Biphenotypic hematologic malignancy: a case report of the 8p11 myeloproliferative syndrome in a child.
The 8p11 myeloproliferative syndrome, also known as stem cell leukemia/lymphoma, is a rare, atypical, myeloproliferative disorder and lymphoid malignancy associated with chromosomal abnormalities involving the 8p11 chromosomal band. Translocations associated with this syndrome result in the fusion of the fibroblast growth factor receptor 1 (FGFR 1) gene with various partners, resulting in ligand-independent FGFR activity. To date, 8 partner genes have been identified in association with FGFR1 rearrangements. The most frequent FGFR1 translocation partner is the zinc finger gene ZNF198 located at 13q11. Disease phenotypes associated with this translocation include poor prognosis and transformation to acute leukemia and non-Hodgkin lymphoma. In common with a T-cell phenotype, obtaining and maintaining remission is difficult by conventional chemotherapy. This study describes an illustrative case of 8p11 myeloproliferative syndrome/stem cell leukemia/lymphoma outlining its chief features and historical developments.
Chen
Xiangli
X
Department of Clinical Hematology, Henan Provincial People's Hospital, First Hospital of Zhengzhou University, Zhengzhou, Henan Province, China.
Zhang
Yin
Y
Li
Yulong
Y
Lei
Pingchong
P
Zhai
Yaping
Y
Liu
Linxiang
L
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics*; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D006801:Humans; D008297:Male; D009196:Myeloproliferative Disorders / Q000151:congenital / Q000235:genetics* / Q000503:physiopathology; D010641:Phenotype; D011230:Precancerous Conditions / Q000151:congenital / Q000235:genetics*; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D011241:Prednisone / Q000627:therapeutic use; D013577:Syndrome; D014178:Translocation, Genetic; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D005047:Etoposide; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone