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In addition to the patient’s clinical history, an unusual characteristic may provide a clue as to the nature of an antibody. Solve it!

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PCH is a rare autoimmune hemolytic anemia (AIHA) but is one of the most common causes of AIAH in children. For the diagnosis, it is important to perform the appropriate methods of serological investigation and show the typical biphasic reaction. This is a case report of a child who presented with features of haemolysis and was diagnosed with PCH of this way.
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Article
Background: Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare. Case report: We have reported a unique case of recurrent PCH in a 5-year-old boy with two acute episodes of hemolysis separated by 21 months of hematologic remission. Each episode was severe requiring red blood cell transfusions, intravenous methylprednisolone, and intravenous immunoglobulin during the second episode. Testing identified recurrence of the D-L antibody with the classic anti-P biphasic hemolysis. Conclusion: This demonstrates that PCH can be a recurrent disease in the pediatric population (in the absence of syphilis) with the classical D-L antibody.
Article
Red blood cell (RBC) autoimmunization is a relatively uncommon cause of anemia in children and presents some differences from those of adults. Due to its frequency, autoimmune hemolytic anemia (AIHA) in childhood has prompted very few studies, and the literature consists mostly of sporadic case histories. The objective of this study was to stress the importance of an appropriate serologic diagnosis in suspected cases. This report describes the immunohematologic features of 100 patients with AIHA studied in the Immunohaematologic Unit of Blood Bank, "La Sapienza" University of Rome. The patients were diagnosed in the same department from 1983 to 2003. The peak incidence of AIHA was in the first 4 years of life. No sex predominance was noted. Warm AIHA was the most common type of acquired immune hemolytic anemia; it comprised 64 of the 100 patients, whereas 26 patients showed a cold AIHA. Associated AIHA showed a slightly more frequent incidence (54/100) compared to idiopathic forms of AIHA (46/100). In this study serologic records of 100 children with confirmed AIHA are reported. This series, much larger than any previously reported, is critically reviewed and analyzed to delineate the immunologic features of the disease in childhood.
Article
Paroxysmal cold hemoglobinuria (PCH) is a rare autoimmune hemolytic anemia (AIHA) attributed to a biphasic hemolysin known as the Donath-Landsteiner (DL) antibody. It is most commonly encountered as an acute transient AIHA after a viral infection in children; the disease resolves after cessation of the infection. The rarest form of PCH is a chronic form in adults that is not (nowadays) associated with infection and is not responsive to conventional therapies. Rituximab has been found to be effective therapy in other forms of AIHA, such as cold agglutinin syndrome, that are refractory to conventional therapies. We describe a case of PCH refractory to steroids that responded to rituximab therapy on two separate occasions. A 64-year-old woman with fatigue was found to be profoundly anemic with laboratory findings consistent with AIHA. She was admitted for the workup and management of her disease after she failed to respond to a course of oral steroids. Laboratory evaluation demonstrated a positive DL test suggesting PCH. She was given a course of rituximab that resulted in normalization of her hemoglobin concentration. She presented 9 months later with recurrent hemolysis. She was given another course of rituximab that again resulted in termination of hemolysis. The patient remained in remission since her last dose of rituximab 19 months previously. To our knowledge, this is the first report of an adult case of refractory PCH successfully treated with rituximab.
The positive direct antiglobulin test and immune-mediated hemolysis
  • P D Borge
  • P M Mansfield
  • C S Cohn
  • M Delaney
  • S T Johnson
  • L M Katz