Flashcards in Myeloproliferative and myelodyplasia Deck (76):
Who is usually diagnosed with MDS?
What is the phenotype of MDS?
Peripheral cytopenias with normocellular or hypercellular bone marrow
Those with MDS have an increase risk for what?
What is the cause of primary MDS?
What are the causes of secondary MDS?
HOw do you characterize MDS?
What cell line is decreased
Leukemia is caused by bad type of what cells?
SSx of MDS?
anemia (if low Hb/RBCs)
infections (if low WBCs)
Bleeding manifestations (if low platelets)
What is sweet syndrome?
Acute neutrophilic dermatosis and pyoderma gangrenosum
What is the cause of sweet syndrome?
How do you confirm MDS?
Bone marrow biopsy with normo or hypercellular bone marrow
What is trilineage dyspoiesis?
Abnormal WBC, RBC, platelet precursors
What is the value of Fe studies in MDS?
r/o Fe deficiency anemia
What is pseudo-Pelger huet anomaly?
Bilobed nucleus with a string between, characteristic of MDS
Ringed sideroblasts = ?
MDS or sideroblastic anemia
Can B12/folate deficiencies result in decrease WBCs or platelets?
True or false: ethanol exposure cannot cause bone marrow suppression
What is the function of G-CSF?
Macrophage released cytokine that increases PMNs
What are the two types of antineoplastics that can cause MDS?
Treatment with what chemotherapeutic agents can lead to MDS, prior to AML?
Treatment with what chemotherapeutic agents can lead to AML, without MDS as an intermediary?
What are the factors that go into the prognostic scoring for MDS, and probability for developing to AML? (3)
What is 5q syndrome? What are the three blood anomalies? Who gets this?
MDS subtype more common in older women
Platelet count normal to increase
What is the usual cause of death with MDS?
What is the prognosis for 5q syndrome?
(favorable)--Transformation to AML is less common that other subtypes of MDS
but require transfusions
What do you treat MDS with?
What is CMML?
Form of MDS/MPD, characterized by increased monocytes in BM and pS
What is the treatment for CMML?
What is hypocellular MDS?
MDS that has the same clinical features as hypercellular MDS
Resembles aplastic anemia
What is the low intensity treatment for MDS?
Growth factor support
What is the high intensity treatment for MDS?
Stem cell transplant
Clinical trials of novel therapies
What are the growth factors used for MDS treatment?
G-CSF or GM-CSF
EPO if low Hb
What is the drug that is used to treat 5q syndrome?
What are the two drugs that are used to treat MDS outside of 5q syndrome?
Hypomethylating agents like azacytidine and decitabine
What is the only cure to MDS?
Allogenic stem cell transplant
What can MDS turn into if untreated?
What are MPD?
Overproduction of one or more blood cell lines, but normal maturation
Is hematopoiesis effective in MPD?
What can MPD turn into if left untreated?
What are the four myeloproliferative disorders?
What is the gene mutation that causes MPD?
JAK2 mutations (tyrosine kinase inhibitor)
What is the cause of CML?
What are the ssx of CML?
Fatigue, night sweats
What are the labs outside of the CBC that are elevated with CML?
LDH and uric acid
What are the CBC results in CML?
Absolute basophilia (invariable)
What is the chronic phase of CML?
Peripherl bone marrow blasts
What is the accelerated phase of CML?
Bloood or bone marrow blasts >10%
What is the blast phase f CML?
Blood or marrow blasts >20%
How do you diagnose CML?
bone marrow biopsy
What is the treatment for CML?
What are the second generation Y-kinase inhibitors?
What is the cause of imatinib failure? How do you overcome this?
Development of mutations in the ABL kinase domain
Increase dose or change meds
What is polycythemia vera?
Elevated RBC mass in the absence of secondary conditions
What happens to WBCs and platelets in polycythemia vera?
What are the symptoms of polycythemia vera?
Who usually has polycythemia vera?
What is the cause of polycythemia vera?
What are the signs of polycythemia vera?
What are the major criteria for polycythemia vera?
Hg >18.5 in men (>16.5 women)
Presence of JAK2 mutation
What are the minor criteria for polycythemia vera?
Bone marrow with hypercellularity
Serum EPO below reference range for normal
What is needed to diagnose polycythemia vera?
Both major criteria and 1 minor,
What is the treatment for polycythemia vera?
What is the HCT goal with polycythemia vera?
What is the drug that can be used for polycythemia vera if not prego?
What is anagrelide?
Drug that selectively inhibits platelet production
What is essential thrombocytosis? Who get is?
Isolated increase in platelet count
What is the genetic cause of essential thrombocytosis?
What are the SSx of essential thrombocytosis?
Thrombosis or hemorrhage, usually GI tract
What is the problem with essential thrombocytosis?
First trimester spontaneous abortions (but rare)
What are the major criteria of essential thrombocytosis?
1. Sustained high platelet count
2. bone marrow biopsy with proliferation of megakaryocytes
3. Not polycythemia vera or other causes
4. JAK2 mutation
What is the treatment of essential thrombocytosis?
Anagrelide or IFN-alpha
What is the goal platelet count for essential thrombocytosis if symptomatic?
Which MPD has the worst prognosis?
What is primary myelofibrosis?
FIbrotic changes of one marrow
What are the ssx of primary myelofibrosis? (4)