Donath-Landsteiner Hemolytic Anemia Treatment & Management

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:

• Severe anemia
• Respiratory or circulatory compromise
• Renal failure
• Severe infection

Transfer patients with severe anemia or complications to a facility where pediatric hematology physicians, blood banking, and pediatric intensive care services are available.

Go to Pediatric Chronic Anemia, Anemia of Prematurity, Fanconi Anemia, Pediatric Acute Anemia, and Pediatric Megaloblastic Anemia for complete information on these topics.

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.^[16]

For blood typing, perform compatibility testing using the techniques described to minimize the interference caused by the autoantibody (see Workup). Consultation with a hematologist and/or 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. 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.^[17]

Procedures

Plasmapheresis has been used successfully in extremely severe cases refractory to transfusion.^[18]

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.

Folic acid supplementation is appropriate because active hemolysis can consume folate and result in megaloblastosis.

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.

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.

Frequency of surveillance testing must be determined by the clinical course. Perform the following periodic evaluations until the values are normal and stable:

• Clinical examination
• CBC count
• Reticulocyte count
• Total bilirubin
• Lactate dehydrogenase
• Microscopic urinalysis
• Direct antiglobulin test (DAT)