Path 2 White Cell Disorders

Front 1

What are the blue cytoplasmic inclusions and what does it indicate?

Back 1

Dohle Bodies (rER remnants) due to left shifts

Front 2

What is occurring with this cell? why?

Back 2

Toxic Granulation- due to impaired cytoplasmic maturation due to Left shift

Front 3

What infection is characterized by these large atypical B cells infected with Epstein-Barr virus?

Back 3

Infectious Mononucleosis

What helps clear the infected cells?

Hint 3

cytotoxic/suppressor CD* (+) T cells

Front 4

Classify this reactive lyphoid hyperplasia

Back 4

Paracortical hyperplasia

main cell type?

Hint 4

T cells

Front 5

Most common cause of basophilia?

Back 5

CML

Front 6

define Leukoerythroblastosis

Back 6

Leukomoid reaction with addition of nucleated red blood cells.

cause?

Hint 6

myelofibrosis of BM due to metastatic cancer or hemoatologic malignancy leading to extramedullary hematopoesis

Front 7

define chronic granulomatous disease

Back 7

lethal sex-linked disorder where neutrophils can phagocytose but not kill due to impaired respiratory burst oxidase system -> chronic infections

Front 8

reactive follicular hyperplasia may closely resemble what cancer??

Back 8

metastatic cancer or follicular lumphoma

main cell type

Hint 8

B cell

Front 9

Define this reactive lymphoid hyperplasia

predominant cell type?

Back 9

sinus hyperplasia

T cells

Front 10

define the cell type- increased numbers of these cells are due to?

Back 10

eosinophils- allergic rxns, parasites, CML, tumors, Hodgkin's lymphoma

Front 11

define the cell type- increased numbers of these cells are due to?

Back 11

basophil- allergic reaction, CML, and other MPDs, hypothyroidism, splenectomy

Front 12

congenital neutropenia results in?

Back 12

cyclic neutropenia- abnormality of feedback mechanisms

Front 13

DD of neutrophilia in leukocytosis?

Back 13

bacterial infection, inflammation, emotions, drugs, neoplasm, hematologic abn

Front 14

Major difference between acute and chronic leukemia

Back 14

Acute Leukemia- uncontrolled blast proliferation

Chronic leukemia- uncontrolled proliferation of mature cells

Front 15

acute lymphoblastic vs myeloid leukemia- age? categorized by?

Back 15

ALL- children, B/Tcell (phenotype)

AML- adults, chromosomal translocations

Front 16

Stages of lymphomas

Back 16

I- one lymph node
II- 2+ lymph nodes, same side diaphragm
III- opp sides of diaphragm
IV- extranodal site

Front 17

T cell markers

Back 17

CD 1,2,3,4,7,8

Front 18

B cell markers

Back 18

CD10 and up

Front 19

what type of non-Hodgkin lymphoma has the highest incidence?

Back 19

B-cell

Front 20

what is needed for the diagnosis of acute leukemia?

Back 20

blasts in periph blood, 20%blasts in BM

Front 21

three types of acute leukemias

Back 21

myeloid (AML), B lymphoblastic & T-lymphoblastic (ALL)

Front 22

what is the most common childhood cancer? peak ages?

Back 22

ALL-

Bcell type (85%) ~3yrs
Tcell type (15%) adolescence, male

Front 23

How do you differentiate between T and B-ALL?

Back 23

immunophenotype (not morphology!)

Front 24

phenotypic detection of B-ALL and early/late differentiation

Back 24

CD19 (pan B-cells)

early= no CD10
late= CD10, CD20 & cytoplamic IgM heavy chain

Front 25

Good prognosis factors for B-lymphoblastic leukemias

bad prognosis factors

Back 25

2-10 yrs
low White Cell Count
hyperdipoidy (>50 chrom)
t(12;21) TEL-AML1 genes

Hint 25

<2/adolescence/adult
high WCC
t(9;22) BCL-ABL (phili chrom)
11q23 translocations (MLL)- associated w/ age<2
hypodipoid (<44 genes)

Front 26

Most common lymphoblastic lymphoma?

Back 26

T-LBL (85-90%)

most common in what patient pop?

Hint 26

adolescent males

Front 27

phenotypic markers for T-lymphoblastic leukemias

Back 27

CD1,2,5,7

early vs late markers

Hint 27

late- (+) for CD 3,4,8

Front 28

(T/F) cytogenetic abnormalities do not play a prominent role in the prognosis of B-ALL

Back 28

False- they do not play a prominent role in the prognosis of T-ALL

what does?

Hint 28

age, stage, LDH levels

Front 29

common cytogenetic abnormalities in T-lymphoblastic leukemias

Back 29

mutation of NOTCH 1 gene (normal T-cell development)

T cell receptor gene translocations

Front 30

main cell type in Burkitt's leukemia/lymphoma, markers? appearance?

chromosomal abnormalities?

treatment?

Back 30

mature B-cells with Blastic morphology, CD20 & CD10/BCL6 germinal center markers, vacuolated cytoplasm

t(8;14)

treated as an acute leukemia

Front 31

clinical presentation of AML

Back 31

fatigue, bleeding, infections, no lymph node enlargement, granulocytic sarcomas, skin and gingival involvement, DIC

Front 32

AML with Good prognosis, presents young, "AML with maturation"

Back 32

t(8;21)

Front 33

AML with Good Prognosis

Back 33

inv(16)

Front 34

AML with the best Prognosis, inc risk of DIC, and is treated with ATRA

Back 34

t(15;17) characterized by promyelocytes

ATRA= all-trans retinoic acid

Front 35

AML characterized by intermediate prognosis with t(9;11) as the best one to have

Back 35

11q23 abnormalities

Front 36

AML characterized by poor prognosis

Back 36

MDS- related changes with dysplasia (hypolobulation/granulation)

Front 37

AML can result from cancer therapy such as

Back 37

alkylating agents (cyclophosphamid), ionizing radiation, and topoisomerase II inhibitors (doxorubicin)

Front 38

Compare AML and ALL:
age-
blasts w/ granules-
MPO, non-sp esterase-
tdt-
Lymph node and organ involvement-
cytogenetic importance-

Back 38

AML:
age- adults
blasts w/ granules- yes
MPO, non-sp esterase- (+)
tdt- (-)
Lymph node and organ involvement- no
cytogenetic importance- yes

Hint 38

ALL:
age- kids
blasts w/ granules- no
MPO, non-sp esterase- (-)
tdt- (+)
Lymph node and organ involvement- yes
cytogenetic importance- in B-ALL

Front 39

cytogenetic abnormality associated with Burkitt's leukemia/lymphoma?

Back 39

t(8;14)

Front 40

Precautions needed when treating Burkitts lymphoma

Back 40

-treat spinal fluid due to high CNS relapse
-b/g w/ chemo reduction b/c of high lysis risk
-b/g on low chemo doses to avoid tumor breakdown products overwhelming kidney -> renal failure
-high cure rate
-use CD20 therapy targets

Front 41

8 yr old boy presents with frequent respiratory infections, enlarged lymph nodes, hepatosplenomegaly, and CD (+) 2,3,5, & 7

Back 41

T-ALL

Front 42

11q23 rearrangement and prognosis of ALL and AML

Back 42

poor prognostic of ALL
intermediate prognostic in AML

Front 43

which AML chromosomal abnormalities are associated with a lower relapse rate?

Back 43

inv (16) and t(8;21)

Front 44

Myelodysplastic syndrome (MDS) has a high risk of transformation to what?

Back 44

AML

Front 45

Difference between primary and secondary MDS

Back 45

Primary= de novo
secondary= to prior genotoxic exposure (chemotherapy)- most likely to progress to AML

Front 46

MDS- chromosomal abnormalities: Good/bad/worse prognosis

Back 46

Good- 5q-
Bad- monosomy 5 or 7, 7q or 20q deletions, trisomy 8
worse- complicated karyotype

Front 47

4 Examples of Dysplastic Morphologic Changes in MDS

Back 47

1) erythoid- ringed sideroblasts, megaloblastoid
2)bilobed neutrophils
3)megakaryocytes- hypo/ersegmented/ & giant platlets
4)myeloid blasts w/ auer rods

Front 48

Morphologic change of MDS that holds the highest risk of transformation to AML

Back 48

Auer rods

Front 49

define preleukemia

Back 49

refractory anemia with excess blasts (>5% but <20%)

Front 50

main molecular cause of MPN's?

Back 50

mutated tyrosine kinase genes (JAK2 or BCL/ABL)

Front 51

Cells predominantly elevated in CML

Back 51

neutrophilic granulocytes

Front 52

Cells predominantly elevated in PMF

Back 52

megakaryocytes wich induce fibroblasts to make collagen

(primary myelofibrosis)

Front 53

Cells predominantly elevated in PV

Back 53

RBC precursors

(Polycythemia rubra vera)

Front 54

Cells predominantly elevated in ET

Back 54

megakaryocytes and platlets

(essential thrombocythemia)

Front 55

What is Gleevec?

Back 55

protein-tyrosine kinase inhibitor (competitive inhibitor of bcr-abl TK)

Front 56

mutation associated with polycythemia vera?

Back 56

JAK2

Front 57

treatment for PV (polycythemia vera)

Back 57

phlebotomy (remove excess RBC's), mild chronic chemotherapy (hydroxyurea therapy), and mild radionucleotide therapy (radioactive Fe damages RBC precursors in BM)

Front 58

PMF (primary myelofibrotic) patients are at risk for what?

Back 58

infection, hemorrhage, thrombosis, and progression to AML

Front 59

neoplastic megakaryocytes in PMF secrete what factors?

Back 59

platelet derived growth factors and TGF-beta (both stimulate fibroblasts)

Front 60

CLL/SLL
age-
symptoms-
lymph node-
smear abnormalities-
lymph node-

Back 60

age= over 60
symptoms=
fatigue, wt loss, anorexia,
lymphadenopathy & hepatosplenomegaly, hypogammaglobinemia, inc infection,
autimmune hemolytic anemia/ thrombocytopenia

Hint 60

lymph node- diffuse infiltration with proliferation centers with larger activated lymphocytes

-smear= smudge cells!

Front 61

CLL
phenotype

Back 61

B cell markers (CD19, 20, 79a)
CD5 & CD23 coexpression
dim surface Ig
ZAP-70 associated with worse prognosis

Front 62

CLL cell of origin

Back 62

pre-germinal naive B cell - worst prognosis
post-germinal B-cell (memory cell) - better prognosis

Front 63

CLL- Good/Bad genetic prognostic factors

Back 63

Good- 13q14.3
Bad- 11q22-23, 17p

Front 64

What two neoplams can progress to diffuse large B-cell lymphoma?

Back 64

CLL and follicular lymphoma

Front 65

Follicular lymphoma
age/gender-
symptoms-
lymph node-

Back 65

middle age- no sex predilection
painless lymphadenopathy
expanded germinal centers, no mantle zone, deminished interfollicular zones

Front 66

Follicular lymphoma
types of cells, translocation,

and phenotype

Back 66

centrocytes and centroblasts (small cleaved cells and large non-cleaved cells with nucleoli)

t(14;18) 14=IgH, 18=Bcl-2

Hint 66

phenotype-
B cell markers- CD19, 20, 79a
GC markers- CD10, Bcl-6
antiapoptotic- Bcl-2

Front 67

Diffuse B-cell lymphoma
age-
associated with?
cells of origin?

Back 67

60 years
Infection with HIV (EBV), or follicular lymphoma/ CLL
origin- GC's, post-GC cells

Front 68

Diffuse B-cell lymphoma
phenotype
cytogenetics

cells of origin
clinical features

Back 68

B-cell markers (CD19,20 79a)
variable GC markers CD10 & Bcl-6

t(14;18), or dysregulation of Bcl-6 or p53

Hint 68

rapidly growing, nodal or extra-nodal, BM involvement is rare

GC or Post GC cells

Front 69

difference in chemotherapy between follicular lymphoma and diffuse lg B-cell lymphoma

Back 69

folliuclar= low dose, diffuse requries high dose

Front 70

Burkitt Lymphoma-
unique morphology?
cell markers
genetics

age
sites of mass?

Back 70

starry sky- vacuolated cytoplasm
B-cell markers (19,20,79a), GC marks (CD10, Bcl-6)
t(8;14) increases c-myc

Hint 70

kids/young adults

mandibular mass or abdominal mass

Front 71

aggressive but curable NHLs?

indolent but incurable?

Back 71

Burkitt, DLBL

Hint 71

CLL, follicular

Front 72

What is the cause of lytic bone destruction in MM

Back 72

cytokine IL-6 released by tumor and BM stroma that stimulate osteoclasts

Front 73

symptoms of mm

Back 73

CRAB
hypercalcemia, renal impairment, anemia, bone disease

Front 74

difference between MGUS and multiple myeloma?

Back 74

MGUS had ,10% plasma cells in BM, serum M protein , 3g/dl, asymptomatic, no therapy required

Front 75

waldenstrom macroglobulinemia is a complication of what neoplasm? due to? leads to?

Back 75

multiple myeloma- due to IgM, leads to hyerviscosity, visual disturbances, confusion, CHF

Front 76

Reed sternberg cells

Back 76

binucleated cells classic of hodgkin lymphoma, secrete IL-5, which attracts inflammatory cells (eosinophils)

Front 77

Hodgkin Lymphoma
age-
differences from non-hodgkin-

Back 77

young adults and over 50

single group of nodes, contiguous spread, no mesenteric nodal involvement, extranodal presentation rare

Front 78

difference between classical HL and nodular lymphocyte predominant HL

Back 78

classical: CD15+, CD30+, CD20-
nodular:CD15-, CD30-, CD20+
popcorn cells!

Front 79

Four types of Classical hodgkin lymphoma

Back 79

nodular sclerosis- fibrous bands!
mixed celluarity
lymphocyte rish
lyphocyte depleted