Myeloproliferative and myelodyplasia Flashcards by tcarlso 3

Who is usually diagnosed with MDS?

Older

Women

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What is the phenotype of MDS?

Peripheral cytopenias with normocellular or hypercellular bone marrow

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Those with MDS have an increase risk for what?

Acute leukemia

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What is the cause of primary MDS?

Unclear

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What are the causes of secondary MDS?

Genetic factors
Environmental factors
Antineoplastic agents

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HOw do you characterize MDS?

What cell line is decreased

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Leukemia is caused by bad type of what cells?

Blasts

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SSx of MDS?

anemia (if low Hb/RBCs)
infections (if low WBCs)
Bleeding manifestations (if low platelets)
Organomegaly

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What is sweet syndrome?

Acute neutrophilic dermatosis and pyoderma gangrenosum

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What is the cause of sweet syndrome?

Unknown

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How do you confirm MDS?

Bone marrow biopsy with normo or hypercellular bone marrow

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What is trilineage dyspoiesis?

Abnormal WBC, RBC, platelet precursors

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What is the value of Fe studies in MDS?

r/o Fe deficiency anemia

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What is pseudo-Pelger huet anomaly?

Bilobed nucleus with a string between, characteristic of MDS

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Ringed sideroblasts = ?

MDS or sideroblastic anemia

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Can B12/folate deficiencies result in decrease WBCs or platelets?

yes

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True or false: ethanol exposure cannot cause bone marrow suppression

False

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What is the function of G-CSF?

Macrophage released cytokine that increases PMNs

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What are the two types of antineoplastics that can cause MDS?

Alkylating agents

Topoisomerase inhibitors

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Treatment with what chemotherapeutic agents can lead to MDS, prior to AML?

Alkylating agents

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Treatment with what chemotherapeutic agents can lead to AML, without MDS as an intermediary?

Topoisomerases

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What are the factors that go into the prognostic scoring for MDS, and probability for developing to AML? (3)

Marrow blasts
Karyotypes
cytopenia

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What is 5q syndrome? What are the three blood anomalies? Who gets this?

MDS subtype more common in older women

macrocytic anemia
Leukopenia
Platelet count normal to increase

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What is the usual cause of death with MDS?

Infection

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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?

Lenalidomide

What is CMML?

Form of MDS/MPD, characterized by increased monocytes in BM and pS

What is the treatment for CMML?

Imatinib

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?

Supportive care Transfusions PRN Growth factor support

What is the high intensity treatment for MDS?

Stem cell transplant Chemo 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?

Lenalidomide

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?

Leukemia

What are MPD?

Overproduction of one or more blood cell lines, but normal maturation

Is hematopoiesis effective in MPD?

yes

What can MPD turn into if left untreated?

Leukemia

What are the four myeloproliferative disorders?

CML Polycythemia vera Essential thrombocytosis Primary myelofibrosis

What is the gene mutation that causes MPD?

JAK2 mutations (tyrosine kinase inhibitor)

What is the cause of CML?

t(9;22) BCR-ABL

What are the ssx of CML?

Fatigue, night sweats Anemia Platelet dysfunction Neutrophilia

What are the labs outside of the CBC that are elevated with CML?

LDH and uric acid

What are the CBC results in CML?

Neutrophilia Absolute basophilia (invariable) Eosinophilia

What is the chronic phase of CML?

Peripherl bone marrow blasts BCR-ABL rearrangement

What is the accelerated phase of CML?

Essentially leukemia | Bloood or bone marrow blasts >10%

What is the blast phase f CML?

Blood or marrow blasts >20%

How do you diagnose CML?

Cytogenetics/FISH | bone marrow biopsy

What is the treatment for CML?

Imatinib

What are the second generation Y-kinase inhibitors?

Dasatinib | Nilotinib

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?

Also increases

What are the symptoms of polycythemia vera?

Pruritus

Who usually has polycythemia vera?

Older males

What is the cause of polycythemia vera?

JAK2 mutation

What are the signs of polycythemia vera?

HTN HSM gout PHTN

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?

ASA | Phlebotomy

What is the HCT goal with polycythemia vera?

What is the drug that can be used for polycythemia vera if not prego?

Hydroxyurea

What is anagrelide?

Drug that selectively inhibits platelet production

What is essential thrombocytosis? Who get is?

Isolated increase in platelet count Older women

What is the genetic cause of essential thrombocytosis?

JAK2 mutation

What are the SSx of essential thrombocytosis?

High Hb Neutrophilia 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?

Hydrea | Anagrelide or IFN-alpha

What is the goal platelet count for essential thrombocytosis if symptomatic?

Which MPD has the worst prognosis?

Primary myelofibrosis

What is primary myelofibrosis?

FIbrotic changes of one marrow

What are the ssx of primary myelofibrosis? (4)

Marrow fibrosis HSM Portal HTN Hemochromatosis

What is the treatment for primary myelofibrosis?

Palliative and supportive JAK2 inhibitors Deferoxamine