H Pilorget1, A Bangui, M Adam, G Leverger. 1. Service de néonatologie et réanimation néonatale et infantile, CHD Félix-Guyon, Saint-Denis, La Réunion.
Abstract
BACKGROUND: Primary myelofibrosis is rare in infants and children; its association with auto-immune markers has only been reported in adults. CASE REPORT: An 8 month-old girl was admitted because of severe anemia and neutropenia. The marrow aspirate showed dysgranulopoiesis and partial interruption of maturation after the myelocyte level. The bone marrow biopsy revealed reticulinic myelofibrosis. The condition worsened with development of agranulocytosis and thrombocytopenia. Investigations ruled out malignant hemopathy, metastatic infiltration of the marrow and osteopathy. A myelodysplastic syndrome was discussed, but presence of anti-granulocyte auto-antibodies and positive Coombs test led to consider an autoimmune etiology. A corticosteroid therapy was attempted, effective only on the platelet lineage. Addition of intravenous gammaglobulin therapy corrected the problem. After a 24 month-course of the disease, it was necessary to prolong therapy. CONCLUSION: The efficacy of gammaglobulins may be an additional argument for auto-immunity, although no other auto-immune pattern has been observed in our patient, contrary to reported cases in adults.
BACKGROUND:Primary myelofibrosis is rare in infants and children; its association with auto-immune markers has only been reported in adults. CASE REPORT: An 8 month-old girl was admitted because of severe anemia and neutropenia. The marrow aspirate showed dysgranulopoiesis and partial interruption of maturation after the myelocyte level. The bone marrow biopsy revealed reticulinic myelofibrosis. The condition worsened with development of agranulocytosis and thrombocytopenia. Investigations ruled out malignant hemopathy, metastatic infiltration of the marrow and osteopathy. A myelodysplastic syndrome was discussed, but presence of anti-granulocyte auto-antibodies and positive Coombs test led to consider an autoimmune etiology. A corticosteroid therapy was attempted, effective only on the platelet lineage. Addition of intravenous gammaglobulin therapy corrected the problem. After a 24 month-course of the disease, it was necessary to prolong therapy. CONCLUSION: The efficacy of gammaglobulins may be an additional argument for auto-immunity, although no other auto-immune pattern has been observed in our patient, contrary to reported cases in adults.