[Shock and coagulation disorders in systemic mastocytosis]

Area of Science:

• Dermatology
• Hematology
• Allergy and Immunology

Background:

• Systemic mastocytosis is a rare disorder characterized by abnormal mast cell accumulation.
• Mast cell activation can lead to the release of various mediators, including histamine and heparin.
• Cutaneous manifestations like urticaria pigmentosa are common in systemic mastocytosis.

Observation:

• A 41-year-old man presented with circulatory shock, flushing, and maculopapular rash after consuming large amounts of beer.
• Initial management with colloidal solution stabilized his blood pressure.
• Recurrent episodes of flushing and collapse were associated with beer consumption.

Findings:

• Laboratory tests revealed prolonged coagulation times (thromboplastin, partial thromboplastin, and thrombin times), which normalized within 20 hours.
• Elevated serum levels of heparin, histamine, and serotonin were detected after provocation.
• Bone marrow biopsy showed dense mast cell collections, consistent with systemic mastocytosis.
• Skin biopsy confirmed urticaria pigmentosa.

Implications:

• This case highlights a potential trigger (beer consumption) for mast cell activation in patients with systemic mastocytosis.
• The findings suggest that alcohol, or a component thereof, can induce mediator release leading to anaphylactoid reactions.
• Early recognition and avoidance of triggers are crucial for managing patients with systemic mastocytosis and preventing severe reactions.