Paroxysmal nocturnal hemoglobinuria; case study, including evidence of affection of the marrow in the disease.

PUtOXY5MAL NOCTURNAL HEMosLoBrxu1tI.s (P.N.H.) is a chronsic hemolytic disease which affects all the formed blood elements. Once the disease is established these elemensts, includinig their boise marrow precursors, becomsse liable to excessive destructions. We do not yet knsow what causes the blood elements ins P.N.H. to be abmsornnahly labile, though the mechanism insvolved ins their destrunction is reasonsably well umsderstood. The consjoined names of Marchiafava aiid Michehi are popunlarly thounght of ins cousmiectioms with this disease, but those aunthors were certainsly not the first to describe it ansd ins any case their description shows that they did niot appreciate sonic of its basic pheniomemsa. Crosby,2 who most carefully collated the literature on the subject amid who hinssself made fundamenst-al cohstrii)utions to its unsderstansdimsg, has writt-emi a most insterest-insg Isistorical review on P.N.H. He suggests that the patienst described as long ago as 1793 by Charles Stewart was probably a victim of the disease, amid goes oni to pay tribute to the niext available report published by Williann GunlI in 1866. It is clear, however, that Paul Strubinsg’8 in 1882 was the first to recogisize ins a patient of his the essential features of the disease as we uniderstamsd themn, amid evens msow we cats add connparatively little to his observationss. Crosby2 is unsdoubtedly right whets he suggests that this work failed to attract the attemitions it deserved becausse Strubimsg’s ideas were so much in advance of his time ansd beoausse he failed to give the disease a distinsct-ive title. Evens the presenst designsations of the disease is not altogethser appropriate because the hiemolyt-ic process, though liable to periodic exacerbations, is, strict-ly speaking, constinuous. Crosby3 estimated that P.X.H. has ans inscidence of two per nusillions.Its huis consprehenssive review lie was unniable to discover amsy sex preponsderansce, racial or hereditary insfiuences, ansd found the maximuns inscidensce of the disease to be in the t-hsird or fourth decades, with a rausge extensdinsg from unsder 1 year to 72. He was able to collectat that time 162 published cases, ansd of those only 21 appeared ins the British literature. We feel justified, therefore, ins recording a fresh case whsichs sve were able to stundy ins detail.