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Endocrine Exam 3 > 41 - Myeloproliferative Disorders > Flashcards

41 - Myeloproliferative Disorders Flashcards

1
Q

What are the 4 types of myeloproliferative disorders?

A
  • Chronic myelogenous leukemia (CML) = MOST COMMON
  • Polycythemia vera (PCV)
  • Chronic idiopathic myelofibrosis
  • Essential thrombocythemia
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2
Q

What are myeloproliferative disorders?

A
  • A diverse group of clonal, neoplastic hematologic disorders that have abnormal proliferation of hematopoietic precursors
  • All can eventually progress to AML
  • Basophilia tends to occur in all of the myeloproliferative syndromes **
  • Think BASOPHILS ***
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3
Q

Describe CML - chronic myelogenous leukemia

A
  • Annual incidence 1 in 100,000
  • Accounts for 15-20% Leukemias
  • Slightly more common in males (1.4:1)
  • Peak incidence 40-60 years
  • Only known etiology is h/o ionizing radiation
  • 3 phases of CML
    Chronic –> Accelerated –> Blast Phase
  • If someone has CML but is unaffected by it, they are in the chronic phase (little clinical relevance)
  • Very high white count (White count is very high, 65,000 or 85,000 - Anything above 10,000 is bad)
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4
Q

What are the serological indications of CML?

A
  • Leukocytosis *** sometimes 50-600K/mL
  • Increased basophils ** (also increased eosinophils an nucleated RBCs) - (remember basophils are supposed to be low, but here it is HIGH **)
  • Low leukocyte alkaline phosphatase
  • More than 30% blasts in the peripheral blood means your patinet is in a BLAST CRISIS
  • This is one of the three phases in the natural progression of CML and is the worst
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5
Q

What are the cytogenetic indications of CML?

A
  • **KNOW THIS**
  • Philadelphia chromosome
  • This is positive in more than 95% fo CML patients
  • Philadelphia chromosome is an unusually short autosome in the WBCs of CML patients
  • The mutation is t(9, 22) translocation
  • BCR-ABL oncogene (“brotherly love”)

Philadelphia chromosome = CML = TEST Q***

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6
Q

What are the common symptoms of CML?

A
  • Lethargy, weight loss, increasing abdomen girth, sweating, easy bruising/bleeding, Hepato-Splenomegaly ***
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7
Q

How do you diagnose CML

A
  • t(9,22) translocation
  • Philadelphia chromosome
  • Low leukocyte alkaline phosphatase
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8
Q

What happens eventually in almost all CML?

A
  • Becomes blastic
  • Converts to an acute form of leukemia (***80% AML, 20% ALL)
  • This occurs at rate of 25% per year will get AML or ALL***
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9
Q

What is polycythemia vera?

A

A neoplasm in which the bone marrow makes too many red blood cells. It may also result in the overproduction of white blood cells and platelets.

Erythrocytosis and polycythemia mean the same thing

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10
Q

What lab values will you see in polycythemia vera (PCV)?

A
  • Hb and Hct above normal (called erythrocytosis or polycythemia)

Do not confuse this with RELATIVE polycythemia
- Relative polycythemia = caused by dehydration from GI loss or insensible fluid loss, It is relative – they are just dehydrated

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11
Q

What is primary PCV?

A
  • Erythropoietin (aka EPO) – independent red blood cell production (low EPO=PCV)
  • Need more RBCs, so erythropoietin needs ↑
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12
Q

What is secondary PCV?

A
  • Exogenous EPO administration (Lance Armstrong)
  • Excessive EPO production
    • -> Tissue hypoxia (e.g. high altitude, pulm dz, sleep apnea)
    • -> Autonomous secretion from selected tumors
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13
Q

What are the major criteria for PCV diagnosis?

A

Major

  • Elevated RBC mass
  • Normal arterial oxygen saturation
  • Splenomegaly
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14
Q

What are the minor criteria for PCV diagnosis?

A

Minor

  • Thrombocytosis
  • Leukocytosis
  • Elevated leuk alk phosphatase
  • Elevated Vit B12 or Vit B12 binding capacity
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15
Q

What do you need to diagnose PCV?

A

Must have all 3 major or first two major with any 2 minor

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16
Q

What is the clinical presentation of PCV?

A
  • Older patient
  • Puitis after bathing (itchy)***
  • Hyperviscosity smptoms (vertigo, confusion, visual changes)
  • May have sweats, weight loss
  • Ruddy (red faced) complexion ***
  • Splenomegaly**
17
Q

What would you see in the serology of a PCV patient?

A

Serology:

  • Classically elevation of Hgb/Hct
  • Elevated RBC mass (>36mL/kg men, >32 women)
  • EPO level low in PCV patients

TEST Q: NEED to know that the classic PCV patient will have DECREASED EPO

18
Q

What are the complications of PCV?

A
  • Thromboses: CVA’s, MI’s, DVT’s, etc
  • Bleeding (complications of high platelet counts and impaired platelet function)

***These are important causes of morbidity and mortality in 30-40% of PCV patients.

19
Q

What is the median survival of patients with PCV?

A
  • 10 years with phelbotomy treatment

* TEST Q*

20
Q

What is the major cause of death in PCV?

A
  • Thrombosis
  • Less often, hemorrhage

TEST Q

21
Q

What is chronic idiopathic myelofibrosis?

A

AKA myelofibrosis with myeloid metaplasia (MMM)

- Occurs when neoplastic stem cells migrate and make marrow in areas outside of the bone marrow

22
Q

Describe what patients this typically occurs in

A
  • Median age is 65 years

- Occurs primarily in the liver and SPLEEN *** but can occur in any organ

23
Q

Describe what happens in chronic idiopathic myelofibrosis

A
  • HUGE splenomegaly
  • 50% have hepatomegaly
  • Weight loss, night sweats, fevers, fatigue, LUQ pain
  • Things get caught in the spleen
24
Q

What will you see in the serology of chronic idiopathic myelofibrosis?

A
  • Anemia (20% have Hgb
25
Q

What will happen when you do a bone marrow tap on a patient with chronic idiopathic myelofibrosis?

A

You will get a “dry tap” due to increased collagen and reticulin fibers

All the “juice” of bone marrow is dried up and turned into collagen – you try to take out marrow and it comes out dry

26
Q

What is essential thrombocythemia?

A
  • A myeloproliferative disorder with persistent thrombocytosis (platelet count > 600 * 10^9/L) that is not reactive to another disorder and is not caused by another myeloproliferative disorder
  • Bone marrow shows megakaryocyte hyperplasia (platelet precursor) and clustering – megakaryocyte = platelet precursor
27
Q

CBC components

A
  • Hemoglobin
  • Hematocrit
  • WBCs
  • Platelets

Now we are talking about the platelet problem

28
Q

What kind of platelet counts will you see in essential thrombocythemia?

A
  • 600K to 4 million platelets, this is too many

Normal is 100,000 to 200,000

29
Q

Describe the clinical condition of patients with essential thrombocythemia

A
  • Least common and least aggressive of the 4
  • A diagnosis of exclusion from other 3 and from reactive thrombocytosis
  • Median age at diagnosis is 50-60 years, second peak in women in 3rd or 4th decade of life
  • Patients have a near-normal life expectancy

KNOW: near normal life span, least aggressive ***

30
Q

What are the signs and symptoms of essential thrombocythemia?

A

Headache, transient ischemic attacks, paresthesia… etc.

Erythromelagia, digital pain, bleeding, weight loss, sweating, fevers, puritis (all of these were also included on the slides)

31
Q

How is thrombocytosis found?

A

On routing serology

32
Q

What are the complications of essential thrombocythemia?

A
  • Thrombosis (most common - 20%)
  • Hemorrhage (3%)
  • Splenomegaly (30-50%)
  • Can become an acute leukemia (
33
Q

Why would these complications occur?

A
  • Thrombosis (number of platelets is high, too much)
  • Hemorrhage (number and function of platelets are off)
  • Splenomegaly (filters too many platelets, gets big)
34
Q

How do you diagnose essential thrombocythemia?

A
  • Platelet count is more than 600K *****

Others

  • Megakaryocytic hyperplasia
  • Splenomegaly
  • Absence of Ph(+) xome
  • Normal RBC mass
  • Exclusion of all causes of reactive thrombocytosis
  • Stainable iron in marrow
35
Q

What is the general treatment of essential thrombocythemia?

A

Do not pay too much attention to the treatment

The general would be that they will be treated with interferon and hydroxyurea

36
Q

What myeloproliferative disorder carries a 25% conversion risk per year for transforming into acute leukemia?

TEST QUESTION

A

Chronic myelogenous leukemia

37
Q

Which of the myeloproliferative disorders we discussed is the least aggressive and least common?

TEST QUESTION

A

Essential thrombocytopenia

38
Q

In your blood cell count differential, in myeloproliferative disorders, what cell line would abnormally be elevated?

TEST QUESTION

A

Basophils

Endocrine Exam 3 (13 decks)

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