Resolution of platelet function defects with imatinib therapy in a patient with chronic myeloid leukaemia in chronic phase.
Platelet function defects are frequently found in patients with chronic myeloid leukaemia. Major clinical bleeding, however, is a rare and infrequently reported complication. Platelet function abnormalities have also not been previously correlated with molecular monitoring of BCR-ABL in chronic myeloid leukaemia. We report a case of a patient with major clinical bleeding as a presenting feature of chronic myeloid leukaemia. The patient developed compartment syndrome of the thigh secondary to a haematoma developing after minor trauma. Fasciotomy was complicated by severe bleeding requiring massive transfusion. Haemostasis was only obtained after activated recombinant factor VII was administered. Laboratory investigations revealed a platelet function defect with reduced platelet aggregation to collagen, epinephrine and arachidonic acid. As imatinib therapy commenced, molecular response was associated with near-normalization of platelet function, which subsequently became significantly abnormal with molecular relapse. Electron microscopy demonstrated normal platelet ultrastructure. We conclude that dysregulated Abelson kinase plays a pathogenic role in platelet function defects associated with chronic myeloid leukaemia, and discuss the management of clinically significant bleeding in patients with platelet function defects associated with myeloproliferative disorders.
Ng
Ashley P
AP
Department of Diagnostic Haematology, Royal Melbourne Hospital, Melbourne, Australia. dr.ashley.ng@gmail.com
Servadei
Patricia
P
Tuckfield
Annabel
A
Friedhuber
Anna
A
Grigg
Andrew
A
D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001549:Benzamides; D001791:Blood Platelet Disorders / Q000175:diagnosis*; D006801:Humans; D000068877:Imatinib Mesylate; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D010879:Piperazines / Q000627:therapeutic use*; D010979:Platelet Function Tests; D011743:Pyrimidines / Q000627:therapeutic use*
D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate