Eosinophilic variant of chronic myeloid leukemia with vascular complications

Leuk Lymphoma. 2003 Sep;44(9):1609-13. doi: 10.3109/10428190309178786.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Anticoagulants / therapeutic use
  • Antineoplastic Agents / therapeutic use
  • Benzamides
  • Bone Marrow / pathology
  • Diagnostic Errors
  • Eosinophilia / etiology*
  • Fingers / blood supply
  • Gangrene
  • Humans
  • Imatinib Mesylate
  • Leg / blood supply
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / complications*
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / diagnosis
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / drug therapy
  • Male
  • Pain / diagnosis
  • Pain / etiology
  • Piperazines / therapeutic use
  • Pyrimidines / therapeutic use
  • Radiography
  • Vasculitis / diagnostic imaging
  • Vasculitis / drug therapy
  • Vasculitis / etiology*
  • Vitamins / therapeutic use

Substances

  • Anticoagulants
  • Antineoplastic Agents
  • Benzamides
  • Piperazines
  • Pyrimidines
  • Vitamins
  • Imatinib Mesylate