<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Myelodysplastic syndromes are a heterogeneous group of clonal disorders characterized by peripheral blood cytopenia and normal or hypercellular bone marrow with dysplasia in more than one blood cell lineage, unfavorable prognosis, and lack of response to treatment. We present the case of a 12-year-old male patient who was referred to the Hematology and Oncology Department of Pediatric Clinic I Târgu- Mures in May 2016, with splenomegaly and pancytopenia. The osteomedullary biopsy revealed myelofibrosis, discrete dysplasia of the myeloid series and megakaryocytes, blasts CD34+ approximately 10%, which led to the diagnosis of myelodysplastic syndrome with myelofibrosis. The myeloid precursors indicated a high risk of transformation into acute myeloid leukemia, so chemotherapy associated with corticosteroids was started, leading to slight improvements. <inline-graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="graphic/7607_rrlm_45.jpg"></inline-graphic> Although myelodysplastic syndrome associated with myelofibrosis is rare at this age, despite the treatment and favorable progression in the case presented, in the absence of hematopoietic stem cell transplantation the prognosis remains unfavorable.</p></abstract>

Myelodysplastic syndromes are a heterogeneous group of clonal disorders characterized by peripheral blood cytopenia and normal or hypercellular bone marrow with dysplasia in more than one blood cell lineage, unfavorable prognosis, and lack of response to treatment. We present the case of a 12-year-old male patient who was referred to the Hematology and Oncology Department of Pediatric Clinic I Târgu- Mures in May 2016, with splenomegaly and pancytopenia. The osteomedullary biopsy revealed myelofibrosis, discrete dysplasia of the myeloid series and megakaryocytes, blasts CD34+ approximately 10%, which led to the diagnosis of myelodysplastic syndrome with myelofibrosis. The myeloid precursors indicated a high risk of transformation into acute myeloid leukemia, so chemotherapy associated with corticosteroids was started, leading to slight improvements.  Although myelodysplastic syndrome associated with myelofibrosis is rare at this age, despite the treatment and favorable progression in the case presented, in the absence of hematopoietic stem cell transplantation the prognosis remains unfavorable.

Myelodysplastic syndrome with myelofibrosis in a 12-year-old patient – A case report

Revista Romana de Medicina de Laborator

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Myelodysplastic syndromes are a heterogeneous group of clonal disorders characterized by peripheral blood cytopenia and normal or hypercellular bone marrow...

Myelodysplastic syndrome with myelofibrosis in a 12-year-old patient – A case report

Online veröffentlicht: 30. Jan. 2018

Seitenbereich: 95 - 103

Eingereicht: 06. Juli 2017

Akzeptiert: 18. Okt. 2017

DOI: https://doi.org/10.1515/rrlm-2017-0034

Schlüsselwörter
myelodysplastic syndrome, pancytopenia, myelofibrosis, children

© by Mihaela Ioana Chincesan

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Myelodysplastic syndrome with myelofibrosis in a 12-year-old patient – A case report

Online veröffentlicht: 30. Jan. 2018

Seitenbereich: 95 - 103

Eingereicht: 06. Juli 2017

Akzeptiert: 18. Okt. 2017

DOI: https://doi.org/10.1515/rrlm-2017-0034

Schlüsselwörtermyelodysplastic syndrome, pancytopenia, myelofibrosis, children

© by Mihaela Ioana Chincesan

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Schlüsselwörter
myelodysplastic syndrome, pancytopenia, myelofibrosis, children