administration of recombinant erythropoietin may be beneficial
androgens - e.g. oxymetholone, nandrolone or danazol - to reduce transfusion requirement; but poorly tolerated by women
for splenomegaly
therapy should be considered only in patients who are truly symptomatic (1)
hydroxyurea, - most commonly
most commonly used initial medical therapy in splnomegaly
to reduce hypermetabolism, leucocytosis and thrombocytosis
adverse effects include myelosuppression which may exacerbate the underlying anaemia (1)
alkylating agents - busulphan, melphalan or chlorambucil
can be used in some patients with splenomegaly although there is a risk of potential myelosuppression and increased risk of eventual blastic transformation (1)
for both anaemia and splenomegaly
agents which improves splenomegaly do so by non specific myelosuppression effects
immunomodulatory drugs (IMiDs) have shown that it has the ability to improve both the conditions e.g. - thalidomide, lenalidomide and pomalidomide (investigational agent) (1)
splenectomy is indicated if:
the spleen becomes very large and painful
transfusion requirement are high
thrombocytopenia is severe
newer drugs
immunomodulatory drugs (IMiDs)
hypomethylating agents - azacitidine and decitabine
JAK2 Inhibitors (1,2)
the identification of driver mutations in JAK2, CALR, and MPL has contributed to a better understanding of disease pathogenesis, implicating near-universal upregulation of JAK-STAT signaling, and has led to the development of the sole targeted therapy for MF, the JAK2 inhibitor ruxolitinib
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