The bone marrow in urticaria pigmentosa and systemic mastocytosis. Cell composition and mast cell density in relation to urinary excretion of tele-methylimidazoleacetic acid

Ridell, B.

doi:10.1001/archderm.122.4.422

Cited by 25 publications

(10 citation statements)

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“…Our findings indicate a high rate of multi-organ involvement in UP, at least in adult patients, and confirm the need for systemic evaluation of all patients with extensive UP lesions [17, 18]. On the other hand, gastro-intestinal and other visceral symptoms in patients with UP may not always be the result of extracutaneous mastocytosis, since symptoms were unrelated to the underlying disease in some cases.…”

Section: Discussionsupporting

confidence: 68%

Cutaneous Mastocytosis in Adults

et al. 1998

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Background and Objective: Systemic mastocytosis is a rather rare disorder involving the skin and several other organs. The aim of this study was to analyse the extent of extracutaneous manifestations in 14 adult patients who presented with prominent cutaneous involvement within the last 5 years. Results: The cutaneous lesions were clinically diagnosed as telangiectasia macularis eruptiva perstans in 2 patients, urticaria pigmentosa of varying extent in 11 and diffuse erythrodermic mastocytosis in 1 patient. All patients had extracutaneous manifestations with involvement of one additional organ system in 6/14 cases, two in 5/14 and three in 3/14. Ten out of 14 patients suffered from generalized pruritus, and 11/14 reported mild wheal formation, while 3/14 with multi-organ involvement mentioned recurrent flushing episodes. The gastro-intestinal tract was involved in 8/14 cases with an increase in gastric and colon mucosal mast cells in 5/8 cases and gastroduodenitis in 2. Bone marrow involvement was seen in 7/13 patients, hepatosplenomegaly in 2, anaemia in 2 and thrombocytopenia in 3. The disease had a duration of 0.5–32 years, clinical symptoms remaining basically unchanged. Malignant transformation was not seen; only 1 patient developed myelodysplastic syndrome within 2 years after the first cutaneous lesions. Conclusions: Our study shows that extracutaneous involvement should be carefully considered in adult patients with cutaneous mastocytosis. Systemic multi-organ mast cell disease in adults is a long-lasting disorder with recurrent episodes of varying clinical symptomatology. However, the disease shows rather slow progression, and malignant transformation is rare. Satisfactory management is achieved by symptomatic oral drug intake.

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Section: Discussionsupporting

confidence: 68%

Cutaneous Mastocytosis in Adults

et al. 1998

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“…Recently we have shown that increased excretion of MelmAA corresponds well with mast cell infiltra tion of the bone marrow [2]. Our tentative conclusion was that a value of MelmAA in the urine above 4.1 mg/24 h would indicate systemic involvement.…”

Section: Discussionmentioning

confidence: 60%

“…The diagnosis systemic mastocytosis was settled by the finding of mast cells in the bone marrow [1,2]. The diagnosis of the skin eruption was based on the usual clinical criteria for UP, i.e.…”

Section: Methodsmentioning

confidence: 99%

“…Urticaria pigmen tosa (UP) is the pure cutaneous form of the disease, while in systemic mastocytosis extracutaneous in volvement also has taken place. In systemic mastocy tosis, involvement of the bone marrow is the most common finding [1][2][3]. Other organs in which mast cell infiltration also can be found are the skeleton, liver, spleen and the gastrointestinal tract.…”

Section: Introductionmentioning

confidence: 99%

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Long-Term Follow-Up of Histamine Turnover in Mastocytosis

Roupe

Granérus

1987

Int Arch Allergy Immunol

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To study the long-term histamine turnover in patients with mastocytosis, the urinary excretion of the main histamine metabolite tele-methylimidazoleacetic acid was measured. Eighteen patients, 10 with urticaria pigmentosa and 8 with systemic mastocytosis, were followed for several years (mean 4.2 years). Younger individuals (<30 years) had initially normal excretion or tended to normalize their excretion during follow-up, while older patients (>30 years) often had systemic manifestations and higher excretion values, which increased in some cases during the observation period.

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“…9,10 Allowing for differences based on referral patterns (dermatology vs allergy or hematology clinics), most patients with adult-onset MIS have demonstrable bone marrow (BM) involvement with clonal mast cells when modern-era diagnostic tools are used, in most instances, satisfying WHO diagnostic criteria for SM (Table 2). 1,11 While historical series of patients with MIS revealed an 18% to 50% prevalence of systemic involvement based on conventional histologic criteria, [12][13][14][15] more modern series suggest that only a minority of adult patients have skin-limited disease. 10,16 Further, approximately 50% of adults with apparent skin-limited mastocytosis may have a clonal BM mast cell infiltrate that falls short of the diagnostic threshold for SM (satisfies major criterion only or only 1 or 2 minor criteria), suggesting prediagnostic or early stage of ISM.…”

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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The bone marrow in urticaria pigmentosa and systemic mastocytosis. Cell composition and mast cell density in relation to urinary excretion of tele-methylimidazoleacetic acid

Cited by 25 publications

References 0 publications

Cutaneous Mastocytosis in Adults

Cutaneous Mastocytosis in Adults

Long-Term Follow-Up of Histamine Turnover in Mastocytosis

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

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