life-threatening anaphylactic reactions. Close monitoring and the avoidance of known histamine-releasing drugs is necessary in the pediatric dental office, as is a readiness to use resuscitative measures. A case of a 4-year, 6-month-old pediatric dental patient with mastocytosis is presented. Dental treatment was provided in an ambulatory setting utilizing nitrous oxide, oxygen analgesia and H1 and H2 antihistamines to prevent mast cell degranulation and to provide sedation.
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keywords = histamine
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8/33. radiation therapy in a case of systemic mastocytosis: evaluation of histamine levels and mucosal effects.

    plasma histamine levels were obtained during palliative radiation therapy of the spine involved with systemic mastocytosis in a 68-year-old woman. Baseline plasma histamine levels were obtained before irradiation and compared to levels obtained on the third, fifth, eighth, and tenth treatment days. Despite concerns regarding histamine release with mast cell destruction following irradiation, plasma histamine levels remained within normal limits. No change in dermatologic or other systemic manifestations were observed. No untoward systemic or localized sequelae associated with mast cell degranulation in response to the administered large field of radiation was observed. Effective palliation was accomplished, and it was concluded that radiation therapy can effectively be applied in treatment of systemic mast cell disease without significant morbidity.
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keywords = histamine
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9/33. Systemic mastocytosis treated with histamine H1 and H2 receptor antagonists.

    A case of systemic mastocytosis with skin characteristics of telangiectasia macularis eruptiva perstans is reported. Systemic involvement was confirmed as a combination of highly increased urinary excretion of methyl imidazole acetic acid (Melm AA) and increased amounts of mast cells in skin, liver and colon transversum. Treatment with cimetidine (H2 receptor antagonist) resulted in diarrhea, however, the addition of cyproheptadine (H1 receptor antagonist) was beneficial in amelioration of the cutaneous symptoms of mastocytosis. The excretion of Melm AA was unchanged during treatment. After 7 months of continuous cimetidine and cyproheptadine treatment no side effects were observed.
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keywords = histamine
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10/33. Anaesthetic management of systemic mastocytosis.

    Systemic mastocytosis is an uncommon disorder of mast cell proliferation in connective tissues. Mast cell degranulation may occur on exposure to various stimuli and drugs. The release of histamine, heparin and vasoactive substances such as prostaglandin d2 may cause severe hypotension and other anaphylactoid manifestations. Anaesthetic management should include perioperative stabilization of mast cells and avoidance of the use of histamine-releasing drugs. Intradermal skin testing is useful in predicting the sensitivity to drugs that may be used during anaesthesia. We present a patient with systemic mastocytosis who underwent uneventful cholecystectomy.
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keywords = histamine
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