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Adult onset of acute myeloid leukaemia (M6) in patients with Shwachman‐Diamond syndrome
Author(s) -
Dokal Inderjeet,
Rule Simon,
Chen Fred,
Potter Mike,
Goldman John
Publication year - 1997
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1997.3673181.x
Subject(s) - myeloid leukaemia , medicine , myeloid , pediatrics , immunology
Three male patients (two of whom were brothers) with Shwachman‐Diamond (SDS) syndrome presented with acute myeloid leukaemia in adulthood. In all three cases there was trilineage myelodysplasia and the morphology was consistent with FAB subtype M6. SDS is an inherited bone marrow failure syndrome with a high propensity to leukaemic transformation. Since this may not occur until adulthood, SDS should be considered in the differential diagnosis of adults presenting with acute myeloid leukaemia, particularly where features of myelodysplasia are prominent.