Myelofibrosis Flashcards
Etiologies of secondary myelofibrosis
ET or PV
median survival
6 years (people can live for quite a while because it’s a slow process)
3 most commonly mutated genes in PMF
JAK2, CALR, MPL
signs/symptoms
Patient denies systemic symptoms (fatigue, fever, bone pain, night sweats, weight loss). Patient denies pruritus, shortness of breath (pHTN), joint tenderness or pain, abdominal pain.
1) Labs
2) Peripheral smear findings
cytopenias + peripheral smear with teardrop cells and leukoerythroblastosis (immature cells of granulocytic series and nucleated RBCs).
Goal of treatment
if not transplant candidate = palliative
Curative if transplant candidate
Treatment of low/intermediate risk + asymptomatic
*Active surveillance
IF MF-associated anemia → danazol or talidomine +/- prednisone or lenalidomine +/- prednisone
IF EPO low + no splenomegaly → EPO stimulating agents
Management of symptomatic splenomegaly
Cytoreduction with ruxolitinib (preferred over hydrea, which is mutogenic)
Management of intermediate 2 risk or high risk or symptomatic
allogeneic HSC transplant if candidate + ruxolitinib if constitutional symptoms
Ruxolitinib targets
JAK1
Pathophysiology of myelofibrosis
Its an MPN, so it’s
a clonal neoplastic disorder of hematopoiesis. This leads to excess cell productionProduction of cytokines such as fibroblast growth factor by the abnormal hematopoietic cell clone (particularly by megakaryocytes)[11] leads to replacement of the hematopoietic tissue of the bone marrow by connective tissue via collagen fibrosis.
Ruxolitinib trade name
Jakafi
why splenomegaly occurs
Hematopoiesis moves to reticuloendothelial system
what does DIPSS look at?
constitutional symptoms, cell lines, age, peripheral blast count
scientific term for teardrop cell
dacrocyte
