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Flashcards in WBC Disorders Cases Deck (34):
1

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?

20%

2

What types of infections cause neutrophilia?

Bacterial -- like streptococcal tonsillitis

3

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

Lymphocytes

Bordetella Pertussis causes a leukemoid reaction that results in a lymphocytosis

4

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

Indicates the presence of primary granules

--usually associated with an inflammatory process (sepsis)

5

CML

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

6

CML what does the BM look like?

Hypercellular with effective hematopoiesis

7

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

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

8

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

A constitutively active tyrosine kinase

9

What are the three phases of CML?

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

10

What is the treatment for CML?

Imatinib

11

What is Imatinib fails?

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

12

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

The patient will likely progress to acute leukemia

13

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

Polycythemia Vera

14

What are some clinical findings of PV?

HSM
Ruddy faces
H/a
Budd-Chiari syndrome
Gout

15

Is EPO decreased or increased in PV? Why?

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

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

Most likely Acute Myeloid Leukemia
Specifically Acute Promyelocytic Leukemia

17

What cytogenetic abnormality is commonly assoc with APL?

t(15;17)
Produces PML-RARa fusion product

18

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

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

19

What is the common therapy for PML?

ATRA (all-trans retinoid acid)

20

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

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

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

21

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

Myelodysplastic Syndrome

22

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

The bone marrow blast percentage

>20% = AML
<20% = MDS

23

What are some chromosomal abnormalities associated with MDS?

Monosomy 5 or del5q
+8
Monosomy 7 or del7q

In 50% of patients

24

What is the therapy for MDS?

Supportive care + growth factors
SCT

Hypomethylating agents
Lenalidomide
Histone Deacetylase Inhibitors

25

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

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

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

CD34, CD117, CD13, CD33

27

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 +

AML

28

Common clinical presentation of acute leukemias

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

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

29

What are some good prognostic cytogenetic classifications of AML?

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

30

What are some BAD prognostic cytogenetic classifications of AML?

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

31

What is the therapy for AML?

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

32

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

Primary myelofibrosis (PMF)

33

What common mutation is associated with PMF?

JAK2 mutation (in about 50%)

34

What is a possible treatment being explored for PMF?

JAK2 inhibitors