Donath-Landsteiner Hemolytic Anemia Clinical Presentation

Updated: Jun 28, 2023
  • Author: Trisha Simone Natanya Tavares, MD; Chief Editor: Lawrence C Wolfe, MD  more...
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The classic signs of Donath-Landsteiner hemolytic anemia (DLHA) include a sudden onset of hemoglobinuria accompanied by pallor and mild jaundice. [8] Hemoglobinuria may be absent if the autoantibody level is not high enough to cause intravascular hemolysis. Patients and their caretakers may report dark, brown, black, or otherwise abnormally colored urine.

A preceding history of upper respiratory tract infection or other viral infection is common, particularly in the younger age group. Preceding exposure to cold may be elicited on the patient history. [11] It is not consistently reported, however. In fact, only 1 in 52 cases of DLHA reported by Sokol et al occurred after documented exposure to cold. [20]

Anemia may be mild, moderate, or severe.

High fevers, chills, back or leg pain, and abdominal cramping may also be reported, along with headache, nausea, vomiting, or diarrhea. These signs and symptoms typically resolve early in the clinical course. Some may be secondary to the causative viral infection.

Signs and symptoms of moderate to severe anemia also include dizziness, lightheadedness, palpitations, and shortness of breath.


Physical Examination

Signs of anemia include the following:

  • Pallor

  • Tachycardia

  • Fatigue

  • Dyspnea

  • Cardiac murmur

  • Postural hypotension

Signs of hemolysis include the following:

  • Jaundice

  • Dark urine caused by hemoglobinuria with or without renal failure

  • Splenomegaly - Rare; most likely results from the underlying condition

  • Hepatomegaly - Rare; most likely results from the underlying condition

Systemic signs or symptoms include the following:

  • Fever, rigors

  • Pain or cramping in the back, legs, or abdomen

  • Headache

  • Nausea, vomiting, diarrhea

Vasomotor signs include the following: