TY - JOUR
T1 - Treatment options for hydroxyurea-refractory disease complications in myeloproliferative neoplasms
T2 - JAK2 inhibitors, radiotherapy, splenectomy and transjugular intrahepatic portosystemic shunt
AU - Mishchenko, Elena
AU - Tefferi, Ayalew
PY - 2010/9
Y1 - 2010/9
N2 - Clinical care of patients with polycythemia vera, essential thrombocythemia and myelofibrosis (MF) requires not only a broad understanding of general treatment principles but also familiarity with the management of hydroxyurea-refractory disease complications. The latter include progressive splenomegaly, symptomatic portal hypertension (e.g. ascites, variceal bleeding), pulmonary hypertension, bone pain, intractable pruritus, constitutional symptoms (e.g. fatigue, night sweats) and cachexia (i.e. loss of lean body mass, general ill health, poor appetite). Some of these symptoms are directly or indirectly related to extramedullary hematopoiesis (EMH) and others to proinflammatory cytokine excess. Results from recent clinical trials of JAK inhibitors suggest remarkable activity in MF-associated constitutional symptoms, cachexia, pruritus and hydroxyurea-refractory splenomegaly. Involved-field radiotherapy is best utilized in the setting of EMH-associated symptoms, including ascites, bone (extremity) pain and pulmonary hypertension. Splenectomy is indicated in the presence of drug-refractory splenomegaly and frequent red cell transfusion requirement. Transjugular intrahepatic portosystemic shunt is used to alleviate symptoms of portal hypertension.
AB - Clinical care of patients with polycythemia vera, essential thrombocythemia and myelofibrosis (MF) requires not only a broad understanding of general treatment principles but also familiarity with the management of hydroxyurea-refractory disease complications. The latter include progressive splenomegaly, symptomatic portal hypertension (e.g. ascites, variceal bleeding), pulmonary hypertension, bone pain, intractable pruritus, constitutional symptoms (e.g. fatigue, night sweats) and cachexia (i.e. loss of lean body mass, general ill health, poor appetite). Some of these symptoms are directly or indirectly related to extramedullary hematopoiesis (EMH) and others to proinflammatory cytokine excess. Results from recent clinical trials of JAK inhibitors suggest remarkable activity in MF-associated constitutional symptoms, cachexia, pruritus and hydroxyurea-refractory splenomegaly. Involved-field radiotherapy is best utilized in the setting of EMH-associated symptoms, including ascites, bone (extremity) pain and pulmonary hypertension. Splenectomy is indicated in the presence of drug-refractory splenomegaly and frequent red cell transfusion requirement. Transjugular intrahepatic portosystemic shunt is used to alleviate symptoms of portal hypertension.
KW - JAK2
KW - extramedullary hematopoiesis
KW - radiation
KW - splenectomy
KW - transjugular intrahepatic portosystemic shunt
KW - treatment
UR - http://www.scopus.com/inward/record.url?scp=77955828956&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77955828956&partnerID=8YFLogxK
U2 - 10.1111/j.1600-0609.2010.01480.x
DO - 10.1111/j.1600-0609.2010.01480.x
M3 - Review article
C2 - 20528907
AN - SCOPUS:77955828956
SN - 0902-4441
VL - 85
SP - 192
EP - 199
JO - European Journal of Haematology
JF - European Journal of Haematology
IS - 3
ER -