Paroxysmal Nocturnal Haemoglobinuria-Associated Acute Cutaneous Thrombosis and Haemolysis in the Setting of Parvovirus B19 and Varicella Zoster Virus Infection.
Аннотация
A 28-year-old male with paroxysmal nocturnal haemoglobinuria (PNH) presented with headache, nasal bridge discomfort and haemoglobinuria. He developed a macular-papular rash which rapidly progressed into purpura, necrosis and peri-orbital oedema. Investigations demonstrated severe haemolytic anaemia and acute parvovirus B19 infection. A skin biopsy confirmed extensive necrotising vasculitis with intravascular thrombi, affecting the dermal and subcutaneous vessels. Eculizumab, corticosteroid and intravenous immunoglobulin were given, and within 24 h, symptoms markedly improved. The patient later developed breakthrough haemolysis in the setting of varicella zoster virus infection, which responded well to additional anti-complement therapy. This case demonstrates that viral infections can precipitate acute thrombosis and severe haemolysis in PNH patients and that a combined treatment approach confers good recovery. Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.
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