Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms.

BACKGROUND: Systemic mast cell activation disorders (MCADs) are characterized by severe and systemic mast cell (MC) mediators-related symptoms frequently associated with increased serum baseline tryptase (sBt). OBJECTIVE: To analyze the clinical, biological, and molecular characteristics of adult patients presenting with systemic MC activation symptoms/anaphylaxis in the absence of skin mastocytosis who showed clonal (c) versus nonclonal (nc) MCs and to provide indication criteria for bone marrow (BM) studies. METHODS: Eighty-three patients were studied. Patients showing clonal BM MCs were grouped into indolent systemic mastocytosis without skin lesions (ISMs(-); n = 48) and other c-MCADs (n = 3)-both with CD25(++) BM MCs and either positive mast/stem cell growth factor receptor gene (KIT) mutation or clonal human androgen receptor assay (HUMARA) tests-and nc-MCAD (CD25-negative BM MCs in the absence of KIT mutation; n = 32) and compared for their clinical, biological, and molecular characteristics. RESULTS: Most clonal patients (48/51; 94%) met the World Health Organization criteria for systemic mastocytosis and were classified as ISMs(-), whereas the other 3 c-MCAD and all nc-MCAD patients did not. In addition, although both patients with ISMs(-) and patients with nc-MCAD presented with idiopathic and allergen-induced anaphylaxis, the former showed a higher frequency of men, cardiovascular symptoms, and insect bite as a trigger, together with greater sBt. Based on a multivariate analysis, a highly efficient model to predict clonality before BM sampling was built that includes male sex (P = .01), presyncopal and/or syncopal episodes (P = .009) in the absence of urticaria and angioedema (P = .003), and sBt >25 microg/L (P = .006) as independent predictive factors. CONCLUSIONS: Patients with c-MCAD and ISMs(-) display unique clinical and laboratory features different from nc-MCAD patients. A significant percentage of c-MCAD patients can be considered as true ISMs(-) diagnosed at early phases of the disease.

Monofilamento 5.07. Uso en las consultas de Enfermería de Atención Primaria / 5.07 Monofilament: the use of this monofilment in outpatients offices by nurses in primary health care units

El pie diabético es la causa más importante de invalidez y pérdida de calidad de vida en los diabéticos. La inspección del pie y la exploración de la sensibilidad protectora con el monofilamento 5.07 son métodos eficientes para la detección del riesgo de lesiones. Nuestro objetivo era conocer si se utiliza este instrumento en nuestro Servicio de Atención Primaria, para lo que pasamos una encuesta a todas sus enfermeras. El 79% de ellas conoce el monofilamento 5.07; el 62% dispone de él; y el 55% lo utiliza. Los motivos esenciales de no utilización son la falta de disponibilidad y de conocimientos. Es prioritario facilitar este recurso y fomentar los conocimientos sobre su correcta utilización

A diabetic foot is the most important cause of disability and loss of life quality among diabetics. To inspect a diabetic's feet and to explore his/her protective sensibility capability with a 5.07 monofilament are efficient methods to detect the risk of lesions. The authors' objective was to discover if our Primary Health Care Unit utilizes this instrument; therefore, we carried out a poll among all the nurses in our unit. 79% of these nurses are aware of the 5.07 monofilament; 62% have this monofilament available for their use; 55% stated they utilize it. The primary reasons to not use this monofilament are its unavailability and a lack of knowledge regarding it. Priority must be given to facilitate the availability of this instrument and to promote among nurses an understanding how to correctly utilize a 5.07 monofilament

[5.07 Monofilament: the use of this monofilament in outpatient offices by nurses in primary health care units]. / Monofilamento 5.07. Uso en las consultas de Enfermería de Atención Primaria.

A diabetic foot is the most important cause of disability and loss of life quality among diabetics. To inspect a diabetic's feet and to explore his/her protective sensibility capability with a 5.07 monofilament are efficient methods to detect the risk of lesions. The authors' objective was to discover if our Primary Health Care Unit utilizes this instrument; therefore, we carried out a poll among all the nurses in our unit. 79% of these nurses are aware of the 5.07 monofilament; 62% have this monofilament available for their use; 55% stated they utilize it. The primary reasons to not use this monofilament are its unavailability and a lack of knowledge regarding it. Priority must be given to facilitate the availability of this instrument and to promote among nurses an understanding how to correctly utilize a 5.07 monofilament.