An Intriguing Case of Eosinophilia with FIP1L1/PDGFRA Rearrangement Who Presented as Thrombotic Thrombocytopenic Purpura

Myeloid neoplasm with eosinophilia and FIP1-like-1-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFRA) rearrangement is a multi-organ disease with diverse clinical presentation. Thrombotic thrombocytopenic purpura (TTP) is characterized by the concomitant occurrence of often severe thrombo...

Full description

Saved in:
Bibliographic Details
Published in:Case reports in hematology 2019-10, Vol.2019 (2019), p.1-6
Main Authors: AlSwayyed, Azizah, AlSughayyer, Ammar, Zaidi, Syed Z. A., Zaidi, Abdul Rehman Z., Alfayez, Mansour, Alshehry, Nawal, Tailor, Imran, Motabi, Ibrahim, Alghamdi, Mubarak, Alnomani, Mohammad, Alshehri, Hassan, Altaf, Syed
Format: Article
Language:English
Subjects:
Anemia
Blood diseases
Bone marrow
Case Report
Case reports
Eosinophilia
Hemoglobin
Ischemia
Laboratories
Monoclonal antibodies
Platelet-derived growth factor
Thrombocytopenic purpura
Tumors
Vincristine
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Myeloid neoplasm with eosinophilia and FIP1-like-1-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFRA) rearrangement is a multi-organ disease with diverse clinical presentation. Thrombotic thrombocytopenic purpura (TTP) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic organ damage. To our knowledge, only one case of eosinophilia with FIP1L1-PDGFRA rearrangement presented as a case of thrombotic thrombocytopenic purpura reported in the literature. We herein report a case of a young male patient with hypereosinophilic syndrome and FIP1L1-PDGFRA rearrangement who presented with asthma, transient ischemic attacks (TIA), and confusion. He had an acquired TTP that was successfully treated with plasma exchanges (PLEX), corticosteroids, rituximab, and later with the addition of imatinib mesylate (Gleevec, Novartis). He remains in complete remission on imatinib 100 mg daily for more than 28 months of follow-up.
ISSN:2090-6560
2090-6579
DOI:10.1155/2019/2820954