The spectrum of underlying diseases in children with autoimmune hemolytic anemia

Year 2021, Volume: 4 Issue: 6, 772 - 778, 24.09.2021

Alper Özcan , Murat Cansever , Ebru Yılmaz , Ekrem Ünal , Musa Karakükçü , Türkan Patıroğlu

https://doi.org/10.32322/jhsm.985008

Cited By: 1

Abstract

Aim: Autoimmune hemolytic anemia (AIHA) is characterized by the production of antibodies directed against red blood cells. We aimed to describe the clinical presentation, hematologic and biochemical profiles, treatment modalities, underlying diseases and outcomes in children suffering from AIHA.
Material and Method: In a retrospectively manner, we consecutively recrutied 62 children (aged 1 month-18 years) with AIHA who had been followed in Erciyes University Child Hospital between January, 2000 and November, 2017.
Results: The mean age at time of diagnosis was 61.9±51.9 months (range:1-192) in 62 children including 28 girls and 34 boys. The most common complaints included fatigue and fever while the most common findings were jaundice and hepatosplenomegaly. In 22 children (35.4%), there was a comorbid, active, non-specific infection (upper respiratory tract infection, lower respiratory tract infection, diarrhea, urinary tract infection). At time of diagnosis, mean hemoglobin (Hb) level was 6.9±2.5 g/dL (range: 5-9). The glucocorticoid therapy was given in majority of the patients while no medical treatment was given to 4 patients. Of the patients with primary AIHA, 6 patients were unresponsive to the treatment while one patient responded partially. It was found that there were underlying risk factors in 36 patients with secondary AIHA, as immunodeficiency and autoimmune disorders being the most common risk factors.
Conclusion: The immunodeficiencies were highly prevalent in children included. By advance of whole exome sequencing technology, we believe that primary immunodeficiencies was the most common underlying disease in our study detected quite high in presented children.

Keywords

Autoimmune hemolytic anemia, children, immunodeficiency

References

• Liebman HA, WeitzIC. Autoimmune hemolytic anemia. Med Clin North Am 2017; 101: 351-9.
• Aladjidi N, Jutand MA, Beaubois C, et al. Reliable assessment of the incidence of childhood autoimmune hemolytic anemia. Pediatr Blood Cancer 2017; 64: 12.
• Sarper N, Çakı Kılıç S, Zengin E, et al. Management of autoimmune hemolytic anemia in children and adolescents: A single center experience. Turk J Haematol 2011; 28: 198-205.
• Vagace JM, Bajo R, Gervasini G. Diagnostic and therapeutic challenges of primary autoimmune haemolytic anaemia in children. Arch Dis Child 2014; 99: 668-73.
• Carson JL, Grossman BJ, Kleinman S, et al. Clinical Transfusion Medicine Committee of the AABB. Red blood cell transfusion: a clinical practice guideline from the AABB*. Ann Intern Med. 2012; 157: 49-58.
• Go RS, Winters JL, Kay NE. How I treat autoimmune hemolytic anemia. Blood.2017; 129: 2971-9.
• Ladogana S, Maruzzi M, Samperi P, et al. Diagnosis and management of newly diagnosed childhood autoimmune haemolytic anaemia. Recommendations from the Red Cell Study Group of the Paediatric Haemato-Oncology Italian Association. Blood Transfus 2017;15: 259–67. 
• Ozcan A, Canpolat M, Doganay S, et al. Cerebral sinus venous thrombosis and prothrombotic risk factors in children: a single-center experience from Turkey. J Pediatr Hematol Oncol 2018; 40: 369-72.
• Yaralı N, Bilir ÖA, Erdem AY, et al. Clinical features and treatment of primary autoimmune hemolytic anemia in childhood. Transfus Apher Sci 2018; 57: 665-8.
• 10.Naithani R, Agrawal N, Mahapatra M, et al. Autoimmune haemolytic anemia in children. Pediatr Hematol Oncol 2007; 24: 309-15.
• Aladjidi N, Leverger G, Leblanc T, et al. Centre de Référence National des Cytopénies Auto-immunes de l'Enfant (CEREVANCE). New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children. Haematologica 2011; 96: 655-63.
• Sankaran J, Rodriguez V, Jacob EK, et al. Autoimmune hemolytic anemia in children: mayo clinic experience. J Pediatr Hematol Oncol 2016; 38: 120-4.
• Berentsen S, Sundic T. Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. Biomed Res Int 2015; 2015: 363278.
• Hill QA, Hill A, Berentsen S. Defining autoimmune hemolytic anemia: a systematic review of the terminology used for diagnosis and treatment. Blood Adv 2019; 3: 1897-906.
• Apak H, Karaman S, Doğan Y,etal. Varicella-induced haemolytic anemia with hepatitis. Ann Hematol 2006; 85: 64-5.
• Zhang QL, Jia LJ, Zhang JB, et al. HBV and HCV coinfection associated with warm-type autoimmune hemolytic anemia: a case report. Turk J Haematol 2014; 3: 328-31.
• Yen YF, Lan YC, Huang CT, et al. Human immunodeficiency virus infection increases the risk of incident autoimmune hemolytic anemia: a population-based cohort study in Taiwan. J Infect Dis 2017; 216: 1000-7.
• Patiroglu T, Ozcan A, Karakukcu M, et al. Mycophenolate mofetil-induced pseudotumor cerebri in a boy with autoimmune lymphoproliferative disease. Childs Nerv Syst 2011; 27: 853-5.
• Parker V, Tormey CA. The direct antiglobulin test: indications, interpretation, and pitfalls. Arch Pathol Lab Med 2017; 141: 305-10.
• Kim TO, Grimes AB, Kirk S, Arulselvanet al. Association of a positive direct antiglobulin test with chronic immune Thrombocytopenia and use of second line therapies in children: A multi-institutional review. Am J Hematol 2019; 94: 461-6.
• Farmer JR, Foldvari Z, Ujhazi B, et al. Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.J Allergy Clin Immunol Pract 2019; 7: 1970–85.
• Patiroglu T, Haluk Akar H, Gilmour K, et al. A case of XMEN syndrome presented with severe auto-immune disorders mimicking autoimmune lymphoproliferative disease. Clin Immunol 2015; 159: 58–62.
• Patiroglu T, Gungor HE, Unal E. Autoimmune diseases detected in children with primary immunodeficiency diseases: results from a reference centre at middle anatolia. Acta Microbiol Immunol Hung 2012; 59: 343–53.
• Patiroglu T, Akar HH, Gilmour K, et al. Atypical severe combined immunodeficiency caused by a novel homozygous mutation in Rag1 gene in a girl who presented with pyoderma gangrenosum: a case report and literature review. J Clin Immunol 2014; 34: 792–5.
• Miller J, Cai W, Andrews J, et al. A case series of pediatric patients with direct antiglobulin test negative autoimmune hemolytic anemia. Transfusion 2019; 59: 2528-1.
• Patiroglu T, Klein C, Gungor HE, et al. Clinical features and genetic analysis of six patients with Wiskott-Aldrich syndrome reporting two novel mutations: experience of Erciyes University, Kayseri, Turkey. Genet Couns 2016; 27: 9–24.
• Barros MM, Blajchman MA, Bordin JO. Warm autoimmune hemolytic anemia: recent progress in understanding the immunobiology and the treatment. Transfus Med Rev. 2010;24:195-210.
• Dierickx D, Kentos A, Delannoy A. The role of rituximab in adults with warm antibody autoimmune haemolytic anemia.Blood 2015; 125: 3223-9.
• von Baeyer H. Plasmapheresis in immune hematology: review of clinical outcome data with respect to evidence-based medicine and clinical experience. Ther Apher Dial 2003; 7: 127-40.
• Barcellini W. Immune Hemolysis: Diagnosis and Treatment Recommendations. Semin Hematol 2015; 52: 304-12.
• Hirano Y, Itonaga T, Yasudo H, et al. Systemic lupus erythematosus presenting with mixed-type fulminant autoimmune hemolytic anemia. Pediatr Int 2016; 58: 527-30.
• Vaglio S, Arista MC, Perrone MP, et al. Autoimmune haemolytic anemia in childhood: serologic features in 100 cases. Transfusion 2007; 47: 50-4.
• Becheur M, Bouslama B, Slama H, et al. [Autoimmune haemolytic anemia in children]. Transfus Clin Biol 2015; 22: 291-8.
• Miano M. How I manage Evans Syndrome and AIHA cases in children. Br J Haematol 2016; 172: 524-34.
• Price V. Auto-immune lymphoproliferative disorder and other secondary immune thrombocytopenias in childhood. Pediatr Blood Cancer. 2013; 60: S12-4.
• Fan J, He H, Zhao W, et al. Clinical features and treatment outcomes of childhood autoimmune hemolytic anemia: a retrospective analysis of 68 Cases. J Pediatr Hematol Oncol 2016; 38: 50-5.
• Naithani R, Agrawal N, Mahapatra M, et al. Autoimmune haemolytic anemia in India: clinico-hematological spectrum of 79 cases. Hematology 2006; 11: 73-6.
• Garratty G. Immune hemolytic anemia caused by drugs. Expert Opin Drug Saf 2012; 11: 635- 42.
• Albayrak M, Kaya Z, Kocak U, et al. Fatal autoimmune hemolytic anemia in a child with B-cell leukemia. Erciyes Med Journal 2009; 31: 37-40.
• Parker CJ. Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria. Hematology Am Soc Hematol Educ Program 2016; 2016: 208-16.