Anemia, Autoimmune Hemolytic

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.

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.