Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy

This is the report of the clinical case of a patient who presents the association of a JAK-2 positive chronic myeloproliferative neoplasia to a subsequent 5q- myelodysplastic syndrome, developed after about 14 years from the first diagnosis. Patient’s symptoms had rapidly worsened, and she became tr...

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Main Authors: Antonella Vaccarino, Irene Dogliotti, Fabio Marletto, Andrea Demarchi, Mario Bazzan
Format: Article
Language:English
Published: PAGEPress Publications 2016-10-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
Subjects:
Online Access:http://www.mjhid.org/index.php/mjhid/article/view/2622
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spelling doaj-a32bf44538ca4fac89b05b81058a88892020-11-24T23:02:30ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062016-10-0180e2016050e201605010.4084/mjhid.2016.0501644Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapyAntonella Vaccarino0Irene Dogliotti1Fabio Marletto2Andrea Demarchi3Mario Bazzan4Rare Diseases, Immunology, Immunohaematology and Haematology Department, Turin, ItalyUniversity of Turin, ItalyRare Diseases, Immunology, Immunohaematology and Haematology DepartmentPathology Unit, San Giovanni Bosco Hospital, Turin, ItalyRare Diseases, Immunology, Immunohaematology and Haematology Department, Turin, ItalyThis is the report of the clinical case of a patient who presents the association of a JAK-2 positive chronic myeloproliferative neoplasia to a subsequent 5q- myelodysplastic syndrome, developed after about 14 years from the first diagnosis. Patient’s symptoms had rapidly worsened, and she became transfusion-dependent. Therapy with low-dose Lenalidomide quickly reduced the splenomegaly, and completely brought white cells counts, haemoglobin and platelets back to normal.  After more than one year from start, blood cell count is still normal. As far as we know this is the first case of an effective treatment with Lenalidomide reported in this clinical setting.http://www.mjhid.org/index.php/mjhid/article/view/2622myeloproliferative syndrome, myelodysplastic syndrome, 5q deletion, JAK-2 mutation, lenalidomide
collection DOAJ
language English
format Article
sources DOAJ
author Antonella Vaccarino
Irene Dogliotti
Fabio Marletto
Andrea Demarchi
Mario Bazzan
spellingShingle Antonella Vaccarino
Irene Dogliotti
Fabio Marletto
Andrea Demarchi
Mario Bazzan
Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy
Mediterranean Journal of Hematology and Infectious Diseases
myeloproliferative syndrome, myelodysplastic syndrome, 5q deletion, JAK-2 mutation, lenalidomide
author_facet Antonella Vaccarino
Irene Dogliotti
Fabio Marletto
Andrea Demarchi
Mario Bazzan
author_sort Antonella Vaccarino
title Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy
title_short Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy
title_full Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy
title_fullStr Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy
title_full_unstemmed Diagnosis of del(5q) MDS, 14 years after JAK-2 positive PV appearance: complete remission of both diseases with lenalidomide monotherapy
title_sort diagnosis of del(5q) mds, 14 years after jak-2 positive pv appearance: complete remission of both diseases with lenalidomide monotherapy
publisher PAGEPress Publications
series Mediterranean Journal of Hematology and Infectious Diseases
issn 2035-3006
publishDate 2016-10-01
description This is the report of the clinical case of a patient who presents the association of a JAK-2 positive chronic myeloproliferative neoplasia to a subsequent 5q- myelodysplastic syndrome, developed after about 14 years from the first diagnosis. Patient’s symptoms had rapidly worsened, and she became transfusion-dependent. Therapy with low-dose Lenalidomide quickly reduced the splenomegaly, and completely brought white cells counts, haemoglobin and platelets back to normal.  After more than one year from start, blood cell count is still normal. As far as we know this is the first case of an effective treatment with Lenalidomide reported in this clinical setting.
topic myeloproliferative syndrome, myelodysplastic syndrome, 5q deletion, JAK-2 mutation, lenalidomide
url http://www.mjhid.org/index.php/mjhid/article/view/2622
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