Myeloproliferative (Exam 3) Flashcards
Antineoplastics that can cause MPD
Alkylating agents
5q- Sydrome
MDS entity predominate in females, median age 68, macrocytic anemia, leukpenia, normal or increased platelets. Better prognosis. Can become transfusion dependent. Treated with Lenalidomide.
Lenalidomide
Treats 5q- Syndrome, more potent version of thalidomide. Need an anticoagulant.
CHronic myelomonocytic leukemia (CMML)
Increased monocytes in BM and PS, variable degrees of dysplasia. Can respond to Imatinib, otherwise use Azacytidine
Azacytidine
Used to treat CMML and MDS in general. Can take 4 to 6 cycles. Prolongs shift to leukemia.
Hypocellular MDS
15-20% of MDS, same symptoms, clinically resembles aplastic anemia. Treated with ATG+cyclosporin.
Typical treatment of MDS
Growth factor (70% response rate), EPO if levels below 500. ATG + cyclosporin.
MPS Classic disorders
CHronic myeloid leukemia, polycythemia vera, essential thrombocytosis, primary myelofibrosis.
Genetic defects associated with MPS
JAK2 mutation(PCV), BCR-ABL(CML)
CML symptoms
Fatigue, nightsweats, weight loss, splenomegaly, anemia, platelet dysfunction.
CML labs
Hyperleukocytosis (Above 100k). Neutrophilia nad immature circulating myeloid cells. Absolute basophilia. Eosinophilia is common. Elevated LDH and uric acid.
CML treatment
Imatinib 400mg daily, nilotinib 300mg BID, or dasatinib 100mg daily.
Polycythemia Vera
RBC elevation in the absence of secondary conditions. Can have an elevated WBC and platelets. Male predominat 60-65 median onset. JAK2
PCV symptoms/signs
Itching, thrombosis, gi complaints, headaches, dizziness, vision changes, Hypertension splenomegaly, hepatomegaly, cuntaneous ulcers, gouty features, pulmonary hypotension
PCV diagnosis
Hb higher than 16.5 in men (16 in women) OR Hct about 49% OR increased red cell mass AND panmyelosis with prominent erythroids AND JAK2 mutation. Subnormal EPO.
Essential Thrombocytosis
Median age 60 years, more common in women, JAK2 in 40-50%.
ETC Clinical features
Vasomotor, thjrombotic, hemorrhagic, and 1st semester spontaneous abortion.
ETC diagnosis
platelet count about 450X10^9/L AND Bone marrow biopsy with proliferation in megakaryocytes AND not meeting criteria for other disorders AND JAK2 OR clonal marker
ETC treatment
Hydrea: High risk patients should receive low dose ASA and hydrea.
Anagrelide: second line
INFa: Used in women who are pregnant or may become pregnant
Primary myelofibrosis
Worst prognosis of all MPS, median survival 3.5 to 5.5 years. Median age 65. Marrow fibrosis and extramedullary hematopoeisis. Treatment mainly palliative.
