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[Systemic infantile mastocytosis: about a case with respiratory and digestive involvement]
1Service de pédiatrie, CHU Sahloul, 4000 Sousse, Tunisie. jalel_chemli@yahoo.fr
Insights
Systemic mastocytosis in children is rare, with this case showing unusual respiratory and digestive symptoms. Treatment with histamine antagonists improved symptoms, highlighting a rare presentation of this condition.
Area of Science:
- Pediatric Hematology
- Gastroenterology
- Immunology
Background:
- Systemic mastocytosis (SM) is a rare disorder characterized by mast cell proliferation.
- Pediatric SM is uncommon, often presenting with diverse symptoms.
Observation:
- A neonate presented with cutaneous symptoms of SM.
- The child later developed recurrent respiratory infections and gastrointestinal issues, including hepatosplenomegaly and esophageal infiltration.
Findings:
- Diagnosis was confirmed by histology and elevated histamine levels.
- Histamine H1 and H2 receptor antagonists led to symptom improvement.
Implications:
- This case highlights rare respiratory and digestive manifestations of pediatric SM.
- Early diagnosis and management are crucial for improving outcomes and preventing complications.
Unlabelled:
Systemic mastocytosis is rare in children and is characterized by an abnormal proliferation and infiltration of mast cells in different tissues.
Case Report:
We report a case of systemic mastocytosis presenting cutaneous symptoms during the neonatal period. Later evolution was characterized by systemic manifestations consisting of recurrent respiratory infections with wheezing and a digestive involvement that included abdominal pain, hepatosplenomegaly and a nodular, hemorrhagic infiltrate in a low esophagus. The diagnosis was confirmed by histology and biology, notably increased histamine concentrations in blood and urines. Improvement of the respiratory and digestive symptoms was obtained with treatment by histamine H1 and H2 receptors antagonists.
Conclusion:
Respiratory manifestations and nodular infiltration of the digestive tract are rare in systemic mastocytosis. The prognosis is conditioned by complications such as malignancy and the persistence of the disease till the adult age.