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(J2) WBCs, Spleen, and Thymus > Myeloproliferative Disorders (MPD) > Flashcards

Myeloproliferative Disorders (MPD) Flashcards

1
Q

What are MPDs?

A

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*

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2
Q

What are the main types of MPD?

A
  • CML, chronic myeloid leukemia
  • PV, polycythemia vera
  • ET, essential thrombocythemia
  • primary myelofibrosis
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3
Q

What is CML?

A

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
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4
Q

What is the common presentation of CML?

A
  • 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
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5
Q

What are possible complications of CML?

A
  • splenic rupture
  • progression to acute leukemia
  • hyperuricemia/gout from purine degradation due to high cell turnover (give allopurinol)
  • marrow fibrosis
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6
Q

What is the course/prognosis for CML?

A

-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)
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7
Q

How can CML be differentiated from a leukemoid reaction?

A

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
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8
Q

What is PV?

A

Polycythemia vera (MPD)

  • neoplasm of mature myeloid cells (particularly erythrocytes)
  • Jak2 kinase mutation
  • low serum erythropoietin
  • elevated hematocrit -> increased viscosity
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9
Q

What is the common presentation of PV?

A

-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

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10
Q

What are possible complications of PV?

A
  • 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%)
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11
Q

What is the course/prognosis for PV?

A
  • insidious course
  • good prognosis w/ treatment (median 10 years) (phlebotomy to reduce RBC mass)
  • w/o treatment death within months
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12
Q

What is ET?

A

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%)

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13
Q

What is the common presesntation of ET?

A
  • primarily adults >60; can occur in younger ages
  • symptoms limited and related to thrombosis or bleeding
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14
Q

What are possible complications of ET?

A
  • MI/DVT
  • portal vein thrombosis
  • hemorrhage
  • erythromelalgia (throbing/burning in hands in feet due to arteriole obstruction by platelets)
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15
Q

What is the course/prognosis of ET?

A

-indolent course with indisious onset

-asymptomatic periods interupted by occasional hemorragic/thrombotic crisis

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

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16
Q

How is ET diagnosed?

A

Diagnosis of exclussion

Should rule out:

  • PCV
  • primary myelofibrosis
  • reactive thrombocytosis
  • iron deficiency anemia
17
Q

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

A
  • rarely progresses to AML
  • minimal fibrosis
  • no risk of hyperuricemia/gout (megakaryocytes do not lose nuclei when producing platelets)
18
Q

What is primary myelofibrosis?

A

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
Q

What is the common presentation of primary myelofibrosis?

A
  • primarily adults >60
  • splenomegaly (sometimes only finding)
  • fatigue (anemia)
20
Q

What histologic features are present in primary myleofibrosis?

A
  • tear drop RBCs
  • nucleated RBCs
  • immautre granulocytes
21
Q

What is the course/prognosis of primary myelofibrosis?

A
  • difficult to treat (median survivial 3-5 years)
  • better in younger individuals who can withstand HSCT
22
Q

What are possible complications of primary myelofibrosis?

A
  • progression to AML (5-20%)
  • infections
  • thrombosis
  • hemorrhage
  • hyperuricemia/gout from purine degradation due to high cell turnover (give allopurinol)
23
Q

What lab findings are expected with CML?

A

Blood:

  • leukocytosis often >100,000 cells/cm3 (primarily granuloytes)
  • blasts present
  • markedly increased platelets

Bone marrow:

  • hypercellular
  • sea-blue histocytes
24
Q

What lab findings are expected with PV?

A

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
Q

What lab findings are expected with primary myelofibrosis?

A

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)

(J2) WBCs, Spleen, and Thymus (12 decks)

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