Details

Autor(en) / Beteiligte

• Berger, Daniela
• Bauer, Karin
• Kornauth, Christoph
• Gamperl, Susanne
• Stefanzl, Gabriele
• Smiljkovic, Dubravka
• Sillaber, Christian
• Bettelheim, Peter
• Knöbl, Paul
• Schiefer, Ana-Iris
• Greiner, Georg
• Thalhammer, Renate
• Hoermann, Gregor
• Schwarzinger, Ilse
• Staber, Philipp B.
• Sperr, Wolfgang R.
• Valent, Peter

Titel

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

Ist Teil von

• Neoplasia (New York, N.Y.), 2021-12, Vol.23 (12), p.1183-1191

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2021

Quelle

MEDLINE

Beschreibungen/Notizen

• 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.