D-L auto-antibodies appear approximately one week following an acute illness, usually a virus, causing sudden onset of hemoglobinuria, jaundice, and anemia. DLHA is caused by Donath-Landsteiner (D-L) auto antibody activation against the P-antigen on the red blood cell (RBC), which causes activation of complement, resulting in RBC membrane perforation and intravascular hemolysis (hence, the red urine and elevated urobilinogen in the urine). DHLA is relatively rare (3/100,000) representing 30 percent to 40 percent of autoimmune hemolytic anemia in children. More specifically, this child has a type of paroxysmal cold hemoglobinuria also known as Donath-Landsteiner hemolytic anemia (DLHA). This patient's clinical presentation and laboratory results suggest that he has a form of autoimmune hemolytic anemia (AIHA). Warmed intravenous fluids, packed red blood cell transfusion, observation.What is the best initial therapy for this patient? Abdominal ultrasound to detect splenomegalyĢ.What would be the next best test to help determine this patient’s diagnosis? Urinalysis: dark, red-brown urine, 3+ blood, 3+ protein, 0-4 RBCs/hpf, 0-4 WBCs/hpf, urobilinogen >8mg/dL.Ĭase study submitted by Robyn Dennis, MD, Texas Children’s Cancer Center, Houston, TX.ġ. Electrolytes are all within normal limits. Unconjugated bilirubin is 3.7 mg/dL, Conjugated bilirubin is 1.2 mg/dL. LDH is 5056 Units/ml, haptoglobin 12 mg/dL (low). Labs: Initial hemoglobin 10.4 g/dL-> decreased to 6.3 g/dL 12 hours later, Platelets 153,000 cells/UL, Reticulocyte count 0.4%, White blood cells 11.1 x10 3 cells/UL (48% bands, 16% lymphocytes), Absolute neutrophil count 7.90 x 10 3 cells/UL. Physical Exam: (+) scleral icterus, (+) jaundice, (+) soft flow murmur on cardiac exam. Vitals: Temperature 104.6 degrees Fahrenheit, HR 100, RR 22, Oxygen saturations 98% on room air. He has been having intermittent fevers for three days. He has no increased bruising, no petechiae, and no extremity pain. ROS is negative for dysuria, bloody stools, hemoptysis, or epistaxis. He was seen by his PCP approximately five days ago for evaluation of cough and rhinorrhea and was prescribed Augmentin for an ear infection. He has a two-year-old sister who is healthy. Birth history and past medical history are unremarkable. A four-year-old male presents to the emergency department with a history of six days of fever and acute onset of red colored urine.
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