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Flashcards in WBC Disorders Cases Deck (34)
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1
Q

In order to make a diagnosis of acute leukemia, how many blasts (at a minimum) would you have to see on PB (or BM) smear?

A

20%

2
Q

What types of infections cause neutrophilia?

A

Bacterial – like streptococcal tonsillitis

3
Q

What kind of cell type is likely to be increased in the PB with whooping cough?

A

Lymphocytes

Bordetella Pertussis causes a leukemoid reaction that results in a lymphocytosis

4
Q

What is the significances of “toxic change” in the peripheral blood neutrophils?

A

Indicates the presence of primary granules

–usually associated with an inflammatory process (sepsis)

5
Q

CML

A

Middle aged (40-60)
Extreme neutrophilia
Fatigue, wt loss, splenomegaly, LAD
Labs: WBC = 10K-500K, basophilia, myeloblasts < 10%, decreased LAP

6
Q

CML what does the BM look like?

A

Hypercellular with effective hematopoiesis

7
Q

What is a common (95%) cytogenetic abnormality in CML?

A

t(9;22) BCR-ABL fusion geen
Philidelphia chromosome
Not specific to CML

8
Q

What is the defect associated with BCR-ABL fusion gene?

A

A constitutively active tyrosine kinase

9
Q

What are the three phases of CML?

A
Chronic phase (median survival 3-5 years)
Accelerated phase (12-24 months)
Blast phase (3-6 months)
10
Q

What is the treatment for CML?

A

Imatinib

11
Q

What is Imatinib fails?

A

SCT

Or new oral tyrosine kinase inhibitors: Dasatinib, Nilotinib, Bosutinib, Ponatinib

12
Q

If a patient stops taking CML meds what will likely happen?

A

The patient will likely progress to acute leukemia

13
Q

Which myeloproliferative neoplasm has a JAK2 mutation in nearly ALL cases?

A

Polycythemia Vera

14
Q

What are some clinical findings of PV?

A
HSM
Ruddy faces
H/a
Budd-Chiari syndrome
Gout
15
Q

Is EPO decreased or increased in PV? Why?

A

EPO is decreased with sustained increased in HCT/Hb
Normal SaO2

Marrow is doing it’s own thing producing a lot of RBC’s independent of EPO production

16
Q
Patient presents with weakness, bleeding from nose and mouth
Petechiae and ecchymoses
WBC: 50,000/uL
Increased D-dimer, PT and PTT 
Consistent with DIC
A

Most likely Acute Myeloid Leukemia

Specifically Acute Promyelocytic Leukemia

17
Q

What cytogenetic abnormality is commonly assoc with APL?

A

t(15;17)

Produces PML-RARa fusion product

18
Q

What functional defect is associated with product of PML-RARa fusion gene?

A

A block in terminal differentiation due to retinoic acid receptor disruption
RA is important for myeloid maturation – arrest at promyelocyte stage

19
Q

What is the common therapy for PML?

A

ATRA (all-trans retinoid acid)

20
Q

Which cytogenetic abnormality has a similar prognostic significance to t(15;17) in a patient with AML?

A

t(8;21)*** answer
inv(16)
t(15;17)

All have favorable prognoses
11q23 has an intermed/unfavorable prognosis

21
Q

76 yo female with weakness and pancytopenia
Hypercellular BM with frequent hypogranular and hypolobated neutrophils
Small, hypolobated megakaryocytes

A

Myelodysplastic Syndrome

22
Q

What is the most important diagnostic criterion for differentiating a myelodysplastic syndrome from an acute myeloid leukemia with myelodysplasia-associated changes?

A

The bone marrow blast percentage

>20% = AML 
<20% = MDS
23
Q

What are some chromosomal abnormalities associated with MDS?

A

Monosomy 5 or del5q
+8
Monosomy 7 or del7q

In 50% of patients

24
Q

What is the therapy for MDS?

A

Supportive care + growth factors
SCT

Hypomethylating agents
Lenalidomide
Histone Deacetylase Inhibitors

25
Q

What is the name of the hypomethylating agent we should know? What is their mechanism of action?

A

Decitabine

HYPER methylation blocks gene transcription and allows cell to divide (silences genes)
HYPO methylation blocks DNMT and prevents methyl group binding and allows gene expression

26
Q

What are blasts in MDS likely to be positive for on flow cytometry?

A

CD34, CD117, CD13, CD33

27
Q
50 yo male with fatigue
Hb: 9
MCV: 100
Plt: 30,000
WBC: 80,000
Blasts: 60,000
Normal fibrinogen 

Blood smear shows Auer bodies
Flow cytometry: CD13, CD33, CD34, CD117, MPO +
HLAl-DR +

A

AML

28
Q

Common clinical presentation of acute leukemias

A

HSM, PAINLESS LAD, skin involvement, testicular involvement, bone pain
THROMBOCYTOPENIA

ALL (children <20)
AML (adults 30-60)

29
Q

What are some good prognostic cytogenetic classifications of AML?

A

t(8;21)
inv(16)
t(15;17)
Or with FAB type M3

30
Q

What are some BAD prognostic cytogenetic classifications of AML?

A

Any patient with adverse karyotypic abnormalities (-5,-7, del(5q), abn(3q), complex or resistant disease

31
Q

What is the therapy for AML?

A

Induction: 7 + 3 with Ara-C + Daunorubicin
Consolidation: HIDAC or Ara-C

32
Q
60 yo male
Fatigue and night sweats
Hemoglobin 11 gm/dl 
Exam: spleen palpable 4cm at MCL 
Labs: otherwise unremarkable 
Teardrop cells
A

Primary myelofibrosis (PMF)

33
Q

What common mutation is associated with PMF?

A

JAK2 mutation (in about 50%)

34
Q

What is a possible treatment being explored for PMF?

A

JAK2 inhibitors