Bone Marrow Disorders Flashcards
(21 cards)
What is Myelofibrosis?
Disease of older patients with a pancytopenia associated with fibrosis of bone marrow
Where does blood production shift to in Myelofibrosis?
The spleen and liver: resulting in hepatosplenomegaly
How do you test for Myelofibrosis?
Blood smear: showing teardrop-shaped cells and nucleated RBCs
What is seen on blood smear in Myeofibrosis?
Teardrop-shaped cells and nulceated RBC
How do you treat Myelofibrosis?
TNF-Inhibitors to increase Bone marrow production
Allogenic Bone marrow transplant if age <50
Ruxolitinib
What are commonly used TNF-Inhibitors in Myelofibrosis Treatment?
Thalidomide and Lenalidomide
How does Ruxolitinib work in treating Myelofibrosis?
Inhibits JAK2 and suppress myelofibrosis
What is another name for Myelodysplasia?
Myelodysplastic Syndrome (MDS)
What is Myelodysplasia (myelodysplastic syndrome)?
Preleukemic disorder in older patients >60 with a pancytopenia despite a hypercellular bone marrow
What genetic change is associated with Myelodysplasia (Myelodysplastic syndrome)?
5q deletion: patients with 5q have better prognosis than those wihout
What is a key clinical clue to Myelodisplasia?
Prior chemotherapy or radiation in a patient with pancytopenia
Why don’t most patients with Myelodysplasia ever develop AML?
They die of infection or bleeding beforehand
How does Myelodysplasia present?
many are asymptomatic with pancytopenia on CBC
What are possible symptoms seen in Myelodysplasia?
Fatigue and weight loss
Infection
Bleeding
Splenomegaly
What is the most distinct lab abnormality in Myelodysplasia?
Pelger-Huet Cells
How do you diagnose Myelodysplasia?
CBC, Marrow and Prussian Blue stain
What is seen on CBC in Myelodysplasia?
Anemia: Increased MCV, Nucleated RBC and small # of blasts
What is seen in marrow in Myelodysplasia?
Hypercellular marrow
What is seen on Prussian-blue stain in myelodysplasia?
Ringed Sideroblasts
What are the treatment options in Myelodysplasia?
Transfusion
Erythropoietin (EPO)
Azacitidine or Decitabine
Lenalidomide for 5q deletion: decreases transfusion dependence
Luspatercept: Growth Factor: decreases transfusion dependence
Bone marrow transplant for patients <50
What is the role of Azacitidine in treating Myelodysplasia?
Decreases transfusion dependence and increases survival
