WBC III Flashcards
1
Q
pseudo pelger huet cells, nuetrohils with only 2 nucelear lobes instead of 3-4
A
MDS
2
Q
AML with MDS like features
- with prior MDS which has now transofmred
- without prior MDS prognosis of each
A
very poor
poor
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
4
Q
which types of HL not assocaited with EBV
A
sclerosing
lymphocytre predominance
5
Q
punched out defects of bones
A
MM
6
Q
AML, M0
A
minimially differentiated
MPO negative
blasts lack definitive cytologic markers of myeloblast
express myeloid lineage antigens
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
8
Q
what CD marker do ALK tumors usually have
A
CD30
9
Q
what are the MPD
A
CML
PV
ET
Progressive myelofibrosis (PM)
10
Q
tisse infiltration may be prominent with what AML
A
monocytic
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
12
Q
clnical ET
A
mainly adults over 60
onset insidious
increased prolif megakaryocytes with high periph platelet counts
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
14
Q
who gets mantle cell lymphoma most often
A
5th and 6th decades of life and shows male predominance
15
Q
variants of mantle cell lymphoma
A
blastoid variant and a proliferative expression profiling signature are associated with even shorter survivials
16
Q
diagnosis ET
A
bone marrow biopsy
megkarycytes high in number clustering together
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)
18
Q
granulocytic sarcoma
A
an AML rarely presenting as localized primary tumor mass (orbital granulcytic sarcoma)
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
20
Q
HL or NHL has mesenteric nodes and walderyer ring involvement
A
NHL
21
Q
tumor cells in mycosis fung/sezary syndrome express what
A
CLA
CCR4
CCR10
all bring cd4+ cells to skin
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
23
Q
clinical features of mantle cell lymphoma
A
painless lymphadenopathy
spleen and gut symptoms comon
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
25
if monocytic differentiation of AML then positive for
non specific esterase
26
3 broad categories of myeloid neoplasias
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
27
immunophenotype of mantle cell lymphoma
high level cyclin D1
most tumors have CD19 and 20
usually CD5+ and CD23- to distinguish from CLL/SLL
28
CML clinical
```
primarily adult males at 50
onset insidious
non specific symptoms
splenomegaly bc of EMH
LUQ pain with splenic infarct
```
29
what is blast crisis
MPD then start seeing superimposition of blast that accumulates over 20% in BM, then pt with AML that has arise now
30
AML with t(15;17) prognosis
intermediate
| APL, DIC
31
LPL bone marrow biopsy
mixture of small lyphoid cells with varying degree of plasma cell diff
32
MPD cells
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
33
first sign of PV
25% with DVT, MI, stroke
34
mutations in hairy cell leukemia
BRAF
35
smoldering myeloma
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
36
RS ells release cytokines and chemokines that induce accumulation of
reactive lymphocytes, macrophages, and granulocytes
37
megakaryocytes with multiple nuclei instead of normal single multilobulated nucleus
MDS
38
what are the plasma cell neoplasms/dyscrasias and related disorders
multiple myeloma
waldenstrom macroglobulinemia
heavy chain disaease
primary or immunocyte assocaited amyloidosis
monoclonal gammopathy of undeterined significance
39
average age of diagnosis for HL, curable?
32, curable in most cases
40
prognosis of mycosis fungoides/sezary syndrome
indolent tumors
median surivial is 8-9 years
transformation to aggressive T cell lymphoma occurs occasionally as terminal event
41
AML, M6
AML with erythroid maturation (acute erythroleukemia)
dysplastic erythroid precursors (some megaloblastoid, others with giant or multiple nucelei)
older age, 20% of therapy related aml
42
marginal zone lymphomas arise where
LNs, spleen or extranodal tissues
43
solitary myeloma (plasmacytoma)
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
44
prognosis of hairy cell leukemia
excellent, indolent course
45
AML, M4
aml with myelomonocytic maturation (AMML)
myelocytic and monocytic diffevident
mpo positive
monoblast positive for nonspecific esterases
inv(16)
46
lymphocyte rich HL
EBV?
prognosis
morph
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
47
what Ig on surface of hairy cell leukemia
IgG
48
nodular sclerosing type HL
ebv related?
location?
prognosis?
most common
not common associated with EBV
lower cervical supraclavicular sites but aslo frequent mediastinal involvement
excellent prognosis
49
clinical features of AML
similar clinical picture as ALL
anemia (fatigue), neutropenia (fever/infection, oportunsitic) thrombocytopenia (bleeding)
-petechiae/ecchymoses/mucosal hemorrhages/hematuria
50
biggest spleens puthoff has ever seen
in CML
51
peripheral T cell lymphoma, unspecified
presentation
prognosis
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
cause of death MM
1) infections 2) renal insufficiency
53
myedlodysplasia cause
| blood counts
idiotpathic or secondary
cytopenias peripherally
myeloid morph defects like blasts but less than 20% in marrow
54
subsets of multiple myeloma
solitary plasmacytoma
| smoldering myeloma
55
normal marrow cells largely replaced by plasma cells including forms with multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig
MM
56
prognosis and treatment of mantle cell lymphoma
```
prognosis is poor
median survival is 3-4 years
can transform to DLBCL
lymphoma not curable with normal chemo
death from organ dysfunction
```
57
clinical features of lymphoplasmactyic lymphoma
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
adult T cell leukemia/lymphoma
- what cells
- what infection
- where occurs
- what can it cause
- prognosis
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
treatment and prognosis ET
indolent clincnal course, with median survival 12-15 yrs
chemo
progression to aml uncommon
60
AML therapy related prognosis
very poor
61
extranodal marginal zone lymphomas arise how
| may turn to what
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
diagnosis of CML
leukocytosis may be over 100,000 with immature forms, less than 10% blasts
bone marrow markedly hypercellular
LAP low
63
clinical features of MDS
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
what can cause secondary PV
COPD and vHL
65
immunophenotype of MM
CD138
syndecan-1
CD56
66
```
mixed cellularity HL
RS cells and ebv?
gender
age
symptoms
prognosis
```
```
lots of RS cells infected with EBV
males
biphasic
systemic (night sweats and weight loss)
prognosis is very good
```
67
AML with t(11q23;v) prognosis
poor
68
LCH
| 1)mutlifocal/multisystemic
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
ringed sideroblasts, erythroid progenitors with iron laden mitochond seen as perinulcear granules on prussian blue stain
MDS
70
mycosis fungoides/sezary syndrome
what cells
whwere is tumor
seazry syndrome difference
cd4+ t cells
home to skin
S syndrome is variant of generalized exfoliateive erythroderma with associated leukemia of sezary cells with cerebriform nuclei
71
anaplastic large cell lymphoma rearrangement of what gene
ALK gene on chromosome 2
| -alk not normal in lymphocytes or other lymphomas
72
if you see clusters of plsama cells think what
plasma cell dyscrasia
73
3) pulmonary langerhans cell histiocytosis
mutation
clinical
assocaition
treat
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
extranodal NK/T cell lymphoma
- high association
- invades what
- where mostly occurs
- presentation
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
treatment and prognosis of lymphoplasmacytic lymphoma
incurable progressive disease
plasmapheresis can help symptoms
use anti-CD20 antibody
median survival is about 4 years
76
MGUS
most common plasma cell dyscrasia
| pts asymptomatic and serum M protein level less than 3 gm/dL
77
diagnositc reed sternberg cells are what
large cells with multiple nuclei or single nucleaus with multiple nuclear lobes each with large inclusion like nucleolus
78
in a pt with normal CD4 levels and has virally infected LN what kind of hyperplasia do you get
brisk follicular hyperplasia bc B cell response to T cell invasion
79
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
mantle cell lymphoma
80
AML, MI
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
clincial features of MM
```
bone resoption
fractures, pain
hypercalcemia nad neurologic manifestations
renal dysfunction
recurrent bacterial infections
cell immunity unaffected
```
82
prognosis PM
3-5 years
| death from complication of cytopenias
83
primary myelofibrosis mutation
JAK2 and MPL
84
anaplastic LCL
| tumor locations and appearance
tumor cells often tend to cluster about venules and infiltrate lymphoid sinuses, mimick appearance of metastatic carcinoma
85
lacunar cells
in nodular sclerosis subtype
| delicate folded or multilobate nuclei and abundant pale cytopalsm levaing nucleus in empty hole
86
nucleated red cell progentitors with multilobulated or multiple nuclei
MDS
87
plasmablastas with vesicular nuclear chromatin and prominent single nucleolus or bizarre multinucleated cells may predominate
MM
88
AML, NOS (FAB classifcation M0-M7) prognsois
intermediate
89
AML, M2
```
AML with myelocytic maturation
most common
aeur rods present in most cases
full range of myeloid maturation
t(8;21)
```
90
what is the translocation for mantle cell lymphoma
t(11;14)
| IgH and Cyclin D1 promotes G1- S phase progression during the cell cycle
91
AML, M3
```
APL
multiple needle like auer rods
younger pts 35-40
hypergranular promyuelocytes
DIC
t(15;17)
```
92
T cell lymphoma with ALK re arrangement presentation
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
morphology langerhans cell histiocytosis
by light microscopy
findins are vesicular nuclei with linear grooves or folds and vacuolated cytoplasm
birbeck granules in cytoplasm
94
large granular lymphocytic leukemia
```
cells
occurs in who
morphology of cells
clinical features
syndrome involved
```
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
AML, M5
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
immunophenotype hariy cell leukemia
CD19,20
| CD11c, CD25, CD103, annexin A1
97
LPL and hypervisocsity syndrome characterized by
visual impairment, deform retinal veins and retinal hemorrhages
neuro problems like headaches dizziness deafness and stupor
bleeding
cryoglobulinemia (raynaud and cold urticaria)
98
```
lymphocyte predominance HL
ebv?
markers
cells
transofrm
population
```
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
treatment PV
phlebotomy will increase life from months to 10+ years
| jak2 inhibtors
100
PV EPO and cells
increase in red cells granulocytes and platelets but mainly red cells
EPO suppressed
101
typically the proliferation consists of a homogenous population of small lymphocytes with irregular to occasionally deeply clefted nuclear contours
mantle cell lymphoma
102
AML with t(8;21) prognosis
favorable
103
AML, M7
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
common features in MPD
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
polyclonal to monoclonal transtion during lymphomageneisis is in what and what
marginal zone lymphoma nad EBV indcuded lymphoma
106
langerhans cell histiocytosis
S-100+
CD1a+
HLA-DR+
107
is roulexux formation specific to MM
no it can be seen in other elavted Ig level disorders like SLE, and early hIV
108
lymphoplasmacytic lymphoma
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
lymphohistiocytic variants L&H cells
polypoid nuclei, inconspicuous nucleoli, mod abundant cytoplasm
lymphocyte predominant type
110
peripheral T cell tumors are most common in what geographic region
N?K cell tumors
asia
far east
111
prognosis of multifocal/multisystemic langerhans cell histiocytosis
50% 5 year survial rate with intense chemo
| untreated = rapidly fatal
112
excessie production and aggregations of M proteins in MM
usually of IgA and or IgG3 subtype leads to symptoms related to hyperviscosity in 7% of pts
113
CML mutation
BCR-ABL
114
AML with inv(16) prognosis
favorable
115
translocations for marginal zone lymphoma
11;18
14;18
1;14
upregulate funct of BCL10 or MALT1
116
mutation in PV
jak2 pt mutation
117
types of AML
I) AML with genetic aberrations
II) AML with MDS like features
III) AML, therapy related
IV) AML, NOS
118
clinical of primary myelofibrosis
age
cells
anemia
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
MDS
refractory anemia (subtypes too)
CMML
MDS, NOS
120
ET mutation
JAK2 pt mutation and MPL in 5-10%
121
marginal zone lymphoma at extranodal sites deserve special attention bc of unusual pathogenesis and associations
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
what must be ruled out with ET
reactive increase
| inflammation/iron deficiency
123
rouleaux formations and MM
red cells seem to virtually stack on one another bc of acute phase reactants increasing the viscosit ofblood
