[FIP1L1-PDGFRА-positive myeloproliferative disease with eosinophilia: A rare case with multiple organ dysfunction and a response to tyrosine kinase inhibitor therapy]

Ter Arkh. 2015;87(12):89-95. doi: 10.17116/terarkh2015871289-95.
[Article in Russian]

Abstract

The described case of FIP1L1-PDGFRА-positive myeloproliferative disease is characterized by an atypical aggressive course to develop severe specific complications as injuries to the brain, heart, lung, and intestine. Pathogenetic therapy with imatinib could stabilize a patient's state, but failed to produce a complete hematological response. Switching from imatinib to dasatinib could produce sustained clinical, hematological, and molecular remissions.

Представленный случай позитивного по FIP1L1-PDGFRA миелопролиферативного заболевания отличался нетипичным агрессивным течением с развитием тяжелых специфических осложнений в виде поражений головного мозга, сердца, легких, кишечника. Патогенетическая терапия иматинибом позволила стабилизировать состояние больной, но полный гематологический ответ не получен. После замены иматиниба дазатинибом достигнута стойкая клинико-гематологическая и молекулярная ремиссия.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Dasatinib / administration & dosage
  • Dasatinib / pharmacology
  • Eosinophilia / drug therapy*
  • Female
  • Humans
  • Imatinib Mesylate / administration & dosage
  • Imatinib Mesylate / pharmacology
  • Multiple Organ Failure / drug therapy*
  • Myeloproliferative Disorders / drug therapy*
  • Myeloproliferative Disorders / genetics
  • Oncogene Proteins, Fusion*
  • Protein Kinase Inhibitors / administration & dosage
  • Protein Kinase Inhibitors / pharmacology*
  • Protein-Tyrosine Kinases / antagonists & inhibitors*
  • Receptor, Platelet-Derived Growth Factor alpha*
  • mRNA Cleavage and Polyadenylation Factors*

Substances

  • Oncogene Proteins, Fusion
  • Protein Kinase Inhibitors
  • mRNA Cleavage and Polyadenylation Factors
  • Imatinib Mesylate
  • FIP1L1-PDGFRA fusion protein, human
  • Protein-Tyrosine Kinases
  • Receptor, Platelet-Derived Growth Factor alpha
  • Dasatinib