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Hematology > Myeloproliferative Myelodysplastic Syndromes > Flashcards

Myeloproliferative Myelodysplastic Syndromes Flashcards

1
Q

myeloproliferative and myelodysplastic syndromes deal with

A

disorders of clonal stem lines of all three linages platelet, WBC, and RBC are affected

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2
Q

three types of myeloproliferative syndromes

A
  1. polycythemia vera
  2. essential thrombosis
  3. myelofibrosis
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3
Q

erythrocytosis

A

increased RBC volume, increased hemoglobin and hematocrit

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4
Q

polycythemia

A

synonym for erythorocytosis

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5
Q

Secondary factors can cause erythrocytosis

A

these condition tend to lead to erythrocytosis because tissue hypoxia induces EPO to be secrete form the kidneys

  1. OSA
  2. obesity causing hyperventilation
  3. high attitudes
  4. high affinity hemoglobin
  5. cardiopulmonary disorders
  6. high carboxyhemoglobin levels
  7. tumor
  8. treatment with androgens or EPO
  9. rare genetic disorders
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6
Q

erythrocytosis can lead to _______

A

hyperviscosity

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7
Q

hyperviscosity symptoms

A

thickening of the blood; headache, tinnitus, decreased mental functioning, dizziness, visual changes, paresthesias

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8
Q

how to treat hyperviscosity?

A

phlebotomy

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9
Q

polycythemia vera clinical characteristics

A 
erythromelalgia 
pruritis 
hypermetabolic state
thrombosis - Budd Chiari syndrome 
Hemorrhage
splenomegaly 
hepatomegaly 
facial plethora
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10
Q

Budd Chiari syndrome can develop from what two disorders?

A

polycythemia vera and PNH

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11
Q

phases of polycythemia vera

A
  1. latent- asymptomatic
  2. proliferative- hypermetabolic and hyperviscosity
  3. spent- anemia, leukopenia, increasing liver and spleen size, fever, weight loss, secondary myelofibrosis
  4. AML
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12
Q

What mutation is common in patient’s with polycythemia vera?

A

JAK2

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13
Q

Lab findings for polycythemia

A
  • increased hemoglobin hematocrit
  • increased platelets
  • increased EPO
  • positive for JAK2 mutation
  • BASOPHILIA - increased basophils present
  • increased uric acid –> can lead to gout
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14
Q

how does polycythemia usually arise?

A

from a single neoplastic stem cell, mutations in JAK2

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15
Q

treatment for polycythemia?

A
  • phlebotomy
  • low dose aspirin
  • hydroxyurea
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16
Q

90% of patients with essential thrombocytosis have

A

somatic genetic mutation

ex. JAK2, CALR, MPL

17
Q

essential thrombocytosis similar symptoms to p vera except there is increased risk of

A

arterial or venous thrombosis

18
Q

essential thrombocytosis clinical symptoms

A
  • pruritis
  • erythromelagia
  • thrombosis
  • hemorrhage
    differences compared to p vera
    1. digital ischemia
    2. no facial plethora
19
Q

first step in diagnosing essential thrombocytosis

A
  • rule out other causes of thrombocytosis
  • order a ferritin level
    1. infection
    2. cancer
    3. iron deficiency
    4. bleeding
    5. inflammation
20
Q

who do we treat for essential thrombocytosis?

A
  • patients who are greater than 65
  • have greater than 450 platelets on two occasions 1 month apart
  • increased risk of thrombosis
21
Q

treatment is focused on

A
  • decreasing platelet count

- hydroxyurea

22
Q

laboratory findings for essential thrombocytosis

A
  • JAK2 mutation
  • abnormal irregular platelet, usually giant sized
  • increased megokaryocytes
  • ferritin level should be normal
23
Q

between the three myeloproliferative disorders which one has the most risk to turn into AML?

A 
myelofibrosis 
then 
polycythemia vera
then 
essential thrombocytosis
24
Q

myelofibrosis is mainly a disorder that

A

arises from a disorder that affects the clonal stem line for megakaryocytes

25
Q

treatment for myelofibrosis

A
  • there is not definitive treatment
  • supportive care
  • splenomegaly to relieve abdominal pain but increased risk of hemorrhage
  • if young bone marrow transplant
26
Q

clinical features of myelofibrosis

A
  • splenomegaly and heptamegoly
  • fever, chills, weight loss
  • extramedullary hematopoesis that causes splenomegaly which can lead to early satiation
27
Q

laboratory findings of myelofibrosis

A
  • blood smear- teardrops, NRBC, and granulocytes, leukoerythroblastic (immature leukocytes)
  • dry bone marrow tap
  • early on the RBC, WBC and Hb are all elevated but as the disease progresses they will all decrease
28
Q

all myeloproliferative disorders can result in spent phase which is

A

similar to myelofibosis

29
Q

myeloid metaplasia

A

refers to the early proliferative phase of myelofibrosis where there is extrameduallary hematopoiesis

30
Q

myelodysplastic syndrome

A
  • abnormal clonal line that results in cytopenia
31
Q

myelodysplastic syndrome is usually found in the

A

elderly population due to lower levels on CBC

32
Q

peripheral smear for myeldysplastic syndrome should show

A
  1. bilobed neutrophils
  2. monocytosis- increased number of monocytes
  3. macrocytosis - with normal levels of B12 and folate
33
Q

bone marrow biopsy should show

A
  1. ringed sideroblast
  2. megaloblastic erythropoiesis
  3. blast cells should account for less than 20 % of slide (more than 20 would be leukemia)
  4. dyserythropoiesis- abnormal nucleus on RBC
  5. small megokaryocytes with abnormally hypolobated nuclei
34
Q

mylodysplastic is usually are result of

A

cytogenetic abnormalities

35
Q

mylodysplastic treatment

A
  • treatment is usually supportive
  • platelet and rbc transfusion
  • hypermethylation of DNA
  • growth factors
36
Q

once the patient develops AML

A

the chances of achieving remission is much shorter

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