para>Vaccinate against S. pneumoniae, N. meningitidis, and H. influenzae > 2 weeks before splenectomy
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
- Symptomatic patients (tachycardia, dyspnea)
- Unstable patients (significant anemia or hemolysis with end-organ damage) (1,2,7)[C]
- Patients requiring surgery or patients requiring stabilization with transfusions (2,7)[C]
ALERT
Use prewarmed IV fluids in cold AIHA to avoid major hemolytic crises.
Discharge Criteria
- Cessation or reduction in hemolysis (2,7)[C]
- Uneventful posttransfusion or operative course (2,7)[C]
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
- Serial hemoglobin measurements to evaluate degree of anemia and/or ongoing hemolysis (2,7)[C]
- Avoidance of hypothermic fluids, blood products, or surgeries for cold AIHA (2,7)[C]
PATIENT EDUCATION
Behavior modification in cold AIHA (2,4)[C]
PROGNOSIS
- Primary AIHA: good with appropriate lifestyle changes and pharmacologic treatment (1,2)[C]
- Secondary AIHA: dependent on prognosis of underlying disorder (1)[C]
COMPLICATIONS
- End-organ damage related to severe anemia (1,2,7)
- Hepatic or splenic dysfunction (1,2)
- Treatment side-effects (see above) (2,7)
REFERENCES
11 Bass GF, Tuscano ET, Tuscano JM. Diagnosis and classification of autoimmune hemolytic anemia. Autoimmun Rev. 2014;13(4-5):560-564.22 Michel M. Warm autoimmune hemolytic anemia: advances in pathophysiology and treatment. Presse Med. 2014;43(4, Pt 2):e97-e104.33 Vagace JM, Bajo R, Gervasini G. Diagnostic and therapeutic challenges of primary autoimmune haemolytic anaemia in children. Arch Dis Child. 2014;99(7):668-673.44 Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood. 2013;122(7):1114-1121.55 Arndt PA. Drug-induced immune hemolytic anemia: the last 30 years of changes. Immunohematology. 2014;30(2):44-54.66 Bloch EM, Sakac D, Branch HA, et al. Western immunoblotting as a new tool for investigating direct antiglobulin test-negative autoimmune hemolytic anemias. Transfusion. 2015;55(6, Pt 2):1529-1537. doi: 10.1111/trf.13082.77 Zanella A, Barcellini W. Treatment of autoimmune hemolytic anemias. Haemotologica. 2014;99(10):1547-1554.88 Salama A, Hartnack D, Lindemann HW, et al. The effect of erythropoiesis-stimulating agents in patients with therapy-refractory autoimmune hemolytic anemia. Transfus Med Hemother. 2014;41(6):462-468.99 Jordan SD, Alexander E. Bovine hemoglobin: a nontraditional approach to the management of acute anemia in a Jehovah's Witness patient with autoimmune hemolytic anemia. J Pharm Pract. 2013;26(3):257-260.
ADDITIONAL READING
Reynaud Q, Durieu I, Dutertre M, et al. Efficacy and safety of rituximab in auto-immune hemolytic anemia: a meta-analysis of 21 studies. Autoimmun Rev. 2015;14(4):304-313.
SEE ALSO
- Leukemia; Lupus Erythematosus, Systemic (SLE)
- Algorithm: Anemia
CODES
ICD10
- D59.1 Other autoimmune hemolytic anemias
- D59.0 Drug-induced autoimmune hemolytic anemia
ICD9
283.0 Autoimmune hemolytic anemias
SNOMED
- Autoimmune hemolytic anemia (disorder)
- Drug-induced autoimmune hemolytic anemia (disorder)
- Warm autoimmune hemolytic anemia (disorder)
- Cold autoimmune hemolytic anemia (disorder)
CLINICAL PEARLS
- AIHA is a rare cause of anemia; diagnosis requires evidence of hemolytic anemia and a positive direct antiglobulin test (Coombs).
- In AIHA secondary to an underlying process (e.g., CLL, SLE, EBV, Mycoplasma), treatment of the underlying process determines prognosis.
- For primary warm AIHA, corticosteroids are first-line treatment; alternatives include rituximab, IVIG, and splenectomy.
- For primary cold AIHA, avoidance of hypothermic conditions (weather, fluids, surgery) is preventative.
- For primary cold AIHA requiring treatment, rituximab is more efficacious than corticosteroid therapy.