Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis

Heide, Rogier; Doorn, Kevin van; Mulder, Paul; Toorenenbergen, A. W. Van; Beishuizen, Auke; Groot, Hans de; Tank, Bhupendra; Oranje, Arnold P.

doi:10.1111/j.1365-2230.2008.03005.x

Cited by 53 publications

(58 citation statements)

References 18 publications

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“…24 The clinical severity of MIS can be assessed using the SCORMA (SCORing Mastocytosis) system; patients are scored across 3 fields including the extent of MIS, severity of representative cutaneous lesions, and symptom severity. 27,28 Other criteria for grading MIS and cutaneous and systemic symptoms related to mast cell mediator release have also been proposed.…”

Section: Does the Patient Have Extracutaneous Or Systemic Disease?mentioning

confidence: 99%

How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

Pardanani

2013

Blood

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Indolent systemic mastocytosis (SM) patients have a varied clinical presentation, ranging from predominantly cutaneous symptoms to recurrent systemic symptoms (eg, flushing, palpitations, dyspepsia, diarrhea, bone pain) that can be severe and potentially life threatening (anaphylaxis). Mastocytosis patients without skin involvement pose a diagnostic challenge; a high index of suspicion is needed in those with mast cell–degranulation symptoms, including anaphylaxis following Hymenoptera stings or other triggers. Modern-era molecular and flow-cytometric diagnostic methods are very sensitive and can detect minimal involvement of bone marrow with atypical/clonal mast cells; in some cases, full diagnostic criteria for SM are not fulfilled. An important aspect of treatment is avoidance of known symptom triggers; other treatment principles include a stepwise escalation of antimediator therapies and consideration of cytoreductive therapies for those with treatment-refractory symptoms. The perioperative management of mastocytosis patients is nontrivial; a multidisciplinary preoperative assessment, adequate premedications, and close intra- and postoperative monitoring are critical. Smoldering mastocytosis is a variant with high systemic mast cell burden. While its clinical course can be variable, there is greater potential need for cytoreductive therapies (eg, interferon-alpha, cladribine) in this setting. A systematic approach to the diagnosis and treatment of indolent SM using a case-based approach of representative clinical scenarios is presented here.

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Section: Does the Patient Have Extracutaneous Or Systemic Disease?mentioning

confidence: 99%

How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

Pardanani

2013

Blood

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“…4 Tryptase levels were also found to correlate with the scoring index of mastocytosis (SCORMA) ratings in mastocytosis patients. 5 SCORMA is based on a semi-quantitative analysis of the extent, intensity, and subjective complaints, and each item is scored from 0 to 3, where 0 is for an absent item and 3 represents the highest severity. 6 Five subjective symptoms-provocative factors, flushing, diarrhea, pruritus, and local bone pain-are scored by patients from 0 to 10 using a visual analogue scale.…”

Section: Research Studies Related To Histamine and Tryptasementioning

confidence: 99%

Pediatric Mastocytosis: A Review of the Literature

Frieri

Quershi

2013

Pediatric Allergy, Immunology, and Pulmonology

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Mastocytosis has a bimodal distribution often presenting in children from birth to 2 years of age and in those over the age of 15. Pediatric mastocytosis is due to the effects of mast-cell degranulation enzymes such as histamine and tryptase causing the presentation of pruritis, flushing, vesicles, abdominal and bone pain, or headache. Three different forms of mastocytosis can occur: urticaria pigmentosa, diffuse cutaneous, and solitary mastocytoma. Systemic symptoms are typically a result of mast-cell mediator release but do not prove systemic mast-cell hyperplasia. In this review, we present several research studies related to pediatric mast-cell disorders, and discuss several cases of pediatric mastocytosis, acute myeloid leukemia, pathophysiology, genetic studies, and treatment. Introduction and Research Studies

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“…The majority of the cases have no complaints throughout their whole lives or complaints of mild symptoms at times of attacks (8). In recent years, increased serum triptase has been found to be effective on the severity of the complaints (20). In our patients, the second prominent complaint following eruption was itching.…”

Section: Factors Triggering Symptomsmentioning

confidence: 74%