Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

Abstract

Objective: To present and discuss the treatment of autoimmune hemolytic anemia (AIHA). Materials and Methods: The medical records of all patients (n=19) diagnosed in a tertiary hematology center between 1999 and 2010 were retrospectively reviewed. Results: Median age at diagnosis of AIHA was 5 years (range: 4 months-17 years). In all, 13 patients had primary (idiopathic) AIHA, whereas 2 had primary Evans Syndrome (ES), 2 had autoimmune lymphoproliferative syndrome (ALPS)+ES, and 1 had Wiskott-Aldrich syndrome (WAS)+AIHA. Among the 13 primary idiopathic AIHA patients, 9 recovered following a 4-8-week course of prednisolone treatment without relapses, whereas 3 patients required a longer course of prednisolone. One AIHA patient that was very resistant to prednisolone recovered after cyclosporine A was added to the treatment. All patients with primary idiopathic AIHA were in remission for a median of 3 years (range: 4 months-10 years) at the time this manuscript was written. Among the patients with primary ES, 2 had relapses similar to the ALPS patients. Splenectomy was performed in 1 primary ES patient, who at the time this report was written was also in remission. One ALPS patient required the addition of mycophenolate mofetil due to prednisolone resistance. The WAS patient was treatment resistant and died due to septicemia. Conclusions: Primary AIHA in pediatric patients generally has an acute onset and good response to corticosteroids. Primary or secondary ES has a chronic or relapsing course, and treatment may require other immunosuppressive agents in addition to corticosteroids. Complications of splenectomy must not be underestimated in patients with underlying immunodeficiency. AIHA often causes considerable morbidity and mortality in WAS. (Turk J Hematol 2011; 28: 198-205)

The spectrum of underlying diseases in children with autoimmune hemolytic anemia

“…In children, the warm AIHA is generally provoked by viral infections and hemolysis may occur. In the literature, warm AIHA cases caused by basic viral infections such as mycoplasma, parvovirus, HBV and HCV, cytomegalovirus, varicella, toxoplasma and Ebstein-Barr virus were reported (3,9,(15)(16)(17)(18). While non-specific infections were detected in most of our patients, 1 had varicella, 1 had HBV, 1 had HEV and 1 had tuberculosis infection.…”

“…In infantile period, it mostly develops due to viral and bacterial infections or vaccination. However, in teenagers and young adults, there is an increased association with an underlying systemic illnesses, most commonly with immunodeficiency, malignancy and autoimmune disorders (systemic lupus erythematosus (SLE), collagen vascular diseases) (3). The AIHA diagnosis generally depends on clinical features, positive direct anti-globulin test (DAT) / Coombs test, laboratory analysis for hemolysis,anemia, hyperbilirubinemia, reticulocytosis.…”

Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma

“…We canceled the scheduled splenectomies for five patients with massive splenomegaly. Furthermore, some patients with cITP and AIHA might be resistant to standard treatment, as in previous reports [ 25 , 26 ]. Partial response to rituximab was observed in cases of AIHA.…”

Coomb’s Test Negative Autoimmune Hemolytic Anemia

“…5 However repeat DAT done by gel technique was also negative in our patient as in some cases, repeat evaluation of DAT may show positive result. 6 There is no single optimal test for investigation of AIHA with negative DAT and there is need for combination of tests to detect different antibodies. 7 Such combination of techniques includes ß ow cytometry, enzyme linked antiglobins test, solid phase or direct polybrene.…”