Fisher – Evans syndrome: rare cause of cytopenias in children

Abstract

Interested in the problem of cytopenic conditions in children is associated with a various causes of them. The spectrum of differential diagnostic search is very wide: hemoblastoses, myelodysplastic and paraneoplastic syndromes, aplastic anemia. It is necessary for assessment of the main cause of cytopenia to pay attention on the patient’ common status (his complaints, anamnestic data — features of the start of the disease, previous diseases, concomitant somatic pathology, constant medication). Also on the complex and features of the clinical manifestations, the functional state of organs and systems, and in particular the liver, hematological changes in peripheral blood and bone the brain. The combination of immune thrombocytopenic purpura with autoimmune hemolytic anemia are named Fisher — Evans syndrome (FES). The article presents a clinical case of the combination Fisher — Evans syndrome and preceding B-lymphoma in a preschool child, and also reflects the main etiopathogenetic mechanisms of development this pathology. Fisher — Evans syndrome (SFE) is a rare autoimmune disease characterized by the immune thrombocytopenia (ITP) and Coombs–positive autoimmune hemolytic anemia (AIGA), which can develop simultaneously or sequentially, and in some cases are combined with immune neutropenia. The primary and secondary variants of Fisher — Evans syndrome are described. The secondary is the background of the following pathology: Epstein — Barr viral, cytomegalovirus, immunodeficiency virus, hepatitis C, parvovirus, varicella zoster virus, mycoplasma infection, tuberculosis), immunodeficiency and lymphoproliferative conditions, rheumatoid, autoimmune and malignant diseases such as systemic lupus erythematosus (SLE), antiphospholipid syndrome (AFLS), Sjogren’s syndrome, immunoglobulin A deficiency (IgA), autoimmune lymphoproliferative syndrome (ALPS), general variable immune deficiency (OVIN), lymphogranulomatosis (LGM), chronic lymphoblastic leukemia (CLL), autoimmune thyroiditis, autoimmune hepatitis, Takayasu arteritis, systemic sclerosis. The difficulties of searching causes are and indicated this diagnosis of exclusion. The question remains: is B-lymphoma the cause or accomplice of SFE in a child?