Laboratory characteristics, pathophysiology, and therapy for primary autoimmune hemolytic anemia observed in children

Characteristic	Warm-reactive AIHA	Paroxysmal cold hemoglobinuria	Cold agglutinin disease
Autoantibody isotype	IgG	IgG	IgM
Thermal reactivity	Warm	Cold	Cold
Ability to fix complement	Variable	Yes	Yes
DAT (Coombs test) result	4°C: Not typically performed 37°C: Positive for IgG, ± C3	4°C: Positive for IgG and C3 37°C: Negative for IgG; positive for C3	4°C: Negative for IgG; positive for C3 37°C: Negative for IgG; positive for C3
Antigenic reactivity	Rh, others	P	I or i
Site of hemolysis	Spleen	Intravascular	Liver, intravascular
First-line therapy	Glucocorticoids	Avoidance of cold	Avoidance of cold
Secondary therapy	Splenectomy, Rituximab*	Glucocorticoids	Rituximab*

AIHA: autoimmune hemolytic anemia; IgG: immunoglobulin G; IgM: immunoglobulin M; DAT: direct antiglobulin test (Coombs test); C: Celsius; C3: complement component 3; Rh: Rhesus.
* This is an off-label use of rituximab.

Adapted from: Chou ST, Schreiber AD. Autoimmune hemolytic anemia. In: Nathan and Oski's Hematology and Oncology of Infancy and Childhood, 8^th ed, Orkin SH, Fisher DE, Look T, et al. (Eds), WB Saunders, Philadelphia 2015. p. 411.

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Laboratory characteristics, pathophysiology, and therapy for primary autoimmune hemolytic anemia observed in children