Myeloproliferative Neoplasms - Polycythemia, Thrombocytosis, Myelofibrosis, Myelodysplasia Flashcards
What is an MPN?
Proliferation of myeloid lineage
-RBCs, platelets, megakaryocites, granulocytes
Classification based on dominant cell line involved
-all can proliferate
Polycythemia vera
-pathophysiology
-presentation
-diagnosis
-management
-complications
JAK2 mutation => constantly active EPO receptor on stem cells => high RBC production independent of EPO
- hyperviscosity => clots
- plethoric
- splenomegaly
- gout <= increased DNA synthesis and metabolism
- heat => itch (increaed histamine release)
FBC, film - high Hb, hematocrit, RBC mass
-slight increase in other myeloid cells
U&E, LFT
Low EPO
BM biopsy - JAK2
Aspirin - reduce clots
Venesection - 1st line to reduce Hb
Chemo - hydroxyurea
Thrombotic events
Progression to myelofibrosis or acute leukemia
Essential thrombocytosis
-pathophysiology
-presentation
-diagnosis
Megakaryocyte proliferation => overproduction in platelets
JAK mutation
Increased clotting/bleeding
Splenomegaly
Burning hands
Platelet count
Diagnosis of exclusion
High platelets - 600+
BM biopsy - JAK2, increased megakaryocytes
Chemo - hydroxyurea
Reduce clots - aspirin
Myelofibrosis
-pathophysiology
-presentation
-investigations
-management
Abnormal megakaryocytes produce cytokines => BM replaced by collagen
-B symptoms
-BM failure => splenomegaly (spleen tries to compensate for lack of blood cells)
Blood smear - teardrop cell
BM biopsy - dry tap, cannot get any cells, JAK2
-bone marrow trephine biopsy - assess structure within bone
Symptomatic - supportive transfusions, JAK2 inh
Definitive - stem cell transplant
Myelodysplasia
-pathophysiology
-presentation
-diagnosis
-management
-complications
Ineffective development of blood cells => reduced no of blood cells
BM failure
Can be asymptomatic
Peripheral blood counts
BM biopsy - dysplastic changes in hematopoetic cells
Supportive - blood transfusions, growth factors
Immunosuppresants and stem cells
RISK OF PROGRESSION INTO AML
