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Heme and Lymph > WBC III > Flashcards

WBC III Flashcards

1
Q

pseudo pelger huet cells, nuetrohils with only 2 nucelear lobes instead of 3-4

A

MDS

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2
Q

AML with MDS like features

  • with prior MDS which has now transofmred
  • without prior MDS prognosis of each
A

very poor

poor

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3
Q

hairy cell leukemia clinical manifestations

increased incidence of what

A

infiltration of BM, liver and spleen

  • splenomegaly often massive is most common abnormal physical finding
  • hepatomegaly less freq
  • lymphadenopathy rare
  • pancytopenia in over 50% of cases, 1/3 have infections, incresaed incidence of atypical mycobacterial infection from monocytopenia
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4
Q

which types of HL not assocaited with EBV

A

sclerosing

lymphocytre predominance

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5
Q

punched out defects of bones

A

MM

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6
Q

AML, M0

A

minimially differentiated
MPO negative
blasts lack definitive cytologic markers of myeloblast
express myeloid lineage antigens

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7
Q

prognosis of CML

A

better with philadelphia chrom
3 yr survival without treatment
50% of pts enter accelerated phase after 6-12 motnsh enter blast crisis, most AML like some ALL like (pooer prognosis)
-other 50% develop abrupt blast crisis without accelerated phase

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8
Q

what CD marker do ALK tumors usually have

A

CD30

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9
Q

what are the MPD

A

CML
PV
ET
Progressive myelofibrosis (PM)

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10
Q

tisse infiltration may be prominent with what AML

A

monocytic

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11
Q

MDS and MPD often do what

A

transform to AML

worse prognosis with MDS bc cytopenia sets up for infection and more severe disease

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12
Q

clnical ET

A

mainly adults over 60
onset insidious
increased prolif megakaryocytes with high periph platelet counts

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13
Q

lymphocyte depletion

A

EBV common
predominantly in elderly, in HIV+ of any age, and in non industrialzied countries

poor prognosis

paucity of bacground reactive cells

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14
Q

who gets mantle cell lymphoma most often

A

5th and 6th decades of life and shows male predominance

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15
Q

variants of mantle cell lymphoma

A

blastoid variant and a proliferative expression profiling signature are associated with even shorter survivials

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16
Q

diagnosis ET

A

bone marrow biopsy

megkarycytes high in number clustering together

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17
Q

cytologic variants from dysregulated synthesis and secretion of Ig which leads to accumulation of this protein in MM which cells

A

flame cells
mott cells (grapelike cytoplasm, motts grapjuice)
cells with firbils, crystalline rods and globules (russel bodies if cytoplasmic) or (dutcher bodies if nuclear)

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18
Q

granulocytic sarcoma

A

an AML rarely presenting as localized primary tumor mass (orbital granulcytic sarcoma)

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19
Q

most myelomas are associated with more than ___ serum Ig and or more than ____ of urinary bence jones proteins

A

3 gm/dL

6mg/dL

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20
Q

HL or NHL has mesenteric nodes and walderyer ring involvement

A

NHL

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21
Q

tumor cells in mycosis fung/sezary syndrome express what

A

CLA
CCR4
CCR10

all bring cd4+ cells to skin

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22
Q

clinical PV,
onset
symproms
hematocrit

A

adults late middle age
insidious onset, cyanosis, intense pruritius, HA, HTN, GI ulcer
splenomegaly in late stage
hematocrit over 55% in periphery
hyperviscosity, major bleeding and thrombotic episodes
leukocytosis
hyperuricemia

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23
Q

clinical features of mantle cell lymphoma

A

painless lymphadenopathy

spleen and gut symptoms comon

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24
Q

LCH

2) uniocal and multifocal unisystem

A

eosinophilic granuloma
langerhans cells with mixed eosniophils, lymphocyts, plasma cells, neutorphils

unifocal: skeletal systemi in older children mainly calvarium, ribs, femur

multifocal: multiple erosive bony masses young children
50% have diabetes insipidus and hand schuller christian triad (DI, exophthalmos, calvarial bone defects)

treatment: excision in unifocal, chemo if multifocal

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25
Q

if monocytic differentiation of AML then positive for

A

non specific esterase

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26
Q

3 broad categories of myeloid neoplasias

A

AML, in which ccumulation of immature myeloid blasts in bone marrow suppress normal hematopoiesis
(20% myeloid blasts in bone marrow at least)

myelodysplastic syndomes: ineffective hematopoiesis–>cytopenias

MPD–>increaed production of one or more types of commited/mature white/red cells of myeloid series

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27
Q

immunophenotype of mantle cell lymphoma

A

high level cyclin D1
most tumors have CD19 and 20
usually CD5+ and CD23- to distinguish from CLL/SLL

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28
Q

CML clinical

A 
primarily adult males at 50
onset insidious
non specific symptoms
splenomegaly bc of EMH
LUQ pain with splenic infarct
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29
Q

what is blast crisis

A

MPD then start seeing superimposition of blast that accumulates over 20% in BM, then pt with AML that has arise now

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30
Q

AML with t(15;17) prognosis

A

intermediate

APL, DIC

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31
Q

LPL bone marrow biopsy

A

mixture of small lyphoid cells with varying degree of plasma cell diff

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32
Q

MPD cells

A

primary neoplastic cell is usually multipotent and gives rise to mature erytrhocytes, platelets and granulocytes which may be high in peripherl blood

less commonly pleuripotent stem cells gives rise to both lymphoid and myeloid cells

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33
Q

first sign of PV

A

25% with DVT, MI, stroke

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34
Q

mutations in hairy cell leukemia

A

BRAF

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35
Q

smoldering myeloma

A

uncommon variant of MM defined by lack of symptos and high plamsa M component greater than 3 gm/dL
-75% pts progress to MM over 15 yr period
plasma cells make up 10-30% of marrow cellularity

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36
Q

RS ells release cytokines and chemokines that induce accumulation of

A

reactive lymphocytes, macrophages, and granulocytes

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37
Q

megakaryocytes with multiple nuclei instead of normal single multilobulated nucleus

A

MDS

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38
Q

what are the plasma cell neoplasms/dyscrasias and related disorders

A

multiple myeloma
waldenstrom macroglobulinemia
heavy chain disaease
primary or immunocyte assocaited amyloidosis
monoclonal gammopathy of undeterined significance

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39
Q

average age of diagnosis for HL, curable?

A

32, curable in most cases

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40
Q

prognosis of mycosis fungoides/sezary syndrome

A

indolent tumors
median surivial is 8-9 years

transformation to aggressive T cell lymphoma occurs occasionally as terminal event

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41
Q

AML, M6

A

AML with erythroid maturation (acute erythroleukemia)
dysplastic erythroid precursors (some megaloblastoid, others with giant or multiple nucelei)
older age, 20% of therapy related aml

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42
Q

marginal zone lymphomas arise where

A

LNs, spleen or extranodal tissues

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43
Q

solitary myeloma (plasmacytoma)

A

infrequent variant of MM that presents as a single mass in bone or soft tissue

  • extraosseuous lesions in lungs, and nasla sinuses
  • almost inevitably progresses to MM in 10-20 yrs or longer
  • the extraosseous plasmacytomas can be cured by resection
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44
Q

prognosis of hairy cell leukemia

A

excellent, indolent course

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45
Q

AML, M4

A

aml with myelomonocytic maturation (AMML)
myelocytic and monocytic diffevident
mpo positive
monoblast positive for nonspecific esterases
inv(16)

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46
Q

lymphocyte rich HL
EBV?
prognosis
morph

A

ebv in 40% of csaes
frequent mononuclear and diagnositic RS cells
background infiltrate rich in T lymphocytes
RS cells are CD15+, and CD30+
older adults
very good to excellent prognosis

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47
Q

what Ig on surface of hairy cell leukemia

A

IgG

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48
Q

nodular sclerosing type HL
ebv related?
location?
prognosis?

A

most common
not common associated with EBV
lower cervical supraclavicular sites but aslo frequent mediastinal involvement
excellent prognosis

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49
Q

clinical features of AML

A

similar clinical picture as ALL
anemia (fatigue), neutropenia (fever/infection, oportunsitic) thrombocytopenia (bleeding)

-petechiae/ecchymoses/mucosal hemorrhages/hematuria

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50
Q

biggest spleens puthoff has ever seen

A

in CML

51
Q

peripheral T cell lymphoma, unspecified
presentation
prognosis

A

diagnosis requires immunophenotyping : CD3,5,4,8 and t cell receptors

-lymphadenopathy sometimes with eosinophilia, pruritus, fevere, and weight loss
worse prognosis than B cell analogues like DLBCL

52
Q

cause of death MM

A

1) infections 2) renal insufficiency

53
Q

myedlodysplasia cause

blood counts

A

idiotpathic or secondary
cytopenias peripherally
myeloid morph defects like blasts but less than 20% in marrow

54
Q

subsets of multiple myeloma

A

solitary plasmacytoma

smoldering myeloma

55
Q

normal marrow cells largely replaced by plasma cells including forms with multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig

A

MM

56
Q

prognosis and treatment of mantle cell lymphoma

A 
prognosis is poor
median survival is 3-4 years
can transform to DLBCL
lymphoma not curable with normal chemo
death from organ dysfunction
57
Q

clinical features of lymphoplasmactyic lymphoma

A

nonspecific like weakness fatigue, weight loss
1/2 pts have lympadenopathy, HSM
anemia from marrow infiltartion common
10% pts have autoimmune hemolysis from cold agglutinsins, IgM

58
Q

adult T cell leukemia/lymphoma

  • what cells
  • what infection
  • where occurs
  • what can it cause
  • prognosis
A

CD4+ t cells
HTLV-1 virus infection
occurs in japan, africa, caribbean
may cause progressive demyelinating diseasse of CNS and spinal cord
prognosis: most pts have rapid progressive disease that is fatal within months to a year

59
Q

treatment and prognosis ET

A

indolent clincnal course, with median survival 12-15 yrs
chemo
progression to aml uncommon

60
Q

AML therapy related prognosis

A

very poor

61
Q

extranodal marginal zone lymphomas arise how

may turn to what

A

continuum btwn reactive lymphoid hyperplasia and full blown lymphoma
they begin as polyclonal
with acquisition of mutation, a b-cell clone emerges that still depends on antigen stim t cells for signals that drive growth and survival of b cells at distant sites
-transformation to DLBCL may occur

MALTOMA

62
Q

diagnosis of CML

A

leukocytosis may be over 100,000 with immature forms, less than 10% blasts

bone marrow markedly hypercellular
LAP low

63
Q

clinical features of MDS

A

usually older adults (70)
progression to AML 10-40% of time
cytopenia worse in t-MDS with more rapid progression of AML and survival of 4-8 months

64
Q

what can cause secondary PV

A

COPD and vHL

65
Q

immunophenotype of MM

A

CD138
syndecan-1
CD56

66
Q 
mixed cellularity HL
RS cells and ebv?
gender
age
symptoms
prognosis
A 
lots of RS cells infected with EBV
males
biphasic
systemic (night sweats and weight loss)
prognosis is very good
67
Q

AML with t(11q23;v) prognosis

A

poor

68
Q

LCH

1)mutlifocal/multisystemic

A

letterer-siwe disease
freq before age 2
cutanesous lesions resembling seborrheic eruption
fever, infections, chronic otitis media, mastoiditis
HSM, LAD, pulmonary lesions and bone lesions

69
Q

ringed sideroblasts, erythroid progenitors with iron laden mitochond seen as perinulcear granules on prussian blue stain

A

MDS

70
Q

mycosis fungoides/sezary syndrome

what cells
whwere is tumor
seazry syndrome difference

A

cd4+ t cells
home to skin
S syndrome is variant of generalized exfoliateive erythroderma with associated leukemia of sezary cells with cerebriform nuclei

71
Q

anaplastic large cell lymphoma rearrangement of what gene

A

ALK gene on chromosome 2

-alk not normal in lymphocytes or other lymphomas

72
Q

if you see clusters of plsama cells think what

A

plasma cell dyscrasia

73
Q

3) pulmonary langerhans cell histiocytosis

mutation
clinical
assocaition
treat

A

bilateral intersitial disease
multiple fine nodules and cysts in middle and upper lung zones
BRAF mutations
cig smoking associated
may regress spont on cessation of smoking

74
Q

extranodal NK/T cell lymphoma

  • high association
  • invades what
  • where mostly occurs
  • presentation
A

highly associated with EBV
surround and invade small vessels–>extensive ischemic necrosis

asia

highly destructive nasopharyngeal mass, less commonly involves testes and or skin
necrotizing lesion in the midline

75
Q

treatment and prognosis of lymphoplasmacytic lymphoma

A

incurable progressive disease
plasmapheresis can help symptoms
use anti-CD20 antibody
median survival is about 4 years

76
Q

MGUS

A

most common plasma cell dyscrasia

pts asymptomatic and serum M protein level less than 3 gm/dL

77
Q

diagnositc reed sternberg cells are what

A

large cells with multiple nuclei or single nucleaus with multiple nuclear lobes each with large inclusion like nucleolus

78
Q

in a pt with normal CD4 levels and has virally infected LN what kind of hyperplasia do you get

A

brisk follicular hyperplasia bc B cell response to T cell invasion

79
Q

at presentation, extranodal lymphadenopathy and H?sm
lymphomatoid polyposis which may lead some to bleieve pt has IBS
symptoms related to BM, liver, spleen, and GI tract common

A

mantle cell lymphoma

80
Q

AML, MI

A

AML without maturation
-very immature but >3% of blasts are MPO postive
few granules or auer rods
CD33 and 34 but no CD64 (mature myeloid cells) and low CD15

81
Q

clincial features of MM

A 
bone resoption
fractures, pain
hypercalcemia nad neurologic manifestations
renal dysfunction
recurrent bacterial infections
cell immunity unaffected
82
Q

prognosis PM

A

3-5 years

death from complication of cytopenias

83
Q

primary myelofibrosis mutation

A

JAK2 and MPL

84
Q

anaplastic LCL

tumor locations and appearance

A

tumor cells often tend to cluster about venules and infiltrate lymphoid sinuses, mimick appearance of metastatic carcinoma

85
Q

lacunar cells

A

in nodular sclerosis subtype

delicate folded or multilobate nuclei and abundant pale cytopalsm levaing nucleus in empty hole

86
Q

nucleated red cell progentitors with multilobulated or multiple nuclei

A

MDS

87
Q

plasmablastas with vesicular nuclear chromatin and prominent single nucleolus or bizarre multinucleated cells may predominate

A

MM

88
Q

AML, NOS (FAB classifcation M0-M7) prognsois

A

intermediate

89
Q

AML, M2

A 
AML with myelocytic maturation
most common
aeur rods present in most cases
full range of myeloid maturation
t(8;21)
90
Q

what is the translocation for mantle cell lymphoma

A

t(11;14)

IgH and Cyclin D1 promotes G1- S phase progression during the cell cycle

91
Q

AML, M3

A 
APL
multiple needle like auer rods 
younger pts 35-40
hypergranular promyuelocytes
DIC
t(15;17)
92
Q

T cell lymphoma with ALK re arrangement presentation

A

present in young adults or children
present as soft tissue masses often
have very good prognosis

morphologically similar lymphomas without ALK reararangements occur in adults and have poor prognosis

93
Q

morphology langerhans cell histiocytosis

A

by light microscopy
findins are vesicular nuclei with linear grooves or folds and vacuolated cytoplasm
birbeck granules in cytoplasm

94
Q

large granular lymphocytic leukemia

cells
occurs in who
morphology of cells
clinical features
syndrome involved
A

t cells and NK variants
in adults
tumor cells are large lymphocytes with abundant blue cytoplasm and scant coarse azurophilic granules
neutrophenia and anemia common
felty syndrome (rhematoid arthritis splenomegaly, neutropenia)

95
Q

AML, M5

A

AML with monocytic maturation
acute monocytic leukemia
MPO negative, non specific esterase positive
promonocytes predominate in marrow and mature monocytes in periph blood
older pts
organomegaly, LA

96
Q

immunophenotype hariy cell leukemia

A

CD19,20

CD11c, CD25, CD103, annexin A1

97
Q

LPL and hypervisocsity syndrome characterized by

A

visual impairment, deform retinal veins and retinal hemorrhages

neuro problems like headaches dizziness deafness and stupor

bleeding

cryoglobulinemia (raynaud and cold urticaria)

98
Q 
lymphocyte predominance HL
ebv?
markers
cells
transofrm
population
A

EBV not associated
CD15- and 30-
frequent L&H popcorn variants in background of folicular dendritic cells and reactive B cells
3-5% of cases transform into tumor resembling diffuse large b cell lymphoma
young males

99
Q

treatment PV

A

phlebotomy will increase life from months to 10+ years

jak2 inhibtors

100
Q

PV EPO and cells

A

increase in red cells granulocytes and platelets but mainly red cells
EPO suppressed

101
Q

typically the proliferation consists of a homogenous population of small lymphocytes with irregular to occasionally deeply clefted nuclear contours

A

mantle cell lymphoma

102
Q

AML with t(8;21) prognosis

A

favorable

103
Q

AML, M7

A

aml with megakaryocytic maturation
blasts of megakaryocytic lineage predom
blasts react with Abs aginst GPIIb/IIIa or vWF
myelofibrosis or increased marrow reticulin seen in most cases

104
Q

common features in MPD

A

EMH
spent phase leads to cytopenias later (cytokines have fibrogenic effect, less cells more fibrosis, looks like MDS)

variable transormation to acute leukemia, slow progression blast crisis

105
Q

polyclonal to monoclonal transtion during lymphomageneisis is in what and what

A

marginal zone lymphoma nad EBV indcuded lymphoma

106
Q

langerhans cell histiocytosis

A

S-100+
CD1a+
HLA-DR+

107
Q

is roulexux formation specific to MM

A

no it can be seen in other elavted Ig level disorders like SLE, and early hIV

108
Q

lymphoplasmacytic lymphoma

A

b cell neoplasm of older adults that usually presents in 60s-70s
resemebles CLL/SLL but differs in that a substantial fraction of tumor cells undergo terminal differentiation to plasma cells which secretes monoclonoal IgM = hyperviscosity (waldenstrom macroglobulinemia)

  • no renal problems or bone destruction
  • MyD88
109
Q

lymphohistiocytic variants L&H cells

A

polypoid nuclei, inconspicuous nucleoli, mod abundant cytoplasm
lymphocyte predominant type

110
Q

peripheral T cell tumors are most common in what geographic region

N?K cell tumors

A

asia

far east

111
Q

prognosis of multifocal/multisystemic langerhans cell histiocytosis

A

50% 5 year survial rate with intense chemo

untreated = rapidly fatal

112
Q

excessie production and aggregations of M proteins in MM

A

usually of IgA and or IgG3 subtype leads to symptoms related to hyperviscosity in 7% of pts

113
Q

CML mutation

A

BCR-ABL

114
Q

AML with inv(16) prognosis

A

favorable

115
Q

translocations for marginal zone lymphoma

A

11;18
14;18
1;14

upregulate funct of BCL10 or MALT1

116
Q

mutation in PV

A

jak2 pt mutation

117
Q

types of AML

A

I) AML with genetic aberrations

II) AML with MDS like features

III) AML, therapy related

IV) AML, NOS

118
Q

clinical of primary myelofibrosis
age
cells
anemia

A

over 60
obliterative marrow fibroris by non neoplastic fibroblasts
early megakaryopoiesis and granulopoiesis
extensive EMH
megakaryocytes prodcue PDGF and TGF-B
JAK2 mutations in 50-60%
early in progression marrow is hypercell but eventurally leads to cytopenias and EMH (agnogenic myeloid metaplasia)
splenomegaly
normochromic normocytic anemia

119
Q

MDS

A

refractory anemia (subtypes too)
CMML
MDS, NOS

120
Q

ET mutation

A

JAK2 pt mutation and MPL in 5-10%

121
Q

marginal zone lymphoma at extranodal sites deserve special attention bc of unusual pathogenesis and associations

A

they arise within tissues exhibiting chronic inflammation of autoimmune or infectious etiolofgy

salivary gland: sjogren syndrome and hashimoto’s throiditis

stomach in h pylori

remain localized for prolonged periods

may regress if inciting agent is eliminated

122
Q

what must be ruled out with ET

A

reactive increase

inflammation/iron deficiency

123
Q

rouleaux formations and MM

A

red cells seem to virtually stack on one another bc of acute phase reactants increasing the viscosit ofblood

Heme and Lymph (4 decks)

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