Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA).

BACKGROUND: Mast cells (MCs) play a key role in allergic diseases through the release of inflammatory mediators, which are responsible of allergic symptoms. Mastocytosis is characterized by an abnormal proliferation and accumulation of mast cells, in which mediators are released intermittingly or continuously. Despite these clinical similarities, few studies have addressed the presence of allergic symptoms in mastocytosis patients, including anaphylaxis. OBJECTIVE: A prospective evaluation was carried out to study the prevalence of allergic diseases in patients with mastocytosis and their impact on the natural history of mastocytosis. METHODS: A questionnaire was given to 210 patients with mastocytosis to evaluate the history of asthma, rhinitis, conjunctivitis, atopic dermatitis, urticaria and anaphylaxis. Patients underwent total IgE, Phadiatop infant (aeroallergens and food allergens), specific IgE to latex and to Anisakis simplex determinations. Skin tests were done to 72 patients. RESULTS: The prevalence of allergy, as defined by clinical symptoms associated to specific IgE, was 23.9%. Total IgE level was significantly higher in patients with allergy as compared with patients without allergy (median 58 vs. 16.5 kU/L, P<0.0001). Anaphylactic symptoms were present in 36 patients (22%), in nine the allergen was identified. Males had more allergy and anaphylactic symptoms than females (61.5% vs. 38.5% and 72% vs. 28%, respectively). CONCLUSIONS: Allergic diseases coexist in patients with mastocytosis with similar frequency as compared with the general population. Anaphylactic symptoms are more prevalent in males with mastocytosis and in patients with elevated IgE. CAPSULE SUMMARY: The prevalence of allergy in mastocytosis is similar to the general population. Anaphylactic symptoms are more prevalent in males and in patients with elevated IgE. The coexistence of atopy does not influence mastocytosis-associated symptoms.

[Idiopathic thrombocytopenic purpura as first manifestation of systemic lupus erythematosus lupus]. / Lupus eritematosos sistémicos que debutan con púrpura trombopénica idiopática.

OBJECTIVES: We studied SLE whose initial symptoms were related to idiopathic thrombocytopenic purpura (ITP) in order to determine if they share clinical and immunologic manifestations. METHODS: We reviewed the clinical backgrounds of 150 SLE (according to the ACR criteria) periodically followed from 1980 until 1998. We found 12 patients with both these conditions. RESULTS: All patients were female with a mean age of 32 at the time of ITP diagnosis and 36 at the time of SLE diagnosis. The most usual clinical manifestations were: arthritis (92%), cutaneous (58%) and hematologic involvement with lymphopenia (58%) and thrombocytopenia again (33%) after the initial ITP episode, always together with autoimmune hemolytic anemia (Evans syndrome). None of these patients presented with neurologic involvement and only one presented with renal involvement. 50% were positive for anti-DNA antibodies, 50% were Ro(+) and 16% were RNP (+). 66% were positive for antiphospholipid antibodies and 33% for lupus anticoagulant. Thrombocytopenia was controlled just with steroids in only 16% of the patients. Splenectomy controlled thrombocytopenia with complete remission achieved in 80% (4 from 5) of the patients and 20% (1 from 5) were refractory to this therapy after a medium follow-up time of 6.5 years. CONCLUSIONS: 1) SLE whose initial symptoms are related to ITP were characterized by joints, cutaneous and hematologic involvement without renal and neurologic manifestations. 2) Splenectomy was able to control refractory thrombocytopenia in the majority of these patients.

[Language and aphasias]. / Lenguaje y afasias.

INTRODUCTION AND OBJECTIVE: Approximately 400,000 years ago men started to use language. Initially it was probably poor with few phonemes. With social evolution it became more complex, with the appearance of new phonemes and a more complete grammatical structure. The current concept of the processing of language dates, with little change, from the nineteenth century. DEVELOPMENT: With the birth of phrenology language began to be studied. This lead to the hypothesis of Wernicke, with two main areas joined by the fasciculo arcuato, which is still held to be valid with modifications by Gerchwind and Damasio, amongst others. Important advances in the study of language are due to Chomsky and his transformational grammar. This supports the universal structure of language, since one learns it following genetically determined laws. Language has three main aspects: creativity which makes both the transmitter and the receiver active participants in communication, the form from which words are constructed and the content of the message. Aphasia is an alteration in the comprehension and understanding of language, which may be the clinical expression of many different aetiologies. They help us to localize the lesion topographically. They are divided depending on the clinical signs, into motor or Broca's aphasia, in which understanding is conserved but the patient uses a language with poor grammatical structure, although the semantic content is acceptable: sensitive or Wernicke's aphasia, with inability to understand and language which is fluid but unintelligible; conduction aphasia due to limitation in the transmission of impulses from Wernicke's area to that of Broca, with acceptable understanding and fluid language and the trans-cortical aphasias where the main characteristic is indemnity of the capacity of repetition. CONCLUSIONS: The aphasias, as the expression of an alteration of language are an important support in the topographical localization of lesions, even before these can be shown on computerized tomography.

[Clinical manifestations associated with antiphospholipid antibodies]. / Manifestaciones clínicas asociadas a anticuerpos antifosfolípido.

The antiphospholipid antibodies are immunoglobulins able to join negative charge phospholipids. The have been related to a great variety of conditions, specially among connective tissue illness although the idiopathic form seems to be the most frequent. Their presence must be ruled out in cases of young patients with stroke, deep veins thrombosis, acute heart attack and woman suffer multiple abortions and foetal death. These antibodies appear to be related to different clinical entities like Sneddon syndrome. Evans syndrome, "chorea gestationis", migraine. The laboratory determinations are based in direct methods (ELISA, RIA, ...) as well as in indirect ones (activated partial thromboplastin time, reptilase time, ...). The appropriate management and treatment may be based upon clinical expression, in case of arterial thrombosis (type II APS), or deep vein thrombosis (Type II) long term anticoagulation is indicated; Association with pentoxifylline in the case of retinal thrombosis (type IIIa), Stroke (type IIIb) cases may require long term anticoagulation as well as aspirin. Type IV cases are better managed with an individualised treatment.