120214 intro to WBC disorders

Question 1

leukemoid rxn

Answer 1

benign, exaggerated response to an infec
absolute leukocyte count over 50,000/uL
may involve neutrophils, lymphocytes, or eosinophils

Question 2

etiology of leukemoid rxn

Answer 2

perforating appendicitus (neutrophils)
whooping cough (lymphocytes)
cutaneous larva migrans-nematodes (eosinophils)

Question 3

neutrophilia

Answer 3

neutrophil count over 7,000/uL

Question 4

causes of neutrophilia

Answer 4

infection
sterile inflam w necrosis (acute MI)
drugs

Question 5

causes of neutropenia

Answer 5

chemo
aplastic anemia
immune destruction (lupus)
septic shock

Question 6

eosinophilia etiology

Answer 6

type I hypersensitivity
invasive helminths
hypocortisolism
neoplasms (Hodgkin’s lymphoma)

Question 7

basophilia etiology

Answer 7

CML (and other chronic myeloproliferative neoplasms)

chronic kidney disease

Question 8

leukemia

Answer 8

proliferation of neoplastic cells primarily in bone marrow and peripheral blood

Question 9

lymphoma

Answer 9

proliferation of neoplastic cells primarily in LNs and extramedullary lymphoid tissue

Question 10

myeloid neoplasms–list them

Answer 10

myeloproliferative neoplasms
meylodysplastic syndromes
acute myeloid leukemia

Question 11

MPN-what do you see in bone marrow

Answer 11

hypercellular with effective hematopoeisis (increased peripheral blood granulocytes, RBCs and/or platelets)

Question 12

general features of MPN

Answer 12

hypercellular bone marrow w effective hematopoeisis
splenomegaly or hepatomegaly

potential for progression (bone marrow fibrosis or acute leukemia)

Question 13

MPN vs MDS –their similarities

Answer 13

both are due to deranged stem cell

both–see hypercellular bone marrow (lot of precursors in bone marrow)

Question 14

MPN vs MDS-differences?

Answer 14

MPN is hypercellular BM with effective hematopoiesis

MDS is hypercellular BM with INeffective hematopoiesis (decreased periph bl counts). why does this happen? b/clonal abnormalities in bone marrow promote cell death

Question 15

ex of myeloproliferative neoplasms

Answer 15

CML, BCR-ABL positive
polycythemia vera
primary myelofibrosis
essential thrombocythemia

Question 16

BCR-ABL fusion gene produces what?

Answer 16

protein with increased tyrosine kinase activity

Question 17

what cytogenetics is associated with CML?

Answer 17

philadelphia chromosome t(9;22) –BCR-ABL

Question 18

basophilia is seen in

Answer 18

CML

Question 19

therapy for CML

Answer 19

BCR-ABL tyrosine kinase inhibitors:

Gleevec (imatinib mesylate)

Question 20

polycythemia vera

Answer 20

increase in RBCs, granulocytes, platelets

Question 21

what cytogenetics is assoc w polycythemia vera?

Answer 21

JAK2 mutation (JAK pathway promotes proliferation and survival)

Question 22

clinical findings for PV

Answer 22

splenomegaly
thrombotic events b/c of hyperviscosity
gout (increased uric acid b/c of increased cell breakdown)
signs of increased histamine (mast cells)-ruddy face, pruritis after bathing, peptic ulcer disease

Question 23

lab findings for PV

Answer 23

increased RBC mass

decreased EPO

Question 24

primary myelofibrosis

Answer 24

rapid development of BONE MARROW FIBROSIS and EXTRAMEDULLARY HEMATOPOIESIS in spleen, liver, LNs

Question 25

clinical findings for primary myelofibrosis

Answer 25

splenomegaly | splenic infarcts with left sided pleural effusions

Question 26

what do you see in BM with primary myelofibrosis

Answer 26

fibrosis and clusters of atypical megakaryocytes

Question 27

what is assoc w JAK2 kinase mutation

Answer 27

PV essential thrombocythemia myelofibrosis

Question 28

teardrop cells

Answer 28

myelofibrosis

Question 29

essential thrombocythemia

Answer 29

neoplastic stem cell disorder with proliferation of megakaryocytes. hypercellular BM with abnormal megakaryocytes

Question 30

peripheral blood of essential thrombocythemia

Answer 30

large hypogranular platelets

Question 31

tx for essential thrombocythemia

Answer 31

alkylating agents or similar drugs to lower platelet count

Question 32

myeloblasts for myelodysplastic syndromes (peripheral blood or bone marrow)

Answer 32

under 20%

Question 33

MDS is due to

Answer 33

chromosomal abnormalities in 50% cases

Question 34

findings for MDS

Answer 34

cytopenias (uni, bi, or pan) dysplastic features hypercellular bone marrow ring sideroblasts (iron around nucleus)

Question 35

monosomy 7

Answer 35

MDS

Question 36

MDS progresses to what in 30% of cases

Answer 36

AML

Question 37

therapy for MDS

Answer 37

supportive-blood products, antibiotics, growth factors hypomethylating agents-decitabine, azacitidine allogeneic stem cell transplant

Question 38

acute leukemia

Answer 38

neoplastic proliferation of immature cells (blasts)

Question 39

ALL is most common in

Answer 39

children, but may be seen in adults

Question 40

compare AML and ALL blasts

Answer 40

see slide 80

Question 41

Auer rods

Answer 41

in 60-70% of AML cases

Question 42

how to put cytochemical stains to use?

Answer 42

MPO--in myeloblasts | NSE (non specific esterase)--monocytic blasts in AMLs with monocytic differentiation

Question 43

markers of immaturity

Answer 43

useful for acute leukemia CD34 (AML and ALL) TdT (mostly seen in ALL) CD117 (AML) CD1a (restricted to immature T cells)

Question 44

general outcome for AML

Answer 44

poor

Question 45

clinical features of AML

Answer 45

related to cytopenias--weakness, fatigue, petechiae, infections

Question 46

diagnostic criterion for AML

Answer 46

greater than 20% myeloid blasts in blood or marrow

Question 47

acute leukemia--what happens to blasts' number in BM and blood stream?

Answer 47

increased blasts make it harder to have normal hematopoiesis in BM blasts normally go into the blood stream--causing increased WBC count

Question 48

AML with recurrent cytogenetic abnormalities--prognosis?

Answer 48

generally favorable

Question 49

ex of recurrent cytogenetic abnormalities for AML

Answer 49

t(8,21) inv(16) t(15;17) the above all have favorable prognoses 11q23 rearrangment--intermediate or unfavorable

Question 50

acute promyelocytic leukemia

Answer 50

AML with t(15;17) Faggot cells (single cells with multiple Auer rods) there's risk for DIC responds to all-trans retinoic acid (ATRA)

Question 51

Langerhans cell histiocytosis

Answer 51

histiocytic neoplasm ``` CD1a, langerin--typical markers Birbeck granules (tennis racket appearance on EM) ```

Question 52

hemophagocytic lymphohistiocytosis/hemophagocytic syndrome

Answer 52

hyperinflam condition primary HLH secondary HLH: EBV, lymphomas VERY HIGH FERRITIN