- Sergio Serrano Villar, resident in internal medicine1,
- Juncal Perez-Somarriba, consultant in internal medicine 1,
- Talia Sainz Costa, resident in paediatrics1,
- Sarah Winstanley, foundation doctor2,
- Tomas Santillana Lopez, consultant in internal medicine1,
- Luis Escribano Mora, consultant in haematology3,
- Baltasar Orejas Gonzalez, professor in internal medicine1
- 1Hospital Clinico San Carlos, Madrid 28040, Spain
- 2Kingston Hospital, London
- 3Centro de Estudios de Mastocitosis de Castilla la Mancha, Hospital Virgen del Valle, Toledo, Spain
- Correspondence to: S Serrano Villar sergio1serrano{at}yahoo.es
Four weeks ago (BMJ 2009;338:b6, doi:10.1136/bmj.b6) we presented the case of Mrs Barroso who presented with serious hypotensive shock triggered by the onset of menstruation. All investigations were negative, except for retroperitoneal fibrosis and periaortic adenopathies found on computed tomography (BMJ 2009;338:b246, doi:10.1136/bmj.b246).
Subsequently, we measured her serum tryptase concentration, which was 30 μg/l (normal range <12 μg/l. Tryptase is an endoprotease exclusively present in mast cells; hence it is a sensitive marker of conditions in which mast cells are implicated, such as anaphylaxis and mastocytosis.
A bone marrow biopsy sample showed normocellularity and multifocal aggregates of abnormal spindle shaped mast cells (figs 1⇓, 2⇓, and 3⇓). Mutation of codon …
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