The syndrome described by Fanconi (1927), which consists of hypoplastic anaemia, multiple anatomical defects and pigmentation of the skin is now well recognized though uncommon; some 28 cases have been recorded.This report is made with the object, not only of placing on record the features of two cases, but of examining the place of splenectomy in treatment. In nine instances splenectomy has now been performed and the results have been unsatisfactory. Opinions as to the value of operation and the indications for performing it differ, though it is generally agreed to be valueless if marrow hypoplasia is extreme.Case Report Dorothy M., aged 8 (born on June 13, 1944), was the only child of a second marriage. There were four children from previous marriages of her parents, and these children were all normal. There was no history of anaemia or congenital abnormality in any other member of the family. The parents of the child were healthy and their blood counts were normal.She had always been well, apart from an attack of measles, followed by pneumonia, when she was aged 6.The parents denied that she was ever lacking in energy, and had not noticed that her appearance was in any way unusual. A month before admission, she had bled from the nose and throat for two days, and had been pale ever since.On adrnission, the child was found to be very small. No abnormality could be found in the heart or lungs, and there was no lymphadenopathy, splenomegaly or hepatomegaly. There was no abnormality of the external urogenital system and the central nervous system appeared normal.Investigations. A full blood count gave: haemoglobin, 37% (5-4 g. %); red blood cells, 152 million; colour index, 1*23; white blood cells 6,400 (polymorphs 29%, lymphocytes 66%, monocytes 3%, basophils 2%, reticulocytes 4 4%). Red blood cells showed anisocytosis and poikilocytosis. Cells were fully haemoglobinized. There were no spherocytes, target cells or sickle cells. Haemolysis was incomplete at 0 45%, complete at 0 3%. The direct Coombs test was negative and no warm or cold agglutinins were detected by Dacie's method.
