In the hypergranular group of acute promyelocytic leukemia (APL) a rar
e subvariant with basophilic granules, metachromatic for toluidine blu
e, is recognizable. To evaluate the incidence as well as the biologica
l and clinical significance of this subtype, we studied 53 consecutive
untreated patients with APL with morphological, cytochemical, immunol
ogical and cytogenetic methods. In 10 cases (19% of the total) granule
s stained metachromatically in percentages of promyelocytes ranging fr
om 16 to 60. In these cases peroxidase positivity was weaker than in t
he classic hypergranular and microgranular M3 and activities of estera
ses were usually present; at the ultrastructural level granules contai
ned particulate material. Immunophenotypic and cytogenetic characteris
tics seemed not to differ from those of other M3 cases. Coagulopathy w
as usually life-threatening, notwithstanding the low white cell count,
and the median survival was short. Hyperhistaminemia-related symptoms
were not observed. Cytochemical, immunologic and cytogenetic findings
are useful to differentiate this form from M2 with basophilic differe
ntiation and from mast cell leukemia.