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.

Changes of Serum IgE and Tryptase in Anaphylactic Shock Rats / 法医学杂志

OJECTIVE@#To explore the changes of serum IgE and tryptase caused by anaphylactic shock rats and discuss the relation to PMI and preservative environment of corpse and specimen.@*METHODS@#Rats were used for establishing anaphylactic shock models and randomly divided into room temperature group, refrigeration group, frozen group, manual hemolysis group, specimen preservation group. And the control group was also established. The blood samples were collected after rats were sacrificed. The degree of hemolysis was graded according to the color of the upper layer of the serum. The mass concentration of IgE and tryptase in each group was detected by ELISA.@*RESULTS@#The levels of serum IgE and tryptase in anaphylactic shock dead rats were higher than that of the control group. Room temperature and frozen made obviously differences on the levels of serum IgE and tryptase with various PMI. The levels of serum IgE and tryptase in refrigeration group showed relatively stable. The levels of serum tryptase and IgE were elevated with differently increasing hemolysis. The levels of serum IgE and tryptase showed no obvious changes during the specimen kept under different temperature conditions for 25 days.@*CONCLUSION@#Serum IgE and tryptase obviously increased in anaphylactic shock rats. However, the levels were influenced by PMI and environmental temperature, especially under the conditions of room temperature and frozen.

The improvement of mixed human serum-induced anaphylactic reaction death model in guinea pigs / 法医学杂志

OBJECTIVE@#To increase the death rate of fatal anaphylaxis in guinea pigs and the detectahie level of the tryptase of mast cell in hlood serum.@*METHODS@#Seventy-four guinea pigs were randomly divided into five groups: original model group, original model control group, improved model group, improved model control group, improved model with non-anaphylaxis group. Using mixed human serum as the allergen, the way of injection, sensitization and induction were improved. ELISA was used to detect the serum mast cell tryptase and total IgE in guinea pigs of each group.@*RESULTS@#The death rate of fatal anaphylaxis in original model group was 54.2% with the different degree of hemopericardium. The severe pericardial tamponade appeared in 9 guinea pigs in original model group and original model control group. The death rate of fatal anaphylaxis in improved model group was 75% without pericardial tamponade. The concentration of the serum total IgE showed no statistically difference hetween original model group and original model control group (P > 0.05), hut the serum mast cell tryptase level was higher in the original model group than that in the original model control group (P > 0.05). The concentration of the serum total IgE and the serum mast cell tryptase level were significantly higher in improved model group than that in the improved model control group (P < 0.05).@*CONCLUSION@#The death rate of the improved model significantly increases, which can provide effective animal model for the study of serum total IgE and mast cell tryptase.

The utility of the World Health Organization-The Uppsala Monitoring Centre (WHO-UMC) system for the assessment of adverse drug reactions in hospitalized children.

Although the World Health Organization-The Uppsala Monitoring Centre (WHO-UMC) system has been suggested as a practical tool for classifying adverse drug reactions (ADRs), verification of such system has not been examined. The objective of this study was to evaluate the usefulness of the WHO-UMC classification for the diagnosis of ADRs. The gold standard was the results of drug challenges and serum tryptase in cases of anaphylaxis. Twenty-seven children had ADRs classified by the WHO-UMC system. The causality terms were 'certain' in 4/27, 'probable' in 6/27, 'possible' in 10/27 and 'unlikely' in 7/27 of the patients. Skin prick tests and intradermal tests were positive in 1/20 and 1/5 of the patients, respectively. Drug challenges and serum tryptase were positive in 8/26 and 1/3 of the patients, respectively. After complete evaluation, the positive and negative ADRs were documented in 9/27 patients (33.33%) and 18/27 patients (66.67%), respectively. The multi-level likelihood ratios for ADRs using the WHO-UMC system were infinity in causality term 'certain', 2 in 'probable', 0.5 in 'possible', and 0 in 'unlikely'. In conclusion, causality term 'certain' and 'unlikely' of the WHO-UMC system had large impact on the likelihood of ADRs. In contrast, the causality term 'probable' and 'possible' had small impact on the likelihood of ADRs. Drug challenges and serum tryptase were helpful to confirm ADRs categorized by WHO-UMC system.

Anaphylaxis.

Anaphylaxis must always be considered a medical emergency. While classic anaphylaxis needs specific antigen to trigger IgE antibody-mediated reaction, idiopathic anaphylaxis spontaneously occurs with no external allergen. Anaphylactoid are not mediated by antigen-antibody but result from substances acting directly on mast cells and basophils. Incidence of anaphylaxis is 21 per 100,000 person-years with fatality in about 0.65% of cases. Food is the most frequent cause of anaphylaxis in children while insect sting is the most common cause in adults. Epinephrine is the first pharmacological treatment. Secondary measures include circulatory support, H1 and H2 antagonists, bronchodilators if necessary and probably corticosteroids. Since life-threatening manifestations may recur during the recurrent phase, it may be necessary to observe the patients for up to 48 hours after apparent recovery from an anaphylactic episode.