Treatment of bullous mastocytosis with disodium cromoglycate

Welch, Elizabeth A.; Alper, Joseph C.; Bogaars, Hendrick; Farrell, David S.

doi:10.1016/s0190-9622(83)70140-4

Cited by 36 publications

(8 citation statements)

References 7 publications

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“…Oral sodium cromolyn has been successfully used in the control of several mast cell-mediator related symptoms such as abdominal pain, diarrhea [29,52,[147][148][149][150][151][152][153][154][155], mixed organic brain syndrome [49], to restore cognitive abilities [14], and in a lesser extent in other manifestation such as pruritus and flushing. Furthermore, improvement of bone pain has been described in a case of mastocytosis with bone sclerosis [156].…”

Section: / Sodium Cromolynmentioning

confidence: 99%

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Escribano¹,

Akin

Castells

et al. 2006

IADT

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Patients with mastocytosis have symptoms related to the tissue response to the release of mediators from mast cells (MC), local mast cell burden or associated non-mast cell hematological disorders. MC contain an array of biologically active mediators in their granules, which are preformed and stored. MC are also able to produce newly generated membrane-derived lipid mediators and are a source of multifunctional cytokines. Mediator-related symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric disturbances; these symptoms are variably controlled by adequate medications. Management of patients within all categories of mastocytosis includes: a) a careful counseling of patients (parents in pediatric cases) and care providers, b) avoidance of factors triggering acute mediator release, c) treatment of acute and chronic MC-mediator symptoms and, if indicated, d) an attempt for cytoreduction and treatment of organ infiltration by mast cells.

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Section: / Sodium Cromolynmentioning

confidence: 99%

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Escribano¹,

Akin

Castells

et al. 2006

IADT

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“…59 Disodium cromoglycate has also been reported to relieve musculoskeletal pain, headache, central nervous system complaints, and skin symptoms in some patients. [60][61][62][63][64] Mastocytosis with bullous lesions in infancy sometimes presents with a shock like syndrome. 65 As shown in one case study where the patient died of massive hypotension secondary to mast cell degranulation, 65 it is prudent to administer adrenaline and other therapy as indicated including fluids, glucocorticoids, and H 1 and H 2 antihistamines.…”

Section: Diagnosismentioning

confidence: 99%

Paediatric mastocytosis

Carter

2002

Archives of Disease in Childhood

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“…In this study, 8 patients with SM were randomly assigned to receive either DSCG + placebo or antihistamines + placebo, with crossover at the end of week 10, and only 6 patients completed the study; although the differences between the two treatments were not statistically significant, the authors noted that most patients had less pruritus and less urticaria while receiving antihistamines, while most patients had improvement of the gastrointestinal symptoms and one patient experienced dramatic relief from bone pain while receiving DSCG [41]. In addition, case reports and small series of patients have stated benefits in using oral and topical DSCG for the treatment of diffuse cutaneous/bullous mastocytosis [42–44]. Moreover, oral and inhaled DSCG have been used in the management of other symptoms associated with SM, such as of bone pain, headache and fatigue, and hypotensive crisis [45, 46].…”

Section: Discussionmentioning

confidence: 99%

Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome

Conde-Fernandes¹,

Sampaio²,

Moreno³

et al. 2017

Allergy Asthma Clin Immunol

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BackgroundMastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(−), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes. The V560G KIT mutation is extremely rare in patients with SM and its biological and prognostic impact remains unknown.Case presentationA 15-year old boy was referred to our hospital because of repeated episodes of flushing, hypotension and syncope since the age of 3-years, preceded by skin lesions compatible with mastocytosis on histopathology that had disappeared in the late-early childhood. Diagnosis of ISM, more precisely the ISMs(−) variant, was confirmed based on the clinical manifestations together with increased baseline serum tryptase levels and the presence of morphologically atypical, mature appearing (CD117+high, FcεRI+) phenotypically aberrant (CD2+, CD25+) MCs, expressing activation-associated markers (CD63, CD69), in the bone marrow. Molecular genetic studies revealed the presence of the KIT V560G mutation in bone marrow MCs, but not in other bone marrow cells, whereas the screening for mutations in codon 816 of KIT was negative. The patient was treated with oral disodium cromoglycate and the disease had a favorable outcome after an eleven-year follow-up period, during which progressively lower serum tryptase levels together with the fully disappearance of all clinical manifestations was observed.ConclusionsTo the best of our knowledge this first report of a patient with ISM, whose bone marrow MCs carry the KIT V560G activating mutation, manifesting as recurrent spontaneous episodes of flushing and vascular collapse in the absence of skin lesions at the time of diagnosis, in whom disodium cromoglycate had led to long term clinical remission.

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Treatment of bullous mastocytosis with disodium cromoglycate

Cited by 36 publications

References 7 publications

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Paediatric mastocytosis

Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome

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