An unusual presentation of eosinophilic variant of chronic myeloid leukemia (eoCML)

Aggrawal, Devender K.; Bhargava, Rahul; Dolai, Tuphan Kanti; Singhal, Deepak; Mahapatra, Manoranjan; Rathi, Satyajeet S.; Bohra, Bijender; Rathod, Niranjan

doi:10.1007/s00277-008-0545-1

Cited by 6 publications

(6 citation statements)

References 3 publications

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“…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].…”

Section: Discussionsupporting

confidence: 85%

“…In a case report from India, a patient presented with recurrent superficial thrombophlebitis and eosinophilia secondary to BCR-ABL fusion gene. There was no evidence of splenomegaly similar to our patient [18]. Rare presentations like digital ischemia have been reported as well [19].…”

Section: Discussionsupporting

confidence: 80%

“…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].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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Chronic myeloid leukemia (CML) is a chronic myeloproliferative neoplasm characterized by excess granulocytes at different stages of maturation and the presence of BCR-ABL1 fusion gene or Philadelphia chromosome. Absolute eosinophilia, basophilia, and monocytosis are not uncommon in CML. However, a rare entity called eosinophilic variant of CML (eoCML) can present with eosinophilia without excess neutrophils or basophils. Here, we report a rare and unusual case of eoCML presenting as a liver mass with abnormal liver function tests, which has not been reported in the literature so far.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionsupporting

confidence: 80%

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

et al. 2020

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“…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.…”

Section: Discussionmentioning

confidence: 62%

A multimodality work‐up of patients with Hypereosinophilia

Boddu

Loghavi

et al. 2018

American J Hematol

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The work-up of patients with hypereosinophilia (HE) is complex. Following the recently revised World Health Organization criteria, we retrospectively reviewed 125 patients who were referred to us to exclude a neoplastic cause of HE (2003-2016). The clinical laboratory work-up confirmed secondary HE in 25 (20%) patients; myeloid/lymphoid neoplasms with rearrangements of PDGFRA (n = 9) or PDGFRB (n = 2) (9%); HE associated with a well-defined myeloid neoplasm in 8 (6%); and abnormal bone marrow and/or molecular genetic abnormalities consistent with chronic eosinophilic leukemia (CEL), not otherwise specified (NOS) in 21 (17%) patients. For the remaining 60 (48%) patients, a specific diagnosis was not identified, and 56 patients had HE related findings consistent with idiopathic hypereosinophilic syndrome (HES), while 4 patients who were asymptomatic. With a median follow up of 35.3 months (range, <1-104), patients with CEL, not otherwise specified (NOS) had a median OS of 26.1 months, significantly inferior to patients with idiopathic HES (not reached, P < .01). Thus, our experience in a single tertiary cancer center shows that the work-up of HE following WHO recommendations requires a multimodality-based approach; and a correct diagnosis determines risk stratification and proper patient management. However, the causes of HE remain unknown in approximately half of referred patients, indicating the need for further studies.

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“…Mild eosinophilia (> 5% eosinophils) at presentation is common in CML and observed in up to 78% patients [29]. However, the term "eosinophilic variant CML" [30,31] is specifically used to describe a variant of CML with prominent eosinophilia with no significant neutrophilia or basophilia. Although these eosinophilic variant CML cases respond to TKI therapy, HE can cause heart, lungs, and CNS damage that may be severe and irreversible.…”

Section: Primary (Clonal) Hementioning

confidence: 99%

The Diagnostic Work-Up of Hypereosinophilia

Wang¹

2018

Pathobiology

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Hypereosinophilia (HE) is defined as a persistent elevated eosinophil count of ≥1.5 × 10⁹/L. HE can be one of the dominant manifestations of a hematopoietic myeloid neoplasm or secondary/reactive to an underlying medical condition. If a cause of HE and its associated tissue/organ damage is not determined, the condition is considered to be idiopathic hypereosinophilic syndrome (HES). The work-up of HE can be challenging due to a broad range of causes of HE that can be either reactive or neoplastic. In recent years, with the advent of molecular genetic testing and the introduction of targeted therapy in the management of these patients, there is a growing interest in better characterization of these diseases. Using a multimodality approach and following a proper algorithm, a diagnosis can be made in a large proportion of patients. In idiopathic HES, myeloid neoplasm associated somatic mutations as evidence of clonality are reported in 20–25% patients; however, the mutation data should be interpreted cautiously considering the prevalence of clonal hematopoiesis of indeterminate potential (CHIP). Bone marrow morphology has been shown to have important value in the identification of a true myeloid neoplasm in these disorders. A genome-wide study may be needed to understand the “idiopathic” cases that would ultimately lead to better patient care.

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An unusual presentation of eosinophilic variant of chronic myeloid leukemia (eoCML)

Cited by 6 publications

References 3 publications

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

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

A multimodality work‐up of patients with Hypereosinophilia

The Diagnostic Work-Up of Hypereosinophilia

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