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A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of PDGFRB, t(4;5)(q21;q33)
Joint Authors
Zhang, Da
Cui, Wei
Persons, Diane
Brown, Laura E.
Yacoub, Abdulraheem
Ponnala, Shivani
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-08-25
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Various translocations involving the PDGFRB gene are identified in myeloid neoplasms.
However, the PRKG2/PDGFRB fusion gene associated with t(4;5)(q21;q33) has previously been reported in only 3 patients.
We present the case of a 26-year-old woman with microcytic anemia, basophilia, thrombocytosis, and massive splenomegaly, who was found to have systemic mastocytosis and associated clonal hematological non-mast cell lineage disease (SM-AHNMD), with myeloid neoplasm with PRKG2/PDGFRB rearrangement.
Initial findings included basophilia (37%, 4.1 k/μL), hypercellular marrow with eosinophilia, and increased and atypical megakaryocytes, suggestive of myeloproliferative neoplasm.
Additional studies revealed large clusters of CD25 positive mast cells, fulfilling the criteria for the diagnosis of systemic mastocytosis.
Consistent with prior reports of this translocation, our patient has responded well to imatinib.
This case, in conjunction with others in the literature, suggests a possible connection between t(4;5)(q21;q33) PRKG2/PDGFRB and systemic mastocytosis and highlights their favorable response to imatinib.
American Psychological Association (APA)
Brown, Laura E.& Zhang, Da& Persons, Diane& Yacoub, Abdulraheem& Ponnala, Shivani& Cui, Wei. 2016. A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of PDGFRB, t(4;5)(q21;q33). Case Reports in Hematology،Vol. 2016, no. 2016, pp.1-4.
https://search.emarefa.net/detail/BIM-1100851
Modern Language Association (MLA)
Brown, Laura E.…[et al.]. A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of PDGFRB, t(4;5)(q21;q33). Case Reports in Hematology No. 2016 (2016), pp.1-4.
https://search.emarefa.net/detail/BIM-1100851
American Medical Association (AMA)
Brown, Laura E.& Zhang, Da& Persons, Diane& Yacoub, Abdulraheem& Ponnala, Shivani& Cui, Wei. A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of PDGFRB, t(4;5)(q21;q33). Case Reports in Hematology. 2016. Vol. 2016, no. 2016, pp.1-4.
https://search.emarefa.net/detail/BIM-1100851
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1100851