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Systemic mastocytosis in a 5-year-old child: successful treatment with disodium cromoglycate
Insights
Systemic mastocytosis (SM) in a child improved with disodium cromoglycate (DSCG). DSCG treatment reduced symptoms and histamine levels, with recurrence during placebo periods, demonstrating its efficacy in managing SM.
Area of Science:
- Pediatric Allergy and Immunology
- Hematology
- Dermatology
Background:
- Systemic mastocytosis (SM) is a rare disorder characterized by abnormal mast cell accumulation.
- Clinical manifestations are often linked to mast cell degranulation and histamine release.
Observation:
- A 5-year-old male child presented with disseminated skin lesions and systemic symptoms including vomiting, diarrhea, flushing, tachycardia, hypotension, somnolence, and transient blindness since 4 months of age.
- Symptoms were triggered by heat and egg ingestion.
Findings:
- A single-blind trial using oral disodium cromoglycate (DSCG) at 100 mg/kg/day demonstrated significant improvement in all systemic manifestations of SM.
- Histaminaemia levels decreased during DSCG treatment.
- Symptoms, clinical signs, and histaminaemia recurred during placebo periods, highlighting the drug's effectiveness.
Implications:
- Disodium cromoglycate (DSCG) shows therapeutic potential in managing severe systemic mastocytosis in pediatric patients.
- This study underscores the role of histamine release in SM symptomatology.
- Further research into DSCG for pediatric SM is warranted.
Abstract:
Most clinical signs and symptoms of systemic mastocytosis (SM) are attributed to histamine release. We report here a 5-year-old male child with SM, who suffered from the age of 4 months from disseminated skin lesions, vomiting, diarrhoea, abdominal pain, flushing, tachycardia, hypotension, somnolence, and transient blindness, triggered by heat and egg ingestion. Oral disodium cromoglycate (DSCG) or placebo were started in a single blind trial at a dose of 100 mg/kg/day in four divided doses. The child was studied for 21 months during the administration of three courses of DSCG, each of 6 months' duration, interspersed with three 1-month courses of placebo. During treatment with DSCG all the systemic manifestations improved, and the histaminaemia decreased. During the placebo periods the symptoms, signs, and histaminaemia recurred.