Heparin liberation in urticaria pigmentosa

Guillet, G. Y.

doi:10.1001/archderm.118.7.532

Cited by 8 publications

(5 citation statements)

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“…12,14 In a few cases, heparin-like anticoagulants have also been found in patients suffering from systemic candidiasis or asthma and in patients with cutaneous or systemic mastocyctosis (Table 2). [15][16][17][18] Degranulation of mast cells leads to release of heparin-like anticoagulants and histamine causing increased vascular permeability. The diagnosis of heparin-like anticoagulants is based on prolongation of thrombin time and activated partial thromboplastin time that can be shortened or normalized by heparin neutralization, for example, through application of heparinase or protamine in vitro, in the absence of exogenously administered heparin.…”

Section: Discussionmentioning

confidence: 99%

Fatal Bleeding Due to a Heparin-Like Anticoagulant in a 37-Year-Old Woman Suffering From Systemic Mastocytosis

Sucker

Mansmann

Steiner

et al. 2008

Clin Appl Thromb Hemost

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A 37-year-old female patient with systemic mastocytosis who was admitted with severe unexplained bleeding symptoms is studied. Laboratory procedures established the diagnosis of a patient-derived-heparin-like anticoagulant as a very rare hemostatic abnormality predisposing to bleeding. The patient died from refractory disease despite therapy with protamine, initiation of chemotherapy, and supportive measures. The case illustrates the clinical presentation and diagnosis of heparin-like anticoagulants. Etiology, pathophysiology, and therapeutic options are discussed.

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Section: Discussionmentioning

confidence: 99%

Fatal Bleeding Due to a Heparin-Like Anticoagulant in a 37-Year-Old Woman Suffering From Systemic Mastocytosis

Sucker

Mansmann

Steiner

et al. 2008

Clin Appl Thromb Hemost

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“…Unpredictable episodes of profound hypotension are a recognised feature of the disease and are due to release of mast cell mediators including histamine [11] and prostaglandin D 2 [12]. Heparin release may lead to haemorrhagic manifestations [13]. Attacks may be precipitated by toxins, venoms or drugs (acetylsalicylic acid, alcohol, morphine, codeine, d‐tubocurarine, gallamine, reserpine, quinine, iodine contrast media) [1, 14].…”

Section: Discussionmentioning

confidence: 99%

Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia

Vaughan

Jones

1998

Anaesthesia

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SummaryMastocytosis is a rare disorder with serious anaesthetic implications. Anaesthetic management is hazardous since trauma, stress, extremes of temperature and drugs may precipitate intra-operative mast cell degranulation. Release of histamine and other mast cell mediators can lead to profound cardiovascular collapse and even death. We present a case report of a patient with mastocytosis who suffered cardiac arrest during anaesthesia. Anaphylactoid/anaphylactic shock may be delayed and lack supporting signs of histamine release such as cutaneous flushing and bronchospasm.

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“…32 Prolonged bleeding of the skin or the gastrointestinal tract has been reported in children with diffuse cutaneous mastocytosis. 12,37 In a few patients, systemic mastocytosis may develop into an aggressive form, with severe systemic symptoms including fever, hypotension, frequent episodes of flushing, fatigue and cachexia. Whereas mast cells are usually not detectable in peripheral blood, a leukaemic spread of circulating, immature mast cells has been reported in these patients during the terminal stage of their disease.…”

Section: Systemic Manifestationsmentioning

confidence: 99%

“…Neuropsychiatric abnormalities such as irritability, poor attention span, depression and impairment of short‐term memory have also been observed 32 . Prolonged bleeding of the skin or the gastrointestinal tract has been reported in children with diffuse cutaneous mastocytosis 12,37 …”

Section: Clinical Presentationmentioning

confidence: 99%

Mastocytosis: recent advances in defining the disease

2001

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Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues, which may present in a variety of clinical patterns. Major advances have been made in recent years in the understanding of the pathogenesis of mastocytosis. This review is aimed at familiarizing dermatologists with these recent findings, and at exploring their possible implications for the diagnosis and treatment of the condition. The heterogeneous clinical presentation of mastocytosis is detailed with respect to the type of skin lesions, age at onset, family history, organ systems involved, associated haematological disorders and prognosis. Recent genetic findings also indicate different pathogenetic forms of mastocytosis, as adult patients and those with associated haematological diseases usually express activating mutations of the stem cell factor receptor c-kit, whereas most cases of childhood-onset and familial mastocytosis seem to lack these mutations. Despite the presence of c-kit mutations, patients with cutaneous lesions generally have a good prognosis, even when there is involvement of other organs. Some patients, particularly those with childhood-onset disease, experience spontaneous remission, mostly by puberty. c-kit mutations do not explain the initial cause of mastocytosis, and their prognostic significance is as yet unclarified, as is the pathogenesis in patients without the mutations. Furthermore, these novel findings have as yet not resulted in a more effective treatment of the cause of the disease, so that counselling, prevention of exposure to mast cell secretory stimuli, and symptomatic treatment remain the mainstays of current patient management.

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Heparin liberation in urticaria pigmentosa

Cited by 8 publications

References 0 publications

Fatal Bleeding Due to a Heparin-Like Anticoagulant in a 37-Year-Old Woman Suffering From Systemic Mastocytosis

Fatal Bleeding Due to a Heparin-Like Anticoagulant in a 37-Year-Old Woman Suffering From Systemic Mastocytosis

Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia

Mastocytosis: recent advances in defining the disease

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