Compromiso óseo en mastocitosis sistémica: una asociación frecuente, pero muchas veces inadvertida / Bone involvement in systemic mastocytosis: report of two cases

Systemic mastocytosis (SM) is characterized by pathologic expansion and activation of mast cells. The main clinical manifestations of SM include skin involvement, gastrointestinal symptoms and anaphylaxis due to the release of its mediators. Thirty percent of pat ients with SM have a low bone mass and 20% fractures. At the same time, SM affects 10% of male patients with idiopathic osteoporosis. Measuring serum tryptase is essential for the screening of MS. We report two cases of SM with bone involvement. A 25-year- old woman with prior diagnosis of SM, based on skin involvement, flushing, high serum tryptase and compatible bone marrow (BM) biopsy and genetic study. Low bone mass was diagnosed and treatment was started with calcium and vitamin D plus oral bisphosphona tes with adequate response. A 47 years old man who presented with multiple osteoporotic vertebral fractures and low bone mass. Treatment with vitamin D and alendronate was started, but the patient developed new vertebral fractures. The study was extended w ith measurement of serum tryptase that was elevated. Diagnosis of SM was confirmed with BM biopsy and the patient was referred to hematology for specific care. These cases emphasize the importance of bone assessment in SM, as well as the need to rule out S M in patients with osteoporosis and no evident cause.

Psychosocial factors and mental work load: a reality perceived by nurses in intensive care units / Fatores psicossociais e carga mental de trabalho: uma realidade percebida pelos enfermeiros em Unidades de Terapia Intensiva / Factores psicosociales y Carga mental de trabajo: una realidad percibida por enfermeras/os en Unidades Críticas

OBJECTIVE: To analyse the perception of psychosocial factors and mental workload of nurses who work in intensive care units. It is hypothesised that nurses in these units could perceive psychosocial risks, manifesting in a high mental work load. The psychosocial dimension related to the position's cognitive demands is hypothesised to mostly explain mental work load. METHOD: Quantitative study, with a descriptive, cross-sectional, and comparative design. A total of 91% of the intensive care unit populations of three Chilean hospitals was surveyed, corresponding to 111 nurses. The instruments utilised included (A) a biosociodemographic history questionnaire; (b) the SUSESO-ISTAS 21 questionnaire; and (c) the Mental Work Load Subjective Scale (ESCAM, in Spanish). RESULTS: In total, 64% and 57% of participants perceived high levels of exposure to the psychosocial risks Psychosocial demands and Double shift, respectively. In addition, a medium-high level of overall mental load was observed. Positive and significant correlations between some of the SUSESO-ISTAS 21 and ESCAM dimensions were obtained. Using a regression analysis, it was determined that three dimensions of the psychosocial risk questionnaire helped to explain 38% of the overall mental load. CONCLUSION: Intensive care unit nurses felt that inadequate psychosocial factors and mental work overload existed in several of the tested dimensions. .

OBJETIVO: analisar a percepção de fatores psicossociais e a carga mental de trabalho de enfermeiros que trabalham em unidades de terapia intensiva. A hipótese é que os enfermeiros dessas unidades podem perceber os riscos psicossociais e manifestar uma alta carga mental de trabalho. Além disso, a dimensão psicossocial relacionada às demandas cognitivas do cargo explicaria a maior parte da carga mental de trabalho. MÉTODO: estudo quantitativo, com delineamento descritivo, transversal e comparativo. Foi examinada 91% da população das Unidades de Terapia Intensiva de três hospitais chilenos, correspondente a 111 enfermeiros. Os instrumentos utilizados incluíram (a) um questionário do histórico biossociodemográfico; (b) o questionário SUSESO-ISTAS 21; e (c) a Escala Subjetiva de Carga Mental de Trabalho (ESCAM). RESULTADOS: no total, 64% e 57% dos participantes perceberam um alto nível de exposição aos riscos psicossociais Demanda psicológica e Jornada dupla, respectivamente. Além disso, foi observado um nível de médio para alto de carga mental global. Foram obtidas correlações positivas e significativas entre algumas das dimensões do SUSESO-ISTAS 21 e do ESCAM. Utilizando uma análise de regressão, determinou-se que três dimensões do questionário de risco psicossocial ajudaram a explicar 38% da carga mental total. CONCLUSÃO: os enfermeiros das unidades de terapia intensiva percebem os fatores psicossociais e a sobrecarga mental de trabalho em várias de suas dimensões. .

OBJETIVO: analizar la percepción de Factores psicosociales y Carga mental de trabajo de enfermeras/os que laboran en Unidades Críticas. Se hipotetiza que los/as enfermeros/as de estas unidades pudieran percibir riesgos psicosociales; Mostrarán una Carga mental de trabajo alta; y la dimensión psicosocial relacionada con exigencias cognitivas del puesto explicará en mayor medida la Carga Mental. MÉTODO: estudio cuantitativo, de diseño descriptivo, transversal y comparativo. Se censó el 91% de la población de Unidades Críticas de tres hospitales chilenos, correspondiente a 111 enfermeras/os. Los instrumentos utilizados fueron: (a) Cuestionario de antecedentes biosociodemográficos; (b) Cuestionario SUSESO-ISTAS 21; y (c) Escala Subjetiva de Carga Mental de Trabajo (ESCAM). RESULTADOS: el 64% y el 57% de los/as participantes perciben un alto nivel de exposición a los riesgos psicosociales Demandas psicológicas y Doble presencia, respectivamente. Además, se obtiene un nivel de Carga mental global media-alta. Se obtuvo correlaciones positivas y significativas entre algunas dimensiones de SUSESO-ISTAS 21 y ESCAM, y mediante un análisis de regresión se obtuvo que tres dimensiones del cuestionario de riesgos psicosociales contribuyen a explicar un 38% de la Carga mental global. CONCLUSIÓN: las/os enfermeras/os de unidades críticas, perciben factores psicosociales inadecuados y sobrecarga mental de trabajo, en varias de sus dimensiones. .

Systemic mastocytosis with associated acute myeloid leukemia with t (8; 21) (q22; q22).

Systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD) is a subtype of mastocytosis associated commonly with myeloid neoplasms, Non-Hodgkin's lymphoma, or other hematological neoplasms. In these conditions, mastocytosis needs to be differentiated from mast cell hyperplasia or mast cell activation states. Neoplastic nature of mastocytosis is proved either by morphology, aberrant immunophenotype, or detection of point mutation at codon-816 of c-kit gene. This is a rare entity, even more so in pediatric population. Herein, we report a case of 14-year-old girl with SM associated with acute myeloid leukemia with maturation with t(8;21). Multifocal dense infiltrate of spindle-shaped mast cells on bone marrow aspirate and biopsy with coexpression of CD2 and CD25 by flow cytometric analysis proved the SM component at the time of diagnosis and persistence at post induction status also.

Mastocitosis sistémica: repercusión ósea / Systemic mastocytosis: bone impact

La mastocitosis sistémica es una enfermedad caracterizada por acumulación de mastocitos en varios órganos, de los cuales el más afectado es la piel. La repercusión ósea de esta enfermedad es poco frecuente y en general se la asocia al desarrollo de osteoporosis secundaria con o sin fracturas óseas. Se presentan tres pacientes con lesiones características en piel de mastocitosis y diferentes manifestaciones óseas; el primer caso es una mujer de 51 años en la que se observó una variante esclerosante muy poco frecuente, con densitometría ósea en valores normales-altos y aumento de la densidad ósea observada en varias vértebras por radiografías. Una punción-biopsia de cresta ilíaca confirmó el compromiso óseo de su enfermedad de base. El segundo caso fue una mujer de 57 años que mostró signos característicos de mastocitosis sistémica con diarreas, gastritis, flushes y reacciones cutáneas particulares ante exposición a alérgenos. Se observó además grave disminución de la densidad mineral ósea tanto en columna lumbar como en cuello de fémur con parámetros bioquímicos de aumento de la resorción ósea, por lo que fue necesario tratarla con bisfosfonatos. La tercera paciente, de 67 años de edad, presentó varias fracturas vertebrales, con densitometría ósea levemente disminuida. En esta última paciente se constató una hiperplaquetosis con diagnóstico histológico de trombocitemia esencial, cuadro que suele asociarse a la mastocitosis sistémica. En definitiva, se presentan y discuten tres variantes óseas diferentes de mastocitosis sistémica.

Systemic mastocytosis is a disease characterized by accumulation of mast cells in various organs of which the most affected is the skin. The bone impact of this disease is very rare and generally associated with the development of secondary osteoporosis with or without fractures. We present three cases of patients with skin mastocytosis lesions and different bone manifestations; the first case was a 51 year old woman in whom we observed a rare sclerosing variant with a normal-high bone density and increased density presented in several vertebrae x-rays. An iliac crest biopsy confirmed bone involvement of the underlying disease. The second case was a 57 year old woman who had characteristic signs of systemic mastocytosis with diarrhea, gastritis, flushes and specific cutaneous reactions to exposure to allergens. She also presented severe decrease in bone mineral density in both lumbar spine and femoral neck, with biochemical parameters of increased bone resorption, so had to be treated with bisphosphonates. The third patient, a 67-year-old woman, had several vertebral fractures, with slightly decreased bone densitometry. This last patient showed a hiperplaquetosis with histological diagnosis of essential thrombocythemia, a picture usually associated with systemic mastocytosis. In conclution, we present and discuss three different bone variants of systemic mastocytosis.

Manejo analgésico y anestésico en la mastocitosis sistémica: a propósito de un caso / Analgesic and anesthetic management in systemic mastocytosis: a case report

Las mastocitosis son un grupo heterogéneo de enfermedades que se caracterizan por la proliferación de mastocitos en uno o más órganos o tejidos. Se denomina mastocitosis sistémica (MS) cuando hay afectación de un tejido distinto a la piel. La MS es una enfermedad poco frecuente, cuya incidencia y prevalencia se desconocen. El manejo anestésico de estos pacientes debe considerar que muchos de los fármacos empleados pueden causar una liberación masiva de mediadores químicos mastocitarios. Se presenta el caso de una mujer con MS programada para una histerectomía total, valorando la importancia del correcto estudio preoperatorio así como la técnica anestésica y el tipo de analgesia elegida en este caso. Se presenta nuestro protocolo de actuación de cara a la cirugía en estos pacientes.

Mastocytoses are a heterogeneous group of entities characterized by mast cell proliferation in one or more organs or tissues. When tissues other than the skin are involved, the disease is called systemic mastocytosis (SM). SM is a highly infrequent disease, whose incidence and prevalence are unknown. The anesthetic management of these patients must consider the fact that many drugs can trigger massive release of chemical mediators of mast cells. We report the case of a patient diagnosed with SM who underwent total hysterectomy and discuss the importance of thorough preoperative study, as well as the anesthetic technique and type of analgesia chosen. We also report our protocol for anesthetic management in this disease.