Pure Erythroid Leukemia in a Sickle Cell Patient Treated with Hydroxyurea

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Abstract

We present a very rare case of pure erythroid leukemia arising in a young patient with sickle cell disease being treated with hydroxyurea for almost 5 years. Diagnosing and managing this rare condition has been a challenge and the majority of patients with pure erythroid leukemia have a very poor prognosis with survival in months despite treatment. This form of leukemia could be therapy related and in our case, hydroxyurea may have been responsible for the development of this aggressive condition.

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Most cited references 17

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The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).

E. Rivas Plata, George Stamatopoulos, A Balassopoulou … (2010)

The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/beta(0)-thal, and 165 with HbS/beta(+)-thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P = .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/HbS, HbS/beta (0)-thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients.

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More than 1 TP53 abnormality is a dominant characteristic of pure erythroid leukemia.

Guillermo Montalban-Bravo, Christopher B Benton, Sa Wang … (2017)

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Acute myeloid leukemia and myelodysplastic syndromes following essential thrombocythemia treated with hydroxyurea: high proportion of cases with 17p deletion.

Y Sterkers, C Preudhomme, J L Lai … (1998)

Treatment with alkylating agents or radiophosphorous (32P) has been shown to carry a certain leukemogenic risk in myeloproliferative disorders (MPDs), including essential thrombocytemia (ET). The leukemogenic risk associated to treatment with hydroxyurea in ET, on the other hand, is generally considered to be relatively low. Between 1970 and 1991, we diagnosed ET in 357 patients, who were monitored until 1996. One or several therapeutic agents had been administered to 326 patients, including hydroxyurea (HU) in 251 (as only treatment in 201), pipobroman in 43, busulfan in 41, and 32P in 40. With a median follow-up duration of 98 months, 17 patients (4.5%) had progressed to acute myeloid leukemia (AML; six cases) or myelodysplastic syndrome (MDS; 11 cases). Fourteen of these patients had received HU, as sole treatment in seven cases, and preceded or followed by other treatment in seven cases, mainly pipobroman (five cases). The remaining three leukemic progressions occurred in patients treated with 32P (two cases) and busulfan (one case). The incidence of AML and MDS after treatment, using 32P alone and 32P with other agents, busulfan alone and with other agents, HU alone and with others agents, and pipobroman alone and with other agents was 7% and 9%, 3% and 17%, 3.5% and 14%, and 0% and 16%, respectively. Thirteen of 17 patients who progressed to AML or MDS had successful cytogenetic analysis. Seven of them had rearrangements of chromosome 17 (unbalanced translocation, partial or complete deletion, isochromosome 17q) that resulted in 17p deletion. They also had a typical form of dysgranulopoiesis combining pseudo Pelger Hüet hypolobulation and vacuoles in neutrophils, and p53 mutation, as previously described in AML and MDS with 17p deletion. Those seven patients had all received HU, as the only therapeutic agent in three, and followed by pipobroman in three. The three patients who had received no HU and progressed to AML or MDS had no 17p deletion. A review of the literature found cytogenetic analysis in 35 cases of AML and MDS occurring after ET, 11 of whom had been treated with HU alone. Five of 35 patients had rearrangements that resulted in 17p deletion. Four of them had been treated with HU alone. These results show that treatment with HU alone is associated with a leukemic risk of approximately 3.5%. A high proportion of AML and MDS occurring in ET treated with HU (alone or possibly followed by pipobroman) have morphologic, cytogenetic, and molecular characteristics of the 17p- syndrome. These findings suggest that widespread and prolonged use of HU in ET may have to be reconsidered in some situations, such as asymptomatic ET.

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Author and article information

Journal

Journal ID (publisher-id): CRO

Journal ID (pmc): CRO

Journal ID (doi): 10.1159/issn.1662-6575

Title: Case Reports in Oncology

Publisher: S. Karger AG

ISSN (Electronic): 1662-6575

Publication date Subscription-year: 2020

Publication date Collection: May – August 2020

Publication date (Electronic): 16 July 2020

Volume: 13

Issue: 2

Pages: 857-862

Affiliations

[_a] _aDepartment of Hematology and Oncology, Henry Ford Health System, Detroit, Michigan, USA

[_b] _bDepartment of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, Michigan, USA

Author notes

*Dhiraj Kumar Yadav, Department of Hematology and Oncology, Henry Ford Health System, 5713 Reveton Rd, West Bloomfield, Detroit, MI 48322 (USA), yadav_dhiraj@hotmail.com

Article

Publisher ID: 508361 Pmcid ID: PMC7443646 Other ID: Case Rep Oncol 2020;13:857–862

DOI: 10.1159/000508361

PMC ID: PMC7443646

PubMed ID: 32884531

SO-VID: ae0c0da5-464c-4e04-8893-ca8eeb065fcf

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This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

History

Date received : 29 April 2020

Date accepted : 30 April 2020

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Figures: 4, Pages: 6

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Pure Erythroid Leukemia in a Sickle Cell Patient Treated with Hydroxyurea

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Karger: Oncology

Most cited references 17

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).

More than 1 TP53 abnormality is a dominant characteristic of pure erythroid leukemia.

Acute myeloid leukemia and myelodysplastic syndromes following essential thrombocythemia treated with hydroxyurea: high proportion of cases with 17p deletion.

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