Premium
Syndrome of Shwachman and Leukaemia
Author(s) -
Huijgens P. C.,
Veen E. A.,
Meijer S.,
Muntinghe O. G.
Publication year - 1977
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1977.tb01473.x
Subject(s) - medicine , exocrine pancreatic insufficiency , neutropenia , pancreas , autopsy , bone marrow , pathology , pediatrics , chemotherapy
The syndrome of Shwachman is characterized by pancreatic insufficiency and bone marrow dysfunction, usually manifesting itself as neutropenia. The pancreas shows replacement of the exocrine glands by adipose tissue; sweat electrolytes are normal. A 23‐year‐old male who was known to suffer from neutropenia and pancreatic dysfunction from early childhood, presented with fever, acquired Pelger‐Huet anomaly (of the polymorphonuclear granulocytes) and sideroblastic anaemia, a combination of symptoms suggestive of preleukaemia. A few months later he died of acute myeloblasts leukaemia and autopsy showed a dystrophic pancreas. Considering this case history it seems possible that the haematological anomalies of Shwachman's syndrome are signs of preleukaemia. Careful follow‐up of patients suffering from Shwachman's syndrome seems warranted.