Approach Considerations
Treatment of Donath-Landsteiner hemolytic anemia (DLHA) may not be necessary in children with stable mild anemia and normal renal function.
Indications for hospitalization in patients with DLHA include the following:
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Worsening anemia
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Severe anemia
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Respiratory or circulatory compromise
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Renal failure
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Severe infection
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Social situation that may preclude prompt access to care if the condition worsens
Transfer patients with severe anemia or complications to a facility where pediatric hematology physicians, blood banking, and pediatric intensive care services are available. Transfer severely ill patients to a facility where consultation with pediatric hematologists, pediatric nephrologists, and pediatric critical care specialists are available.
Anticipate severe anemia and/or acute renal failure. Manage symptoms of underlying infection, if present.
Monitor renal function and hemoglobin until normalized and stable.
Go to Pediatric Chronic Anemia, Anemia of Prematurity, Fanconi Anemia, Pediatric Acute Anemia, and Pediatric Megaloblastic Anemia for complete information on these topics.
Treatment of Severe Disease
Transfusion
Avoid unnecessary transfusions because of the expected transient nature of the condition. Risks of blood transfusion include transfusion reactions and transmission of infection. Blood transfusion is indicated only in selected individuals with Donath-Landsteiner hemolytic anemia (DLHA).
In patients with severe, symptomatic, or rapidly worsening anemia, transfusion of red blood cells (RBCs) must be provided. Guidelines for transfusions in neonates and older children have been established. [29]
For blood typing, perform compatibility testing using techniques to minimize the interference caused by the autoantibody. Consultation with a hematologist and a blood bank specialist may be helpful.
Although P antigen–negative RBCs are most efficacious, these units are extremely rare (most banked blood units are P antigen positive). Blood transfusion may be safely accomplished with P antigen–positive units in most cases, resulting in the expected hemoglobin increases based on the amount administered. Use blood warmers if possible to perform the RBC transfusion at 37°C.
Medications
The use of corticosteroids is controversial but responses have been reported. Corticosteroid therapy may be indicated in patients with DLHA and severe anemia.
Rituximab has been used to treat refractory autoimmune hemolytic anemia, including cold agglutinin disease. Although medical therapy of DLHA beyond the use of steroids is rarely required, rare refractory cases (particularly in adults) have been described in which rituximab was used successfully [30] and in which intravenous immunoglobulin (IVIG) has been used successfully. [2, 31]
Procedures
Plasmapheresis has been used successfully in extremely severe cases refractory to transfusion. [32] Anticipate the need for plasmapheresis early in the patient's clinical course.
Supportive Care
Although exposure to cold is not always clearly associated with disease presentation, supportive care recommendations include avoidance of extreme cold exposure based on an understanding of disease pathophysiology.
Supportive care may also include interventions to prevent or ameliorate renal failure if hemoglobinuria is present. Maintain adequate hydration and urine output. Consult a pediatric nephrologist if renal compromise is identified.
Folic acid supplementation is appropriate because active hemolysis can consume folate and result in megaloblastosis. Folic acid supplementation aids in the production of erythrocytes and in the resolution of anemia.
Management of Infection
Viral infections are self-limited. Treat syphilis and mycoplasmal infections with appropriate therapy. Hemolysis resolves with treatment of the underlying infection.
Splenectomy has no role in treatment of Donath-Landsteiner hemolytic anemia because the hemolysis is intravascular.
Deterrence and Prevention
Currently, prevention of Donath-Landsteiner hemolytic anemia is not possible.
For chronic idiopathic Donath-Landsteiner hemolytic anemia, avoidance of extreme cold exposure may prevent symptoms from recurring.
Long-Term Monitoring
Frequency of surveillance testing must be determined by the clinical course. Perform the following periodic evaluations until the values are normal and stable:
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Clinical examination
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CBC count
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Reticulocyte count
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Total bilirubin
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Lactate dehydrogenase
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Microscopic urinalysis
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Direct antiglobulin test (DAT)
Repeat the above testing if the patient presents with similar signs or symptoms. Have a low threshold to evaluate the child for recurrent disease.
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Blood smear showing spherocytosis, polychromatophilia, and erythrophagocytosis by neutrophils.
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Blood smear showing spherocytosis, polychromatophilia, and erythrophagocytosis by neutrophils.
