Cutaneous thrombosis related to paroxysmal nocturnal hemoglobinuria: clinical report.

ABSTRACT

Paroxysmal nocturnal hemoglobinuria is a clonal stem cell disorder typically characterized by hemolysis, bone marrow failure, and venous thrombosis. The latter can affect up to 40 percent of patients and is a significant cause of mortality. Despite being recognized as a possible complication, cutaneous thrombosis is quite uncommon and a variety of findings have been reported. Owing to the rarity of this complication and the importance of its correct and timely diagnosis, we report the case of a 64-year-old man with paroxysmal nocturnal hemoglobinuria who presented with isolated and well-demarcated erythematous-violaceous plaques, mainly located on the neck and trunk. Given the history and the histological features, the diagnosis of cutaneous thrombosis related to paroxysmal nocturnal hemoglobinuria was assumed and the patient was successfully treated with therapeutic anticoagulation. The early identification of these patients is essential for necessary surveillance because they could carry a higher probability of recurrence compared with those with no prior complications.