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

Sucker, Christoph; Mansmann, Georg; Steiner, Stefan H.; Gattermann, Norbert; Schmitt-Graeff, Anette; Loncar, Robert; Scharf, Rüdiger E.; Stockschläder, M

doi:10.1177/1076029607309173

Cited by 32 publications

(33 citation statements)

References 23 publications

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“…Abrupt hypotension due to iodinated contrast in a patient with urticaria pigmentosa has also been described in the literature [19]. Excessive bleeding associated with mastocytosis may be due to heparin release by mast cells [20]- [23].…”

Section: Discussionmentioning

confidence: 91%

Peripheral Nerve Blockade in a Patient with Mastocytosis

Bailey¹,

Weinmeister

2015

OJAnes

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Mastocytosis is a rare group of disorders with chronic and episodic mast cell release of mediators which can have systemic and cutaneous manifestations. Triggers of anaphylaxis include commonly used medications for anesthesia, analgesia, and muscle relaxation. There is little in the literature regarding local anesthesia in emergent surgery for patients with mastocytosis. This case details the use of a peripheral nerve blockade for multiple surgeries and pain control in a patient with biopsy-proven mastocytosis.

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Section: Discussionmentioning

confidence: 91%

Peripheral Nerve Blockade in a Patient with Mastocytosis

Bailey¹,

Weinmeister

2015

OJAnes

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“…Heparin-like anticoagulants have been described in a number of other diseases, including transitional cell carcinomas [6,7], breast cancer [8], multiple myeloma [9], systemic mastocytosis [10] and hepatocellular carcinoma [11].…”

Section: Discussionmentioning

confidence: 99%

“…Due to its uncommon occurrence, the treatment of HLArelated bleeding disorders is far from clear; the administration of protamine is anecdotal [9], and its efficacy equivocal [7,10,11].…”

Section: Discussionmentioning

confidence: 99%

An uncommon cause of postoperative bleeding

Berlot

Tartamella²,

Bussani³

et al. 2011

Blood Coagulation &Amp; Fibrinolysis

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The postoperative phase can be associated with an increased risk of thrombosis and abnormal bleeding. We report the case of a patient admitted to our intensive care unit for postoperative monitoring who developed two episodes of hemorrhagic shock. A thromboelastogram performed with heparinase I digestion demonstrated the action of endogenous heparin-like substances whose presence was subsequently confirmed at the histological examination of the tumor. Heparinase-modified thromboelastography has proven to be an effective and rapid point-of-care coagulation test and in our case allowed early detection of circulating heparinoids.

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“…32,47,48 There have been multiple case reports of excessive bleeding in patients with SM. [49][50][51] The propensity to bleed is unlikely related to thrombocytopenia (which is usually mild), but instead may be related to heparin-like anticoagulants caused by MC degranulation, along with the disruption of fibrinogen function by trypase. 51,52 Importantly, the increase in MC burden does not affect B-cell and T-cell number or function and, therefore, patients with SM are not immunocompromised, except in cases of advanced SM and SM-AHNMD when the bone marrow is directly affected.…”

Section: Clinical Manifestationsmentioning

confidence: 99%