Cutaneous mastocytosis in adult patients with serum tryptase level under 20 ng/ml: why we should investigate further

2021 ◽  
Author(s):  
F. Jendoubi ◽  
J. Shourick ◽  
M. Negretto ◽  
C. Laurent ◽  
P.A. Apoil ◽  
...  
Keyword(s):  
Adult Patients ◽  
Serum Tryptase ◽  
Tryptase Level ◽  
Cutaneous Mastocytosis

Insektengiftallergie: Anaphylaxie besser verstehen

2021 ◽  
Vol 9 (2) ◽  
pp. 80-81
Author(s):  
Dorothea Wieczorek
Keyword(s):  
Angiotensin Converting Enzyme Inhibitors ◽  
Severe Reaction ◽  
Serum Tryptase ◽  
Converting Enzyme Inhibitors ◽  
Specific Symptom ◽  
Baseline Serum ◽  
Tryptase Level ◽  
Symptom Profile ◽  
Organ Systems ◽  
Increased Risk

<b>Background:</b>Venom-induced anaphylaxis (VIA) is a common, potentially life-threatening hypersensitivity reaction associated with (1) a specific symptom profile, (2) specific cofactors, and (3) specific management. Identifying the differences in phenotypes of anaphylaxis is crucial for future management guidelines and development of a personalized medicine approach. <b>Objective:</b>This study aimed to evaluate the phenotype and risk factors of VIA. <b>Methods:</b>Using data from the European Anaphylaxis Registry (12,874 cases), we identified 3,612 patients with VIA and analyzed their cases in comparison with sex- and age-matched anaphylaxis cases triggered by other elicitors (non-VIA cases [n = 3, 605]). <b>Results:</b>VIA more frequently involved more than 3 organ systems and was associated with cardiovascular symptoms. The absence of skin symptoms during anaphylaxis was correlated with baseline serum tryptase level and was associated with an increased risk of a severe reaction. Intramuscular or intravenous epinephrine was administered significantly less often in VIA, in particular, in patients without a history of anaphylaxis. A baseline serum tryptase level within the upper normal range (8–11.5 ng/mL) was more frequently associated with severe anaphylaxis. <b>Conclusion:</b>Using a large cohort of VIA cases, we have validated that patients with intermediate baseline serum tryptase levels (8–11 ng/mL) and without skin involvement have a higher risk of severe VIA. Patients receiving β-blockers or angiotensin-converting enzyme inhibitors had a higher risk of developing severe cardiovascular symptoms (including cardiac arrest) in VIA and non-VIA cases. Patients experiencing VIA received epinephrine less frequently than did cases with non-VIA.

scholarly journals Patient Reported Symptoms and Tryptase Levels in WHO-Defined Systemic Mastocytosis (SM) Versus Mast Cell Activation Syndrome (MCAS) Versus Neither

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3204-3204
Author(s):  
Darci Zblewski ◽  
Ramy A. Abdelrahman ◽  
Dong Chen ◽  
Joseph H. Butterfield ◽  
Ayalew Tefferi ◽  
...  
Keyword(s):  
Mast Cell ◽  
Symptom Score ◽  
Cell Activation ◽  
Total Symptom Score ◽  
Mast Cell Activation ◽  
Median Score ◽  
Serum Tryptase ◽  
Tryptase Level ◽  
Patient Reported ◽  
Symptom Scores

Abstract Introduction: The utility of patient reported symptoms and serum tryptase levels in distinguishing those with systemic mastocytosis (SM) versus mast cell activation syndrome (MCAS) versus those not meeting formal diagnostic criteria for SM or MCAS has not been systematically examined. Methods: This study was approved by our institutional review board. Patients were referred for suspected SM based on symptoms of mast cell activation, osteopenia, skin rash, etc., or had an established diagnosis of SM. All patients were given a Mastocytosis Symptom Assessment Form (MastSAF) to complete at their initial evaluation. The MastSAF is comprised of 36 symptom questions to be graded on a scale of 0 (absent) to 10 (worst imaginable), and is organized around 9 symptom clusters: gastrointestinal (8 questions), constitutional (3 questions), musculoskeletal (5 questions), cutaneous (4 questions), neuropsychological (6 questions), genitourinary (3 questions), respiratory (4 questions), angioedema (1 question), and cardiovascular (2 questions). All patients underwent bone marrow biopsy and serum tryptase level assessment. SM was diagnosed by 2008 WHO criteria. In the presence of characteristic symptoms, if clonal or abnormal mast cells were not identified, and if serum/urine mast cell mediator levels were increased, then non-SM associated, non-monoclonal MCAS was diagnosed. Results: A total 53 patients were studied. 1) SM: Of 28 patients, 13 had indolent SM (ISM), 9 aggressive SM (ASM) and 6 SM with associated hematological disease (SM-AHD) The median total symptom score was 47 (range 8-159). The median (range) for SM subgroups was: ISM 51 (9-159), ASM 55 (19-157) and SM-AHD 27 (8-131) (p=0.2). The normalized median score for individual symptom categories (total median score/no. of symptoms per category) in order of severity was cutaneous 1.9, gastrointestinal 1.8, constitutional 1.7, neuropsychological 1.3, respiratory 1.3, musculoskeletal 0.9, cardiovascular 0.3, genitourinary 0.3, and angioedema 0. Symptom severity was not significantly different among the 3 SM subgroups, except for constitutional symptoms (median score ASM 9, ISM 4, SM-AHD 2.5, p=0.02). The median (range) tryptase level was 48.4 ng/mL (8.8-282); four patients (14%) had a baseline level <20 ng/mL. The median (range) tryptase level among SM subgroups was: ISM 46.9 (8.8-225), ASM 103 (29.4-282), and SM-AHD 43.5 (28.5-233) (p=0.1). When considering patients with tryptase level ≥50 versus <50 ng/mL, symptom scores were not significantly higher in the former group with the exception of constitutional (p=0.02) and genitourinary symptoms (p=0.04). 2) MCAS: 15 patients The median total symptom score was 127 (range 2-248). The normalized median score for individual symptom categories in order of severity was neuropsychological 4.5, musculoskeletal 4.2, cutaneous 4.0, constitutional 3.3, gastrointestinal 2.1, respiratory and cardiovascular 2.0 each, genitourinary 1.7, and angioedema 1.0. The median (range) serum tryptase level at referral was 12.7 ng/mL (1.7-25.8); five patients (33%) had a baseline level >20 ng/mL. 3) Neither SM/MCAS: 10 patients The median total symptom score was 119 (range 45-177). The normalized median score for individual symptom categories in order of severity was musculoskeletal 4.2, gastrointestinal 3.6, constitutional 3.3, neuropsychological 3.3, cutaneous 3.0, cardiovascular and genitourinary 2.0 each, respiratory 1.3, and angioedema 0. The median (range) serum tryptase level at referral (n=9) was 4.8 ng/mL (2.9-6.6). 4) Comparison: MCAS vs. SM: Symptom scores were significantly higher in MCAS as compared to SM (p<0.05), except for genitourinary and respiratory symptoms, which were not significantly different. ‘Neither SM/MCAS’ vs. SM: Symptom scores (total, gastrointestinal, constitutional, musculoskeletal, cutaneous, and neuropsychological) were significantly higher in the former group (p<0.05). Other symptom scores were not significantly different. Conclusions: The spectrum and severity of patient reported symptoms was broadly similar among WHO subcategories of SM, and serum tryptase level had limited if any correlation with symptom scores. Despite the significantly higher overall symptom burden in MCAS versus SM, tryptase levels in the former group were significantly lower with values >30 ng/mL unusual. Despite overlap, the top ranked symptoms in the 3 groups were different. Disclosures No relevant conflicts of interest to declare.

Change In Mast Cell Bone Marrow Burden and In Serum Tryptase Level Are Not Accurate Markers of Response In Patients with Systemic Mastocytosis

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3073-3073
Author(s):  
Alfonso QuintÁs-Cardama, ◽  
Matjaz Sever ◽  
Jorge E. Cortes ◽  
Hagop M. Kantarjian ◽  
Srdan Verstovsek
Keyword(s):  
Bone Marrow ◽  
Mast Cell ◽  
Mast Cells ◽  
Systemic Mastocytosis ◽  
Median Number ◽  
Response To Therapy ◽  
Serum Tryptase ◽  
Bone Marrow Biopsies ◽  
Tryptase Level ◽  
Bone Marrow Mast Cell

Abstract Abstract 3073 Background: Bone marrow involvement, with or without cutaneous or visceral involvement, is almost universal in patients with systemic mastocytosis (SM). The KITD816V mutation is present in most patients with SM, thus confirming its clonal nature. Patients with ASM are usually managed with cytoreductive agents such as hydroxyurea (HU), cladribine (2CDA), or interferon-alpha (IFN-α), although the activity of these therapies is limited as they do not target specifically the malignant clone. Response assessment in SM relies on symptom improvement and reduction in serum tryptase levels and visceral and/or bone marrow mast cell burden (percent mast cell involvement). We contend that the later two relatively objective metrics may not be appropriate markers of response because serum tryptase levels may vary significantly at different time-points in the same patient in the absence of intervention, do not correlate accurately with mast cell burden, and bone marrow mast cell burden determination is subject to sampling bias given the patchy infiltration observed in many cases of SM. Objectives: To assess the utility of bone marrow mast cell burden reduction and serum tryptase level reduction as criteria for response in patients with SM. Patients and Therapy: We studied a cohort of 50 patients with SM for whom at least 2 sequential bone marrow biopsies and 2 serum tryptase level determinations were available at our center. The KITD816V mutation was present in 20 (59%) of 34 assessable patients. No patient carried the JAK2V617F mutation or the FIP1L1-PDGFRA rearrangement. Patients had a diagnosis of indolent SM (ISM, n=25), aggressive SM (ASM, n=16), or SM-AHNMD (n=9). All but 1 patient received SM-directed therapy (median number of therapies 2, range 1–5), including: imatinib (n=16), dasatinib (n=23), RAD001 (n=8), denileukin diftitox (n=7). The median number of bone marrow biopsies available per patient was 4 (range, 2–14) and the median number of tryptase measurements was 6 (range, 2–18), which were obtained both on and off SM-directed therapies. Results: Four patients had a bone marrow complete response: 1 with imatinib, 2 with dasatinib, and 1 with decitabine (with SM-MDS). However none of the responders normalized their tryptase levels. We used the coefficient of variation (CV) as a normalized measure of dispersion of a probability distribution for the percentage of mast cells in bone marrow biopsies and serum tryptase levels. In this manner, the CV summarizes/describes the variation in tryptase levels and bone marrow mast cell percentage from the baseline (first recorded value) in the patients evaluated. We found that among the 49 treated patients, the percentage of bone marrow mast cells varied significantly with a CV ranging from 6 – 173% and an average of 65%. Forty-four percent of patients had a CV equal or higher to the average. Similar results were observed regarding tryptase levels, with an average CV of 19% that ranged from 0 to 96%. Thirty-six percent of patients had a CV higher than average. Conclusion: While most patients fail to respond to currently available SM-directed therapies, sequential bone marrow biopsies and tryptase level determinations exhibit remarkable variation both during and in the absence of SM-directed therapy. Therefore, it seems that single time point measurements of these values do not represent proper tools to assess accurately response to therapy. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Baseline Tryptase Levels Correlate with Baseline Basophil Levels in Chronic Myeloid Leukemia-Chronic Phase

2020 ◽  
pp. 171-176
Author(s):  
Anisha Mathew ◽  
Manisha Naithani ◽  
Sarama Saha ◽  
Rituparna Chetia ◽  
Uttam Kumar Nath
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Peripheral Blood ◽  
Myeloid Leukemia ◽  
Surrogate Marker ◽  
Chronic Phase ◽  
Newly Diagnosed ◽  
Serum Tryptase ◽  
Baseline Serum ◽  
Tryptase Level ◽  
Blood Basophil

Aims: To study whether there is any correlation between baseline blood basophil count and serum tryptase levels in newly diagnosed chronic phase chronic myeloid leukemia (CML-CP) patients. Settings and Design: 40 newly diagnosed CML-CP patients were enrolled from Medical Oncology Hematology OPD based on their baseline BCR-ABL status (done in department of Biochemistry). Methods and Materials: Serum tryptase level was measured using Sandwich ELISA and peripheral blood basophil count was estimated using automated cell counter & peripheral blood film examination. BCR-ABL quantification was done using real time PCR after conversion of RNA (extracted from whole blood) to cDNA. Statistical Analysis Used: SPSS Version 23. Results: Baseline peripheral blood basophil levels showed a significant correlation with baseline serum tryptase levels (p<0.01) and tryptase level also correlated with EUTOS score, which has basophil count as one of the parameters. This may signify that serum tryptase levels can be a surrogate marker of the basophil compartment in CML-CP. Conclusions: Based on findings of the present study and other studies available in literature, serum tryptase can be utilised as a surrogate marker of the basophil compartment in CML-CP.

scholarly journals Usefulness of serum tryptase level as an independent biomarker for coronary plaque instability in a Chinese population

2011 ◽  
Vol 215 (2) ◽  
pp. 494-499 ◽  
Author(s):  
Meixiang Xiang ◽  
Jiusong Sun ◽  
Yan Lin ◽  
Jie Zhang ◽  
Han Chen ◽  
...  
Keyword(s):  
Chinese Population ◽  
Coronary Plaque ◽  
Serum Tryptase ◽  
Plaque Instability ◽  
Tryptase Level

Serum tryptase level and inflammatory markers in exhaled breath condensate of children with exercise-induced symptoms

2016 ◽  
Vol 37 (5) ◽  
pp. 84-92 ◽  
Author(s):  
Iwona Stelmach ◽  
Magdalena Zaczeniuk ◽  
Anna Sztafińska ◽  
Paweł Majak ◽  
Joanna Jerzyńska ◽  
...  
Keyword(s):  
Inflammatory Markers ◽  
Exhaled Breath Condensate ◽  
Exhaled Breath ◽  
Serum Tryptase ◽  
Tryptase Level ◽  
Exercise Induced ◽  
Breath Condensate
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