Paroxysmal nocturnal hemoglobinuria historical perspective

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

Paroxysmal nocturnal hemoglobinuria was first described by the European physicians in the 19th century. The hypothesis of hemolysis of red blood cells due to the increased plasma acidity in the night time was described by Dr. Strübing in 1815. From 1911 to 1950, more studies were conducted in order to establish the full description of PNH.

Historical Perspective

Discovery

• In the 19th century, the European physicians conducted observational studies which resulted in the establishment of Paroxysmal Nocturnal Hemoglobinuria (PNH) as a separate disease from paroxysmal cold hemoglobinuria and march hemoglobinuria.
• In 1815, Dr. Paul Strübing, a German physician, was the first to put the hypothesis of PNH. The hypothesis mentioned the abnormal hemolysis of the red blood cells due to increased plasma acidity in the night time.
• Dr. Paul returned the abnormal hemolysis of the RBCs due to increased carbon dioxide gas as a result of the slow circulation nocturnally.
• In 1911, Dr. Ettore Marchiafava and Dr. Alessio Nazari added a more description about PNH.
• In 1939, Dr. Thomas Hale Ham also reported an evidence about the complement induced hemolysis in PNH. These landmark leads to development of diagnostic test named on Dr. Ham and called the acidified serum lysis test or the Ham test.
• In 1950, as a result of the discovery of PNH, Dr. Louis Pillemer described the alternative complement pathway.
• In 1951, Dr. Crosby reported a review of history of PNH. The review was built on the obsevations of Dr. Paul Strübing. Dr. Crosby described the natural history of PNH and how thrombosis may develop as a complication of the disease.