Glanzmann thrombasthenia is a rare hereditary thrombopathy, mainly responsible for spontaneous mucocutaneous hemorrhages. It is due to a qualitative or quantitative deficiency of the platelet receptor GPIIbIIIa, involved in platelet aggregation. This disease is mostly observed in populations with high consanguinity. The aim of our work is to study the epidemiological, clinical, biological, therapeutic and evolutionary particularities of Glanzmann thrombasthenia in children. A total of 11 patients were diagnosed and followed up in the pediatric hematology-oncology department of the Hassan II University Hospital of Fez, Morocco. The average age at diagnosis was 2 years and 6 months, 81% of the cases were from consanguineous parents, mucocutaneous hemorrhages were the most reported, and the diagnosis of Glanzmann thrombasthenia was confirmed in all cases by aggregometry. Treatment is based mainly on local symptomatic measures and platelet transfusion, activated factor VII is reserved in case of anti-platelet immunization. The evolution is good in all cases, only one death is reported.
Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare idiopathic disorder with unknown etiology. The présentation of RDD is painless bilateral cervical lymphadenopathy. Extranodale RDD without nodale involvement is very rare. Isolated form to the bone occurs in only 2% of patients. We report a rare case of RDD revealed by left radius involvement in an infant aged 21 months with lung involvement in the assessment of extension, treated with chemotherapy with good outcome. Bone involvement is rare, hence the interest in knowing the different etiological diagnoses in order to make the rapid diagnosis and start the appropriate treatment.
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