Eosinophilic variant of chronic myeloid leukemia with vascular complications.
Eosinophilic variant of CML (eoCML) is a unique disease with a poor prognosis. Like the hypereosinophilic syndrome (HES), eoCML has no clinically identifiable reason for an increased eosinophil count in the peripheral blood. In contrast to HES, eoCML patients carry a distinct chromosomal abnormality. The bcr/abl fusion gene (Philadelphia chromosome) is the genetic basis of this clonal disease. Recently, eoCML has been separated from HES. Patients with eoCML frequently suffer organ damage including the heart and lungs. This damage is related to the release of eosinophilic granules in the blood, which results in fibrosis of the endothelial lining. We report a case of a peripheral vasculitis complicated by gangrene of the fingers in a patient with eoCML. Despite an almost complete response to CML treatment with Gleevac, combined with prednisone, aspirin and coumadin the patient sustained irreversible damage to the vascular lining of the distal arteries of the upper extremities.
Gotlib
Vladimir
V
Division of Hematology/Oncology, The Brooklyn Hospital Center, 121 DeKalb Ave, Brooklyn, NY 11201, USA. vzgotlib@aol.com
Darji
Jaykrishna
J
Bloomfield
Kimberly
K
Chadburn
Amy
A
Patel
Arun
A
Braunschweig
Ira
I
D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D001853:Bone Marrow / Q000473:pathology; D003951:Diagnostic Errors; D004802:Eosinophilia / Q000209:etiology*; D005385:Fingers / Q000098:blood supply; D005734:Gangrene; D006801:Humans; D000068877:Imatinib Mesylate; D007866:Leg / Q000098:blood supply; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D010146:Pain / Q000175:diagnosis / Q000209:etiology; D010879:Piperazines / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D011859:Radiography; D014657:Vasculitis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D014815:Vitamins / Q000627:therapeutic use
D000925:Anticoagulants; D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D014815:Vitamins; D000068877:Imatinib Mesylate