Severe Life-Threatening or Disabling Anaphylaxis in Patients with Systemic Mastocytosis: A Single-Center Experience

Abstract

Background: Mediator-related symptoms in patients with systemic mastocytosis (SM) range from mild episodic to severe life-threatening events. Methods: We examined a series of 137 consecutive patients with mastocytosis (63 females and 74 males) referred to our center between 1988 and 2010. Almost all patients received prophylactic histamine receptor (HR1 and HR2) antagonists. Results: Forty-two patients suffered from one or more mediator-related symptoms (hypotension, headache, flush, abdominal cramping, diarrhea) requiring therapy (SM_SY). Severe life-threatening events (grade IV) occurred in 17 patients (12%). In 4 of these 17 patients, a deteriorating clinical course was recorded. One patient died of an apallic syndrome 1.5 years after an hymenoptera sting and cerebral hypoxia. One patient was disabled for months after an insect sting and cerebral hypoxia. Two patients with smoldering SM (SSM) suffered from severe recurrent hypotension requiring hospitalization and repeated resuscitation. Symptoms in these SSM patients did not respond to any of the antimediator-type drugs applied. However, after therapy with cladribine (2CdA), a major durable response was obtained in both cases. In patients with aggressive SM and mast cell leukemia (n = 6), life-threatening mediator-related events (grade IV) were not recorded. Conclusions: SM may be accompanied by life-threatening mediator-related symptoms. Most of these patients have indolent SM or SSM. In patients with SSM_SY with uncontrolled symptoms (grade IV), therapy with 2CdA should be considered.

Proposed diagnostic algorithm for patients with suspected mastocytosis: a proposal of the European Competence Network on Mastocytosis

“…Some of these patients suffer from an IgE-dependent allergy (18)(19)(20)(21)(22)(23)(24)(25). The symptoms recorded may be mild, but can also be severe or even life-threatening (18)(19)(20)(21)(22)(23)(24)(25).…”

“…Some of these patients suffer from an IgE-dependent allergy (18)(19)(20)(21)(22)(23)(24)(25). The symptoms recorded may be mild, but can also be severe or even life-threatening (18)(19)(20)(21)(22)(23)(24)(25). Various organ systems, including the skin (pruritus and flushing), the gastrointestinal tract (peptic symptoms, cramping and diarrhoea) and the central nervous system (headache, depression, moods and cognitive symptoms), may be involved (1)(2)(3)(4)(5)(6)(7)(8)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27).…”

“…The symptoms recorded may be mild, but can also be severe or even life-threatening (18)(19)(20)(21)(22)(23)(24)(25). Various organ systems, including the skin (pruritus and flushing), the gastrointestinal tract (peptic symptoms, cramping and diarrhoea) and the central nervous system (headache, depression, moods and cognitive symptoms), may be involved (1)(2)(3)(4)(5)(6)(7)(8)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27). In a smaller group of these patients, a mast cell activation syndrome (MCAS) is recorded (18)(19)(20)(21)(22)(23)(24)28).…”

“…Various organ systems, including the skin (pruritus and flushing), the gastrointestinal tract (peptic symptoms, cramping and diarrhoea) and the central nervous system (headache, depression, moods and cognitive symptoms), may be involved (1)(2)(3)(4)(5)(6)(7)(8)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27). In a smaller group of these patients, a mast cell activation syndrome (MCAS) is recorded (18)(19)(20)(21)(22)(23)(24)28). Finally, patients with SM may also suffer from osteopenia or osteoporosis (sometimes with bone fractures) and from the cosmetic consequences of the disease (18,26,29) (Table 1).…”

“…In these patients, symptoms are variable and often atypical, and the basal tryptase level may be relatively low. Some of these patients may suffer from an IgE-dependent allergy, including hymenoptera venom allergy (19)(20)(21)(22)(23)(24). In the past few years, an increasing number of such cases, many of them without definitive signs or symptoms of mastocytosis or another myeloid neoplasm that could explain an increased tryptase level, have been referred to specialized centres.…”