Cromolyn sodium in the management of systemic mastocytosis

Horan, Richard F.; Sheffer, Albert L.; Austen, K. Frank

doi:10.1016/0091-6749(90)90067-e

Cited by 151 publications

(79 citation statements)

References 7 publications

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“…32 Leukotriene antagonists can be useful as adjunctive therapy in patients with suboptimal response to first-line antimediator drugs and may be steroid sparing in this role. 33 Flushing can be severe and subjectively distressing and unresponsive to antihistamines.…”

Section: 29mentioning

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: 29mentioning

confidence: 99%

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

Pardanani

2013

Blood

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“…Cromolyn sodium given orally has been effective at improving the mixed organic brain syndrome [34], indicating that local intestinal mast cell stabilization could contribute to preventing the release of mediators affecting the CNS.…”

Section: Neuropsychiatric Symptomsmentioning

confidence: 99%

Mastocytosis: Mediator-Related Signs and Symptoms

Castells

Austen²

2002

Int Arch Allergy Immunol

115

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Patients with systemic mastocytosis present symptoms related to the tissue response to the release of mediators from mast cells and to the local mast cell burden. Such patients often have a history of chronic and acute mediator-related symptoms. Most patients have indolent disease with a good prognosis and a normal life span. Symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric symptoms, most of which are controlled by medication. Because there is no current cure for mastocytosis, successful therapeutic interventions rely on the recognition of mediator-related symptoms and their treatment, and established intervention approaches for the relatively uncommon leukemic concomitants. Efforts to link a particular mast cell-derived mediator to some aspect of the symptom complex depend on the known actions of the mediator and the efficacy of target-based interventions.

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“…El cromoglicato disódico es el tratamiento más eficaz para los cólicos abdominales de repetición con o sin diarrea 36,37 . Las dosis para adultos es de 200 mg por vía oral, 4 veces al día, media hora antes de las principales comidas y al acostarse; para los niños se empleará una dosis diaria de 10 a 20 mg por Kg de peso.…”

Section: Tratamiento De Los Síntomas Gastrointestinalesunclassified