PMID- 28885361
OWN - NLM
STAT- MEDLINE
DCOM- 20170918
LR  - 20220419
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 36
DP  - 2017 Sep
TI  - Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with 
      FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report.
PG  - e8001
LID - 10.1097/MD.0000000000008001 [doi]
LID - e8001
AB  - RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by 
      hypereosinophilia and organ damage. Some cases of HES are caused by the 
      FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES 
      occasionally evolves into chronic eosinophilic leukemia or into another form of 
      myeloproliferative neoplasm; however, the development of a malignant lymphoma is 
      very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) 
      and HES with the FIP1L1/PDGFRA gene rearrangement. PATIENT CONCERNS: A man in his 
      30s presented to our hospital with fever, hypereosinophilia, widespread 
      lymphadenopathy, and splenomegaly. Laboratory tests showed hypereosinophilia, 
      increased soluble interleukin-2 receptor, and increased vitamin B12. 
      Positron-emission tomography with F fluorodeoxyglucose (FDG) showed positive FDG 
      uptake in multiple enlarged lymph nodes throughout the body and the red bone 
      marrow. A bone-marrow biopsy showed hypereosinophilia without dysplasia and an 
      increased number of blasts. The FIP1L1/PDGFRA fusion gene was positive upon 
      fluorescence in situ hybridization (FISH) analysis of the peripheral blood. 
      Furthermore, biopsy of a lymph node from the neck revealed restiform hyperplasia 
      of capillary vessels, with small lymphoma cells arranged around the capillaries. 
      Lymphoma cells were positive for CD3, CD4, and CD10, and negative for CD20. 
      Lymphoma cells were also positive for the FIP1L1/PDGFRA fusion gene by FISH 
      analysis. DIAGNOSES: From these findings, the patient was diagnosed with HES and 
      AITL with FIP1L1/PDGFRA. INTERVENTIONS: After the diagnosis, corticosteroid was 
      administered but was ineffective. Imatinib was then administered. OUTCOMES: 
      Imatinib was very effective for treating HES and AITL, and complete remission was 
      achieved in both. LESSONS: This report presents the first case in which the 
      FIP1L1/PDGFRA fusion gene was positive both in peripheral blood and lymph nodes, 
      implying the possibility that the tumor cells acquired the FIP1L1/PDGFRA fusion 
      gene in the early stage of hematopoietic progenitor cell developments. Imatinib 
      was very effective in treating both HES and lymphoma, suggesting that the 
      FIP1L1/PDGFRA fusion gene plays a key role in the pathogenesis of both HES and 
      lymphoma.
FAU - Yamamoto, Masayo
AU  - Yamamoto M
AD  - Division of Gastroenterology and Hematology/Oncology, Department of Medicine 
      Oncology Center, Asahikawa Medical University Hospital, Asahikawa, Japan.
FAU - Ikuta, Katsuya
AU  - Ikuta K
FAU - Toki, Yasumichi
AU  - Toki Y
FAU - Hatayama, Mayumi
AU  - Hatayama M
FAU - Shindo, Motohiro
AU  - Shindo M
FAU - Torimoto, Yoshihiro
AU  - Torimoto Y
FAU - Okumura, Toshikatsu
AU  - Okumura T
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Antineoplastic Agents)
RN  - 0 (FIP1L1-RARA fusion protein, human)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 8A1O1M485B (Imatinib Mesylate)
SB  - IM
MH  - Adult
MH  - Antineoplastic Agents/*therapeutic use
MH  - Diagnosis, Differential
MH  - Humans
MH  - Hypereosinophilic Syndrome/*complications/drug therapy/genetics/pathology
MH  - Imatinib Mesylate/*therapeutic use
MH  - Lymphoma, T-Cell/*complications/drug therapy/genetics/pathology
MH  - Male
MH  - Oncogene Proteins, Fusion/*genetics
PMC - PMC6392760
COIS- The authors declare no conflicts of interest.
EDAT- 2017/09/09 06:00
MHDA- 2017/09/19 06:00
PMCR- 2017/09/08
CRDT- 2017/09/09 06:00
PHST- 2017/09/09 06:00 [entrez]
PHST- 2017/09/09 06:00 [pubmed]
PHST- 2017/09/19 06:00 [medline]
PHST- 2017/09/08 00:00 [pmc-release]
AID - 00005792-201709080-00041 [pii]
AID - MD-D-17-00741 [pii]
AID - 10.1097/MD.0000000000008001 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Sep;96(36):e8001. doi: 10.1097/MD.0000000000008001.