Abstract

Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia (AIHA). PCH occurs in acute and chronic forms. The main risk factors for PCH include viral infections, vaccination, and syphilis. PCH presentations are common in the pediatric population. The occurrence of PCH following parvovirus B19 infection in adults is rare. We report a case of a 23-year-old female who presented with giddiness, fatigue, greying of vision, and presyncope for four days, on subsequent evaluation was found to have evidence of hemolysis and bone marrow suppression. Parvoviral intranuclear inclusions were detected in bone marrow biopsy and parvoviral B19 IgM antibody was detected. Donath Landsteiner antibody test was also positive. Hence a diagnosis of PCH secondary to parvovirus B19 infection was made. She was started on pulse dose steroids and intravenous immunoglobulin (IVIG) and showed significant improvement.

Highlights

  • Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia (AIHA), characterized by biphasic, polyclonal IgG autoantibody that binds to the P antigen of RBCs [1]

  • PCH is an AIHA characterized by Donath- Landsteiner (DL) antibody causing red cell lysis [6]

  • Acute forms are more common in the pediatric population, with viral infections and vaccinations being the major etiologies

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Summary

Introduction

Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia (AIHA), characterized by biphasic, polyclonal IgG autoantibody that binds to the P antigen of RBCs [1]. This binding occurs at a lower temperature leading to complement system activation and red cell lysis at 37°C. On the second day of hospital stay, she collapsed suddenly On examination, she was tachycardic and hypotensive with a pulse rate of 112/min, and blood pressure of 80/60 mm Hg. Labs showed a rapid decline in hemoglobin and evidence of hemolysis (Table 2). She was discharged on a tapering dose of steroids with oral prednisolone 50 mg once daily

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Barcellini W
Findings
Heddle NM
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