Bone marrow findings in adult patients with urticaria pigmentosa

Czarnetzki, Beate M.; Kolde, G.; Schoemann, Annemarie; Urbanitz, Schwantje; Urbanitz, D.

doi:10.1016/s0190-9622(88)70007-9

Cited by 54 publications

(37 citation statements)

References 22 publications

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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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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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“…[1][2][3][4] Unlike pediatric cases, most adults with UP-like skin lesions have systemic disease (ie, systemic mastocytosis [SM]) at presentation, a condition generally confirmed by a bone marrow (BM) biopsy. 5 MCs are derived from CD34 ϩ /KIT ϩ pluripotent hematopoietic cells in the bone marrow 6 ; its neoplastic counterparts are morphologically atypical (spindled shape, hypogranular cytoplasm, nuclear atypia), 7,8 and express abnormal cell surface markers (CD25 and/or CD2). 9,10 Most, if not all, adult mastocytosis patients carry gain-of-function KIT receptor mutations, most commonly D816V in the tyrosine kinase domain.…”

Section: Introductionmentioning

confidence: 99%

Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors

Lim

Tefferi

Lasho

et al. 2009

Blood

498

572

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“…9,10 The overall incidence of bone marrow infiltration is unclear, because a range from 10% to 70% was reported, [8][9][10] and no agreement has been reached as to its prognostic significance.…”

Section: Discussionmentioning

confidence: 99%

Urticaria Pigmentosa:A Clinical, Hematopathologic, and Serologic Study of 30 Adults

et al. 1998

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Bone marrow findings in adult patients with urticaria pigmentosa

Cited by 54 publications

References 22 publications

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

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

Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors

Urticaria Pigmentosa:A Clinical, Hematopathologic, and Serologic Study of 30 Adults

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