ISSN - 0973-0958

Pediatric Oncall Journal View Article

Paroxysmal Cold Hemoglobinuria: Two Cases of a Poorly Recognized Entity
Katherine Huenerberg, Michael Stump, Jerry Squires.
Department of Pathology and Laboratory Services, Medical University of South Carolina, Charleston, SC, USA.
Even though paroxysmal cold hemoglobinuria (PCH) is a well-known cause of autoimmune hemolytic anemia, this syndrome is often not recognized at the time of initial presentation. PCH was originally recognized in individuals with secondary and tertiary syphilis. However, this association with syphilis is now very infrequent, and PCH is more commonly seen in children following a recent infection. In children, the term PCH is misleading. PCH generally presents as a single acute episode of intravascular hemolysis leading to hemoglobinuria in the setting of a recent infection and hence is not paroxysmal. Furthermore, PCH in children is not typically associated with cold exposure. Despite these different clinical presentations, the pathophysiology of the syndrome is the same in children as it is in the classic scenario of an adult with syphilis. We present two classic cases of acute PCH in children at a single institution that illustrate how this syndrome can often be overlooked. By keeping PCH on the differential in a child presenting with significant unexplained anemia in the setting of a recent infection, it may be possible to avoid invasive testing with its many associated costs.

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