Leukemias Flashcards

1
Q

What do abnormal CBC patterns indicate

A

Leukocytosis: reactive vs cancer or BM d/o
Leukopenia: meds, splenic sequestration, BM d/o
Polycythemia: dehydration, BM d/o
Thrombocytosis: reactive vs BM do
Thrombocytopenia: consumptive vs BM d/o

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2
Q

What are the WBC

A
Neutrophils 
Eosinophils 
Basophils 
Monocytes 
Lymphoctes
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3
Q

What do different leukocytosis findings indicate

A

Blasts: AML/ALL
Bands: infection
High neuts, low lymphs, no abn cells: Corticosteroids

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4
Q

If you find polycythemia vera (high H&H), inquire about these

A
Dehydration 
Tobacco use 
OSA 
Pickwickian (very obese= high risk OSA) 
JAK2 mutation 
High EPO levels
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5
Q

If you find thrombocytosis, inquire about these

A

Co-existing anemia
infection
removed spleen
non-infx inflammatory condition

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6
Q

What are Myeloproliferative Neoplasm

A

Acquired clonal abnormalities, can lead to MDS
Basically, the thermostat that control blood cell production is turned UP!
MPN and MDS are on a continuum as part of chronic myeloid neoplasms and can progress to acute leukemia*

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7
Q

Compare MPN vs MDS

A

MPN: terminal myeloid expansion= too much blood!
MDS: cell dysplasia= not enough blood!

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8
Q

What are the chronic myeloproliferative neoplasms

A
CML 
polycythemia vera 
essential thrombocytopenia 
primary myelofibrosis 
non-classic MPN
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9
Q

What are the types of polycythemia

A

Primary: 2/2 acquired or inherited mutation; polycythemia vera, chuvash polycythemia, idiopathic erythrocytosis
Secondary: 2/2 elevated Epo; Epo secreting tumor, Hypoxia
Combined: decreased plasma volume + increased RBC mass; tobacco use

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10
Q

What is polycythemia vera

A

JAK 2 mutation (exon 14&12) causes increased RBC mass, with a low Epo level
Dx w/ BM Bx: hypercellular with absent Fe stores (overproducing RBC but not iron)
MC in 60+ y/o

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11
Q

How does polycythemia vera present

A
Hgb: 18 
Pruritis after warm shower 
HA, dizziness 
blurred vision 
tinnitus 
fatigue 
early satiety 
PUD Sx 
Erythromelalgia of hands and feet 
Facial plethora 
plenomegaly 
HTN 
Excoriated skin 
Thrombosis or hemorrhage
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12
Q

How do you treat PV

A

Phlebotomy! Want Hct <45%
Low dose aspirin
Cytoreductive agents: Hydroxyurea* (brings all cell counts down!)
-Prevent it from progressing to primary myelofibrosis

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13
Q

What is essential thrombocythemia (primary thrombocytosis)

A

Plt: 1 million! (can dx at >450)
BM Bx: normocellularity w/ prominent megakaryocytes
JAK2 mutation
-R/o iron deficiency
-Increased risk for hemorrhage and thrombosis

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14
Q

How do you treat Essential Thrombocythemia

A

Low dose ASA

Hydroxyurea

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15
Q

What is CML

A

Overproduction of myeloid cells (WBC 100-200)
MC in middle aged
*Philadelphia chromosome associated

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16
Q

What are the phases of CML

A

Chronic stable: MC. if not Tx, can progress to blast phase
Accelerated: increased blasts. will progress to blast phase in 4-6 months
Blast phase (crisis): survive 1-2 years. May see ALL or AML

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17
Q

How does CML present

A

Fatigue
night sweats
low grade fever
abd pain (2/2 splenomegaly)

18
Q

CML labs will show

A
High WBC (100-200) 
low LAP score 
Presence of BCR or ABL gene 
<5% blasts 
Dont need BM Bx, but it is strongly required
19
Q

How do you treat CML

A

Allogenic stem cell transplant may cure if pt is really sick!
Tyrosine Kinase Inhibitors for disease control: Gleevec* (imatinib) may decrease need for transplant in CML pts
Palliative care w/ cytotoxics: Hydroxyurea (IF + cytarabine have more ADE)

20
Q

What are the types of transplant you can get

A

Allogenic: from others
Autologous: from yourself after it has been cleaned

21
Q

How does Gleevec work in CML

A

Inhibits tyrosine kinase which may be an abnormal defect in philadelphia chromosome

22
Q

What is non-classic MPN

A

Chronic neutrophilic leukemia
hypereosinophilic syndrome (HES)
chronic eosinophilic leukemia (CEL)
-Basically MPN W/O: BCR-ABL gene

23
Q

What do you need to rule out in HES/CEL

A

Parasitic infection!

You suspect it because both have elevated eosinophils, which indicate parasite infx

24
Q

What happens to cells in acute leukemia

A

Progenitor cells lose the ability to differentiate
The worse acute leukemia prognosis is if it arises from MDS or MPN
Can also be caused by benzene and cytotoxic agents (melphalan, etoposide- chemo drugs)

25
Q

Differentiate AML vs ALL

A

AML: MC in elderly, + Auer Rods*
ALL: MC in kids, peak 3-7 y/o

26
Q

Both AML and ALL have

A

*Pancytopenia w/ circulating blasts
Leukocytosis >200 (paradoxical infections)
*Leukostasis (med emergency, decreased tissue perfusion in pulmonary and cerebrovascular)
-Spleen enlargement, purple petechial rash, bone pain, SOB, LAD

27
Q

What is specific to ALL that is really bad

A

Crosses the BBB so you can have CNS Symptoms

28
Q

What are some characteristics of AML

A

Incidence increases w/ age! Mean 65 y/o
Caused by: Radiation, chemicals, drugs, Down Syndrome*, Klinefelter syndrome
Auer Rods are present

29
Q

How do you treat AML

A

Young: treat to cure and try to obtain complete remission- give Chemo! Anthracycline and Cytabine
+/- Consolidation chemo or SCT if candidate
Allogenic SCT- cures 60% of younger

30
Q

What are some characteristics of ALL

A

MC in kids 3-7

Cytogenics determine prognosis

31
Q

How do you treat ALL

A

Chemotherapy: danurubicin, vincristine, prednisone, and asparginase
+/- followed by SCT if needed
IC chemo only for CNS prophylaxis

32
Q

What are some characteristics of CLL

A

B lymphocyte malignancy
CLL Mc than ALL
Found incidentally on CBC (may have fatigue and LAD, and splenomegaly)
Associated with Richter’s transformation (high grade lymphoma) and Smudge cells

33
Q

What are some causes of CLL

A

Abn response to antigenic stimulation
Cytogenic abn
Genetic changes
Microenvironmental changes

34
Q

How do you treat CLL

A

Dont need to know

35
Q

What is aplastic anemia

A

Life threatening pancytopenia caused by drugs, radiation, infection, or inherited
Bone marrow just does not work
Present with bleeding and infection
MC at 25 y/o

36
Q

Drugs that can cause aplastic anemia are

A

Chloramphenicol
Benzene
Toluene
Chemotherapy

37
Q

On PE for aplastic anemia you may find

A

pallor
purpura
petechiae
NO LAD or splenomegaly

38
Q

What is the Mc form of inherited aplastic anemia

A

Fanconi anemia

39
Q

How do you treat aplastic anemia

A

Allogenic SCT or immunosuppression

40
Q

What is hairy cell leukemia

A
Uncommon B cell lymphoproliferation MC in men 
Patients have palpable splenomegaly 
CBC shows pancytopenia w/low monocytes 
BM Bx shows "hairy cells" 
Most pts live >10 years!
41
Q

In a patient with CLL what would you expect to see on CBC

A

Lymphocytosis

Leukocytosis

42
Q

What test would you use to rule in suspected CML

A

BM Bx