Eosinophilic Variant of Chronic Myeloid Leukemia with Vascular Complications

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.

Eosinophilia With Hepatic Mass and Abnormal Liver Function Tests: An Unusual Presentation of Chronic Myeloid Leukemia

“…Patients with eo-CML are treated the same way as classic CML using tyrosine kinase inhibitors. Our patient showed excellent response to Imatinib like other cases mentioned in the literature [18,19].…”

“…There was no evidence of splenomegaly similar to our patient [ 18 ]. Rare presentations like digital ischemia have been reported as well [ 19 ].…”

“…The literature has documented very few cases with isolated eosinophilia without neutrophilia or basophilia with associated BCR-ABL1 rearrangement termed as eoCML. EoCML is reported to be a rare presentation of CML, which differs from other forms of HES and CEL, NOS by the presence of BCR-ABL1 gene rearrangement and splenomegaly [18][19][20].…”

A multimodality work‐up of patients with Hypereosinophilia

“…Other disease entities with a primary manifestation as HE are uncommon, and we only found one case of inv(16) CEBP/MYH11 AML with an oligoblastic presentation and another case of MDS/MPN with ring sideroblasts and thrombocytosis. We did not find any case of “eosinophilic variant CML (CMLeo),” a rare variant of CML with prominent eosinophilia. This may partly attribute to the wide use of BCR‐ABL1 FISH testing for patients with leukocytosis and eosinophilia; and these patients are not referred to our center for the work‐up of HE.…”

In Response to “BCR-ABL Testing by Polymerase Chain Reaction in Patients With Neutrophilia: The William Beaumont Hospital Experience and the Case for Rational Laboratory Test Requests”

“…Although these features are undeniably common, this approach may fail to identify those patients presenting with isolated thrombocytosis, eosinophilia, or even basophilia without concomitant leukocytosis. [6][7][8] Finally, despite the fact that a majority of cases of essential thrombocythemia and primary myelofibrosis are characterized by the presence of mutations of the JAK2, CALR, and MPL genes and that most patients with chronic neutrophilic leukemia possess mutations of the CSF3R gene, exclusion of the BCR-ABL1 fusion gene remains a major diagnostic criterion for the remainder of patients with these types of myeloid malignancies. 9 It would therefore be interesting to learn the final diagnosis of those patients, particularly those with basophilia, in whom the BCR-ABL1 fusion gene was not detected.…”