Date: 09 Jun 2013

Morphological Spectrum of Paroxysmal Nocturnal Hemoglobinuria (PNH)

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Morphological spectrum of paroxysmal nocturnal haemoglobinuria (PNH) is very heterogenous, at one extreme a patient will be diagnosed with hypercellular marrow with unusual thrombosis and mild anaemia and on the other we see a severely aplastic bone marrow with clinical features of PNH and while in some other a diagnosed aplastic anaemia patient with time evolves into PNH like disease. The moot question is whether these diseases are different manifestations of the same disease caught at different time point of their natural history or they are different diseases altogether?

Natural history of PNH has been now been studied in a few well controlled studies [1, 2]. Hillmen et al. [1] showed spontaneous remission in a proportion of these cases on long follow up, a median survival of 10 years and thrombosis and haemorrhage as the major cause of death. Hence the author cautioned against giving potentially dangerous treatment in these patients. Latour et al. [2] describing the natural hist ...