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FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()

Mast cell leukemia (MCL) is a life-threatening disease associated with high mortality and drug-resistance. Only few patients survive more than 12 months. We report on a 55-year-old female patient with acute MCL diagnosed in May 2012. The disease was characterized by a rapid increase in white blood c...

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Autores principales: Valent, Peter, Blatt, Katharina, Eisenwort, Gregor, Herrmann, Harald, Cerny-Reiterer, Sabine, Thalhammer, Renate, Müllauer, Leonhard, Hoermann, Gregor, Sadovnik, Irina, Schwarzinger, Ilse, Sperr, Wolfgang R., Mannhalter, Christine, Horny, Hans-Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3939382/
https://www.ncbi.nlm.nih.gov/pubmed/24596674
http://dx.doi.org/10.1016/j.lrr.2013.11.001
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author Valent, Peter
Blatt, Katharina
Eisenwort, Gregor
Herrmann, Harald
Cerny-Reiterer, Sabine
Thalhammer, Renate
Müllauer, Leonhard
Hoermann, Gregor
Sadovnik, Irina
Schwarzinger, Ilse
Sperr, Wolfgang R.
Mannhalter, Christine
Horny, Hans-Peter
author_facet Valent, Peter
Blatt, Katharina
Eisenwort, Gregor
Herrmann, Harald
Cerny-Reiterer, Sabine
Thalhammer, Renate
Müllauer, Leonhard
Hoermann, Gregor
Sadovnik, Irina
Schwarzinger, Ilse
Sperr, Wolfgang R.
Mannhalter, Christine
Horny, Hans-Peter
author_sort Valent, Peter
collection PubMed
description Mast cell leukemia (MCL) is a life-threatening disease associated with high mortality and drug-resistance. Only few patients survive more than 12 months. We report on a 55-year-old female patient with acute MCL diagnosed in May 2012. The disease was characterized by a rapid increase in white blood cells and mast cells (MC) in the peripheral blood, and a rapid increase of serum tryptase levels. The KIT D816H mutation was detected in the blood and bone marrow (BM). Induction chemotherapy with high-dose ARA-C and fludarabine (FLAG) was administered. Unexpectedly, the patient entered a hematologic remission with almost complete disappearance of neoplastic MC and a decrease of serum tryptase levels to normal range after 2 cycles of FLAG. Consecutively, the patient was prepared for allogeneic stem cell transplantation. However, shortly after the third cycle of FLAG, tryptase levels increased again, immature MC appeared in the blood, and the patient died from cerebral bleeding. Together, this case shows that intensive chemotherapy regimens, like FLAG, may induce remission in acute MCL. However, treatment responses are short-lived and the overall outcome remains dismal in these patients. We propose to separate this acute type of MCL from more subacute or chronic variants of MCL.
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spelling pubmed-39393822014-03-04 FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H() Valent, Peter Blatt, Katharina Eisenwort, Gregor Herrmann, Harald Cerny-Reiterer, Sabine Thalhammer, Renate Müllauer, Leonhard Hoermann, Gregor Sadovnik, Irina Schwarzinger, Ilse Sperr, Wolfgang R. Mannhalter, Christine Horny, Hans-Peter Leuk Res Rep Case Report Mast cell leukemia (MCL) is a life-threatening disease associated with high mortality and drug-resistance. Only few patients survive more than 12 months. We report on a 55-year-old female patient with acute MCL diagnosed in May 2012. The disease was characterized by a rapid increase in white blood cells and mast cells (MC) in the peripheral blood, and a rapid increase of serum tryptase levels. The KIT D816H mutation was detected in the blood and bone marrow (BM). Induction chemotherapy with high-dose ARA-C and fludarabine (FLAG) was administered. Unexpectedly, the patient entered a hematologic remission with almost complete disappearance of neoplastic MC and a decrease of serum tryptase levels to normal range after 2 cycles of FLAG. Consecutively, the patient was prepared for allogeneic stem cell transplantation. However, shortly after the third cycle of FLAG, tryptase levels increased again, immature MC appeared in the blood, and the patient died from cerebral bleeding. Together, this case shows that intensive chemotherapy regimens, like FLAG, may induce remission in acute MCL. However, treatment responses are short-lived and the overall outcome remains dismal in these patients. We propose to separate this acute type of MCL from more subacute or chronic variants of MCL. Elsevier 2013-11-26 /pmc/articles/PMC3939382/ /pubmed/24596674 http://dx.doi.org/10.1016/j.lrr.2013.11.001 Text en © 2013 The Authors http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-No Derivative Works License, which permits non-commercial use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Case Report
Valent, Peter
Blatt, Katharina
Eisenwort, Gregor
Herrmann, Harald
Cerny-Reiterer, Sabine
Thalhammer, Renate
Müllauer, Leonhard
Hoermann, Gregor
Sadovnik, Irina
Schwarzinger, Ilse
Sperr, Wolfgang R.
Mannhalter, Christine
Horny, Hans-Peter
FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()
title FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()
title_full FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()
title_fullStr FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()
title_full_unstemmed FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()
title_short FLAG-induced remission in a patient with acute mast cell leukemia (MCL) exhibiting t(7;10)(q22;q26) and KIT D816H()
title_sort flag-induced remission in a patient with acute mast cell leukemia (mcl) exhibiting t(7;10)(q22;q26) and kit d816h()
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3939382/
https://www.ncbi.nlm.nih.gov/pubmed/24596674
http://dx.doi.org/10.1016/j.lrr.2013.11.001
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