Myeloproliferative Disorders (MPD) Flashcards Preview

SBL Exam 1 WBCs, Spleen, and Thymus > Myeloproliferative Disorders (MPD) > Flashcards

Flashcards in Myeloproliferative Disorders (MPD) Deck (25)
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1

What are MPDs?

Myeloproliferative disorders

-neoplasm of mature myeloid cells (occasionally lymphoid cells)

-leukocytosis with bone marrow hypercellularity

-commonly associated with mutated tyrosine kinases

-frequent extramedullary hematopoiesis

 

*can progress to AML or ALL*

2

What are the main types of MPD?

 

 

-CML, chronic myeloid leukemia

-PV, polycythemia vera

-ET, essential thrombocythemia

-primary myelofibrosis

3

What is CML?

Chronic myeloid leukemia (MPD)

-neoplasm of mature myeloid cells (particularly granulocytes)

-caused by t(9;22)/Philadelphia chromosome with BCR-ABL fusion product

-splenomegaly due to extramedullary hematopoeisis

 

4

What is the common presentation of CML?

-typically adults (median 50-60); can occur in all ages

-more frequent in men

-fatigue, weakness, weight loss (due to anemia and hypermetabolism)

-splenomegaly causing possible LUQ pain

5

What are possible complications of CML?

-splenic rupture

-progression to acute leukemia

-hyperuricemia/gout from purine degradation due to high cell turnover (give allopurinol)

-marrow fibrosis

6

What is the course/prognosis for CML?

-insidious course

-slow progression for ~3 years followed by accelerating phase for 0.5-1 years, and blast crisis (resmbling acute leukemia)

 

-if treated early, good prognosis with 90% remission (imatinib, tyrosine kinase inhibitor treatment)

7

How can CML be differentiated from a leukemoid reaction?

Both have leukocytosis and a left shift

 

CML has:

-increased basophils not present w/ leukemoid reaction

-negative leukocyte alkaline phosphatase (LAP) whcih is positive in leukemoid reaction

-pressence of Philadelphia chromosome

8

What is PV?

Polycythemia vera (MPD)

-neoplasm of mature myeloid cells (particularly erythrocytes)

-Jak2 kinase mutation

-low serum erythropoietin

-elevated hematocrit -> increased viscosity

9

What is the common presentation of PV?

-late middle age adults

-symptoms due to inceased blood viscosity

-cyanosis

-facial flushing

-blurry vision and HA

-MI, CVA, and DVT (presenting s/x 25% of cases)

-DVT in hepatic vein is common -> Budd-Chiari syndrome

-hemorrhage

-pruritis, frequent after bathing, from increased histamine release

 

10

What are possible complications of PV?

-MI, CVA, and DVT

-DVT in hepatic vein is common -> Budd-Chiari syndrome

-hemorrhage

-hyperuricemia/gout from purine degradation due to high cell turnover (give allopurinol)

-progression to myelofibrosis following treatment (15-20%)

-progression to AML (2%)

 

11

What is the course/prognosis for PV?

-insidious course

 

-good prognosis w/ treatment (median 10 years) (phlebotomy to reduce RBC mass)

-w/o treatment death within months

12

What is ET?

Essential thrombocytosis (MPD)

-neoplasm of platelets

-very little elevation of RBCs or WBCs

-no marrow fibrosis

-Jak2 kinase mutation (50%) or MPL mutation (5-10%)

13

What is the common presesntation of ET?

-primarily adults >60; can occur in younger ages

-symptoms limited and related to thrombosis or bleeding

 

14

What are possible complications of ET?

-MI/DVT

-portal vein thrombosis

-hemorrhage

-erythromelalgia (throbing/burning in hands in feet due to arteriole obstruction by platelets)

15

What is the course/prognosis of ET?

-indolent course with indisious onset

-asymptomatic periods interupted by occasional hemorragic/thrombotic crisis

 

-good prognosis (median survival 12-15 years) ("gentle" chemotherapy)

16

How is ET diagnosed?

Diagnosis of exclussion

 

Should rule out:

-PCV

-primary myelofibrosis

-reactive thrombocytosis

-iron deficiency anemia

17

How is ET different from CML, PV, and myelofibrosis?

-rarely progresses to AML

-minimal fibrosis

-no risk of hyperuricemia/gout (megakaryocytes do not lose nuclei when producing platelets)

18

What is primary myelofibrosis?

Primary myelofibrosis (MPD)

-neoplasm of mature myeloid cells (particularly megakaryocytes)

-Jak2 kinase mutation (50-60%) or MPL mutation (1-5%)

-megakaryocytes produce PDGF and TGF which attracts nomral fibroblasts

-progressive obliterative marrow fibrosis resulting in eventual cytopenias

-extramedullary hematopoiesis w/ splenomegaly

19

What is the common presentation of primary myelofibrosis?

-primarily adults >60

-splenomegaly (sometimes only finding)

-fatigue (anemia)

 

20

What histologic features are present in primary myleofibrosis?

-tear drop RBCs

-nucleated RBCs

-immautre granulocytes

21

What is the course/prognosis of primary myelofibrosis?

-difficult to treat (median survivial 3-5 years)

-better in younger individuals who can withstand HSCT

22

What are possible complications of primary myelofibrosis?

-progression to AML (5-20%)

-infections

-thrombosis

-hemorrhage

-hyperuricemia/gout from purine degradation due to high cell turnover (give allopurinol)

23

What lab findings are expected with CML?

Blood:

-leukocytosis often >100,000 cells/cm3 (primarily granuloytes)

-blasts present

-markedly increased platelets

 

Bone marrow:

-hypercellular

-sea-blue histocytes

 

24

What lab findings are expected with PV?

Blood:

-elevated hematocrit (>60%)

-normal to elevated hemoglobin (14-28g/dL)

-leukocytosis 12,000-50,000 cells/mm3

-increased platelets

 

Bone marrow:

-hypercellular (early); fibrotic (spent phase)

-increased progenitors (erythropoietic and granulocytic)(early); decreased (spent phase)

25

What lab findings are expected with primary myelofibrosis?

Blood:

-moderate to severe normochromic, normocytic anemia

-blasts present

-inintial markedly increased WBC; normal to decreased WBC (late)

-normal to elevated platelet count; eventual thrombocytopenia

 

Bone marrow:

-hypercellular (early); hypocellular (late)

-fibrotic (late)