Myeloproliferative Disorders Flashcards
complications common to all myeloproliferative disorders
hyperuricemia/gout (purine degradation)
bone marrow fibrosis
acute leukemia
neoplastic proliferation of mature myeloid cells, esp granulocytes
CML
what type of cell is characteristically increased in CML?
basophils
chromosomal abnormality that drives CML
t(9;22)
what is the consequence of the t(9;22)
BCR-ABL fusion protein –> increased tyrosine kinase activity (signal transducer)
first line of treatment for CML
imatinib (blocks tyrosine kinase activity)
marker of accelerated phase of CML, leading to transformation of acute leukemia
enlarging spleen
what is the worst possible complication of CML
transformation to AML or ALL (because mutation is in the hematopoietic stem cell)
3 characteristics of CML that can be used to distinguish it from leukemoid reaction (infection)
- LAP negative (LAP is used to fight infection)
- basophils
- t(9;22)
neoplastic proliferation of mature myeloid cells, esp RBCs
polycythemia vera (PV)
mutation associated with polycythemia vera
JAK2 kinase
myeloproliferative disorder that presents with blurry vision, headache, flushed face, itching after bathing
polycythemia vera
treatment for polycythemia vera
phlebotomy
second line: hydroxyuria
features that distinguish PV from a reactive polycythemia
- normal SaO2
2. decreased EPO (negative feedback)
neoplastic proliferation of mature myeloid cells, esp platelets
essential thrombocytopenia
mutation associated with essential thrombocytopenia
JAK2 kinase
myeloproliferative d/o that rarely progresses to marrow fibrosis or progresses to acute leukemia and no increased risk for hyperuricemia
essential thrombocytopenia
neoplastic proliferation of mature myeloid cells, esp megakaryocytes
myelofibrosis
mutation associated with myelofibrosis
JAK-2 kinase
mechanism that produces marrow fibrosis in myelofibrosis
megakaryocytes produce excess PDGF
mechanism of splenomegaly in myelofibrosis
extramedullary hematopoiesis - fetal hematopoietic stem cells were located in spleen
blood smear resulting from extramedullary hematopoiesis in myelofibrosis
leukoerythroblastic smear
tear drop cell
myelofibrosis (from RBCs produced in fibrotic bone marrow)
