Hereditary spherocytosis historical perspective
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Overview
- Towards the end of the nineteenth century Vanlair and Masius described the case of a young woman who developed icterus, recurrent attacks of left upper quadrant abdominal pain and splenomegaly shortly after giving birth. The stools were not light coloured, but rather deeply pigmented. The patient's mother and sister were also icteric, and the sister's spleen was enlarged.
Historical Perspective
- Vanlair and Masius (1871), physicians began to note certain chronically icteric patients who had no bile in the urine, no evidence of liver disease and often, but not always, splenomegaly and a family history of jaundice.
- Vanlair and Masius (1871) described the case of a young woman who developed icterus, recurrent attacks of left upper quadrant abdominal pain and splenomegaly shortly after giving birth. The stools were not light coloured, but rather deeply pigmented. The patient's mother and sister were also icteric, and the sister's spleen was enlarged.
- The most remarkable aspect of this paper lies in their description of the blood findings. Although they made no mention of anaemia and had no concept of haemolysis as a pathological process, they unmistakably described spherocytes with remarkable clarity. The authors noted that some of the erythrocytes, which they called microcytes, were smaller than normal cells, 3–4 μm in diameter, spherical in shape (elle est parfaitement spherique) and the contours were completely smooth.
- With remarkable discernment, Vanlair and Masius stated, ‘The jaundice of our patient appears to be a peculiar type of icterus. The fact that the patient’s mother and sister had a slight jaundice and that the sister had an enlarged spleen may indicate that this condition is one disease entity.'.[1]
References
- ↑ Packman, Charles H. (2001). "The spherocytic Haemolytic Anaemias". British Journal of Haematology. 112 (4): 888–899. doi:10.1046/j.1365-2141.2001.02440.x. ISSN 0007-1048.