Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis

During progression of myeloid neoplasms, the basophil compartment may expand substantially and in some of these patients, a basophilic leukemia is diagnosed. In patients with Ph-chromosome+ chronic myeloid leukemia, acceleration of disease is typically accompanied by marked basophilia. In other myel...

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Autores principales:	Daniela Berger, Karin Bauer, Christoph Kornauth, Susanne Gamperl, Gabriele Stefanzl, Dubravka Smiljkovic, Christian Sillaber, Peter Bettelheim, Paul Knöbl, Ana-Iris Schiefer, Georg Greiner, Renate Thalhammer, Gregor Hoermann, Ilse Schwarzinger, Philipp B. Staber, Wolfgang R. Sperr, Peter Valent
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Basophils Basophilic leukemia IgE receptor Azacitidine Venetoclax Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/40211ff7524d46379c4fa4bdb27a7c5a
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spelling	oai:doaj.org-article:40211ff7524d46379c4fa4bdb27a7c5a2021-11-30T04:14:34ZSecondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis1476-558610.1016/j.neo.2021.09.010https://doaj.org/article/40211ff7524d46379c4fa4bdb27a7c5a2021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S1476558621000853https://doaj.org/toc/1476-5586During progression of myeloid neoplasms, the basophil compartment may expand substantially and in some of these patients, a basophilic leukemia is diagnosed. In patients with Ph-chromosome+ chronic myeloid leukemia, acceleration of disease is typically accompanied by marked basophilia. In other myeloid neoplasms, secondary leukemic expansion of basophils is rarely seen. We report on 5 patients who suffered from a myelodysplastic syndrome, myeloproliferative neoplasm, or acute leukemia and developed a massive expansion of basophils during disease progression. In 4 of 5 patients, peripheral blood basophil counts reached 40%, and the diagnosis “secondary basophilic leukemia” was established. As assessed by flow cytometry, neoplastic basophils expressed CD9, CD18, CD25, CD33, CD63, PD-L1, CD123, and CLL-1. In addition, basophils were found to display BB1 (basogranulin), 2D7, tryptase and KIT. In 4 of 5 patients the disease progressed quickly and treatment with azacitidine was started. However, azacitidine did not induce major clinical responses, and all patients died from progressive disease within 3 Y. In in vitro experiments, the patients´ cells and the basophilic leukemia cell line KU812 showed variable responses to targeted drugs, including azacitidine, venetoclax, hydroxyurea, and cytarabine. A combination of venetoclax and azacitidine induced cooperative antineoplastic effects in these cells. Together, secondary basophilic leukemia has a poor prognosis and monotherapy with azacitidine is not sufficient to keep the disease under control for longer time-periods. Whether drug combination, such as venetoclax+azacitidine, can induce better outcomes in these patients remains to be determined in future clinical studies.Daniela BergerKarin BauerChristoph KornauthSusanne GamperlGabriele StefanzlDubravka SmiljkovicChristian SillaberPeter BettelheimPaul KnöblAna-Iris SchieferGeorg GreinerRenate ThalhammerGregor HoermannIlse SchwarzingerPhilipp B. StaberWolfgang R. SperrPeter ValentElsevierarticleBasophilsBasophilic leukemiaIgE receptorAzacitidineVenetoclaxNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENNeoplasia: An International Journal for Oncology Research, Vol 23, Iss 12, Pp 1183-1191 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Basophils Basophilic leukemia IgE receptor Azacitidine Venetoclax Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	Basophils Basophilic leukemia IgE receptor Azacitidine Venetoclax Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Daniela Berger Karin Bauer Christoph Kornauth Susanne Gamperl Gabriele Stefanzl Dubravka Smiljkovic Christian Sillaber Peter Bettelheim Paul Knöbl Ana-Iris Schiefer Georg Greiner Renate Thalhammer Gregor Hoermann Ilse Schwarzinger Philipp B. Staber Wolfgang R. Sperr Peter Valent Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis
description	During progression of myeloid neoplasms, the basophil compartment may expand substantially and in some of these patients, a basophilic leukemia is diagnosed. In patients with Ph-chromosome+ chronic myeloid leukemia, acceleration of disease is typically accompanied by marked basophilia. In other myeloid neoplasms, secondary leukemic expansion of basophils is rarely seen. We report on 5 patients who suffered from a myelodysplastic syndrome, myeloproliferative neoplasm, or acute leukemia and developed a massive expansion of basophils during disease progression. In 4 of 5 patients, peripheral blood basophil counts reached 40%, and the diagnosis “secondary basophilic leukemia” was established. As assessed by flow cytometry, neoplastic basophils expressed CD9, CD18, CD25, CD33, CD63, PD-L1, CD123, and CLL-1. In addition, basophils were found to display BB1 (basogranulin), 2D7, tryptase and KIT. In 4 of 5 patients the disease progressed quickly and treatment with azacitidine was started. However, azacitidine did not induce major clinical responses, and all patients died from progressive disease within 3 Y. In in vitro experiments, the patients´ cells and the basophilic leukemia cell line KU812 showed variable responses to targeted drugs, including azacitidine, venetoclax, hydroxyurea, and cytarabine. A combination of venetoclax and azacitidine induced cooperative antineoplastic effects in these cells. Together, secondary basophilic leukemia has a poor prognosis and monotherapy with azacitidine is not sufficient to keep the disease under control for longer time-periods. Whether drug combination, such as venetoclax+azacitidine, can induce better outcomes in these patients remains to be determined in future clinical studies.
format	article
author	Daniela Berger Karin Bauer Christoph Kornauth Susanne Gamperl Gabriele Stefanzl Dubravka Smiljkovic Christian Sillaber Peter Bettelheim Paul Knöbl Ana-Iris Schiefer Georg Greiner Renate Thalhammer Gregor Hoermann Ilse Schwarzinger Philipp B. Staber Wolfgang R. Sperr Peter Valent
author_facet	Daniela Berger Karin Bauer Christoph Kornauth Susanne Gamperl Gabriele Stefanzl Dubravka Smiljkovic Christian Sillaber Peter Bettelheim Paul Knöbl Ana-Iris Schiefer Georg Greiner Renate Thalhammer Gregor Hoermann Ilse Schwarzinger Philipp B. Staber Wolfgang R. Sperr Peter Valent
author_sort	Daniela Berger
title	Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis
title_short	Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis
title_full	Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis
title_fullStr	Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis
title_full_unstemmed	Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis
title_sort	secondary basophilic leukemia in ph-negative myeloid neoplasms: a distinct subset with poor prognosis
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/40211ff7524d46379c4fa4bdb27a7c5a
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Secondary basophilic leukemia in Ph-negative myeloid neoplasms: A distinct subset with poor prognosis

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