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Things I can't remember Flashcards

1
Q

CMML Criteria?

A

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2
Q

CMML 0, 1, 2 criteria?

What other cell type is common in biopsies?

A
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3
Q

Atypical CML Criteria (BCR-ABL1 Negative)?

Are the megs normal in this entity?

A
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4
Q

JMML Criteria?

Associated with what syndrome?

What Hb is increased/ MC Chr abnormality/ Other Gene?

A

Associated with NF1 and Noonan (RASopathy)

Hb F/ Monosomy 7/ CBL

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5
Q

MDS-RS-T criteria?

If prior MDS that goes to RS-T, call it RS-T?

Findings in RBCs and Megs
Genes?

A

No; it is an evolution of MDS

RBCs atypical and macrocytic; ET like (large)

SF3B1 (60%), JAK2 V617F (<10%), CALR or MPL W515 Rare

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6
Q

MDS/MPN U Criteria

A
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7
Q

CML Accelerated Phase Criteria?

Blast phase criteria?

Marker of poor prognosis?

A

Equal to or > 20% blasts in blood or marrow; presence of extramedullay blasts
Blasts can be myeloid (70%), or lymphoid (20-30%)

CD7+ is poor prognosis on blasts

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8
Q

CML p210 has what features?

p230?

p190?

A

p210 M-BCR: Exon 12-16; classic CML

p230: p-region exons 17-20: Nuetrophilic maturation and thromboctosis
p190: mu- Exon 1-2: Seen in ALL; increased monos (mimics CMML)

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9
Q

CNL Criteria?

Gene associated?

A

CSF3R and ssociated with SETBP1 and ASXL1 (poor prognosis)

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10
Q

PV criteria?

What do megs look like?

A

Megs are highly variable in size

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11
Q

Post PV myelofibrosis criteria?

A
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12
Q

Primary myelofibrosis criteria?

A
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13
Q

PMF overt fibrotic stage criteria?

What lineage looks really weird?

A

Megs are highly atypical and have various sizes and “Cloud nuclei”

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14
Q

ET Criteria?

T//F: ET has dense meg clusters?

A

F: PMF tends to have denser clusters

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15
Q

Post ET MF Criteria?

A
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16
Q

Chronic Eosinophilic Leukemia Criteria?

Differences from hyperesosinophilic syndrome?

A

HES: No end organ damage or dysfunction. Both > 1.5k eos

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17
Q

Mastocytosis criteria?

Mutation?

Aberrant markers?

A

KIT D816V

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18
Q

Mastocytosis is associated with other entities?

Mast cell leukemia has what kind of KIT mutaitons?

A

Can be associated with CMML; KIT mutation still common

Atypical; may not have D816V but others; sequence gene

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19
Q

FIP1L1-PDGFRA eosinophilia criteria?

Associated with what?

Responds to what drug?

A

Mastocytosis (CD2-, CD25+) and can have elevated tripase

Imatinib

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20
Q

ETV6-PDGFRA Eosinophilia Criteria/Findings

Transolocation?

Associated with; responds to what drug?

A

t(5;12)(q32;p13.2)–classic; many others

Mastocytosis (CD2+ and CD25+); imatinib if translocation present

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21
Q

MPN with FGFR1 Criteria/Findings?

Gene on what chr?

Associated with?

A

8p11; copious partners

T-LL (may be clue to dx); B-LL, Mixed phenotype and CEL–>AML

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22
Q

Myeloid/Lymphoid Neoplasm with PCM1-JAK2 Criteria/Findings?

A

Provisional: t(8;9)
Eosinphilia, neutrophil precursors in blood, Monocytosis rare, Basophilia can be seen, maybe dysgran and erythropoiesis

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23
Q

Blastic Plasmacytoid Dendritic Cell Neoplasm Criteria?

Associated with/Rule-out?

DDX from mature plasmacytoid dendritic cell proliferation?

A

Monomorphic blastic cells: (CD56+, CD123+, CD4+, TCF4+ and TCL1+, CD303+, TdT+, MPO-); 8% are CD4- and or CD56-

MDS, other leukemia, CMML and AML; leukemia cutis

Mature is CD56- and more mature

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24
Q

Does ALL havea lower limit of blasts to diagnose?

B-ALL markers?

PAX5 can be positive in what other entity?

A

No lower limit

CD10+, surface and cyto CD22+, CD24+, cCD71a+, PAX5 mostly+, TdT mostly +, CD20 and CD34 variable, CD45 can be dim to absent, and myeloid markers are variable (CD13, CD33)

AML t(8;21) RUNX1-RUNX1T1

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25
Q

Most common translocaiton in mixed phenotype acute leukemia?

Second most common translocation?

A

t(9;22) BCR-ABL1; if CML previously then don’t dx it as MPAL

p190 more common than p210 (consider CML in mixed blast crisis)

KMT2A t(v;11q23.3)

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26
Q

Markers of centroblasts and germinal center cells?

As B-cells mature to plasma cells what happens to BCL6 and MUM1/IRF4?

A

BCL6+, BCL2-, CD10+, LMO2+, and HGAL+ (seen in germinal center malignancies)

BL6 goes down and MUM1 becomes +

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27
Q

T/F: Gamma-Delta T-cells show CD4+, CD8+ phenotype?

T follicular helper cell phenotype?

Markers on Treg cells?

A

F: They usually are CD4-. CD8- and CD5-, rare ones are CD8+

TFH: BCL6+, CD10+, CD4+, CD57+, CD279/PD1+, CXCL13+, CXCR5+

CD25+, FOXP3 and CD4+

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28
Q

What is the most common rearrange in kids <1 yr with ALL?

Prognosis?

ETV6-RUNX1 t(12;21) phenotype and prognosis?

A

t(v;11q23) MLL/KMT2A

Poor in almost every case even young kids

Good prognosis: CD19+, CD10+, CD34+, CD9-, CD20-, CD66c-, and some myeloid expression

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29
Q

B-ALL Hyperdiploid prognosis?

Hypodiploid prognosis?

What B-ALL transolocation has eosinophilia?

A

Good

Poor; near haploid the worst (23-29 chrm)

t(5;14); IGH/IL3

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30
Q

T-ALL can be hard to differentiate from what?

A

NK cell ALL; T-ALL can have CD56+ , and early T-cell markers CD7, Cd2 CD5 and cCD3e can be on early NK cells

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31
Q

ETP-ALL phenotype?

A

CD7+, CD8-, CD1a-
Positive for 1 or more myeloid/stem: CD34, KIT (CD117), HLA-DR, CD13, CD33, CD11b and CD65

cCD3+ (surface might be positive)
CD2 or 4 may be rarely +; CD5- in most cases

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32
Q

NK-LL/LBL hard to ddx from?

A

T-ALL
Both can have CD7, CD2 and even 5
cCD3epsilon on NK cells
NK cells tend to be CD16+
Lacks B-cells and myeloid markers and TCR and IG rearrangements

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33
Q

What percentage of Prolymphs are needed for PLL?

Increased prolymphs in CLL are associated with?

What is LEF1 used for in CLL?

A

>55% is PLL

Trisomy 12

Identifies CLL/SLL cells in tissue (normal B-cells negative)

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34
Q

CLL Poor Prognsis?

A

ZAP70+, CD38+, TP53 Mutations (poor Flutarabine response), Lacks IGV hyper mutation, Complex Karyotype

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35
Q

B-PLL needs what percentage to call it and need to lack?

Is there a good phenotype?

A

Greater than 55%; need to lack SOX11 and t(11;14)

No; highly variable in what B-cell markers are present

36
Q

Spelenic Marginal Zone has what in circulation?
Are node affected?
Phenotype?

A

Villous lymphs

No! Marrow is affected

CD20+, CD79a+, Cd5-, CD10-, CD23-, CD43-, Annexin A1-, Cyclin D1-, LEF1-, BCL6-

37
Q

How does Hairy Cell look in the marrow?

How does it appear in the spleen?

Stains/IHC pattern?

A

Fried egg’s, interstital (vs nodules in splenic marginal zone)
Appears in blood lakes
Tartrate resistance acid phosphatase+, CD20+, CD22+, CD11c, CD103+, CD25+, CD123+, Annexin A1+, FMC7+, CD200+, can be c cyclin D1+, TBX1+, CD5-, CD10-

38
Q

IgM MGUS Criteria/ progresses to?

What percentage have monoclonal B-cells?

Do PC’s have CD56?

A

Progresses to LPL, B-cells dx, and amylodosis, not myeloma

75%; phenotype is non-specific

No; CD56-

39
Q

Myeloma and smoldering myeloma criteria?

MGUS to MM rate, what about Light chain MGUS to MM?

A

1% for heavy chain/year, 0.3% for Light chain MGUS
IgA MGUS highest (1.5%/yr)

40
Q

Small cell myeloma and lymphoplasmacytic can be confused with?

Non-secretory myeloma needs to be ddxed from?

A

B-cell lymphomas; both entities tend to be CD20+ and/or have the t(11;14) IgH;CCND1/VD7 translocation= Cyclin D1+

IgD and IgE myeloma (not tested for on most IFE)

41
Q

Solitary plasmacytoma and minimal bone marrow involvement criteria?

A
42
Q

POEMS Criteria?

POEMS stands for?

A

Polyneuropathy, organomegaly, ednocrinopathy, monoclonal gammopathy (G and A; lambda most common), and skin changes (not all need to be present

43
Q

MALT Phenotype?

What new marker might be useful?

A

Not a great one: CD20+, CD79a+, CD5-, CD10-, CD23-, CD43-/+, CD11c-/+, rare CD10+, BCL6-
CD21, 23, and 35 show disrupted follicules

IRTA1

44
Q

Stains to DDX Nodal Marginal Zone from Follicular

A

Follicular tends to have positve germinal center markers: CD10+. HGAL+, BCL6+, and LM02+

NMZL: IRTA1+ and MNDA+ in 75% of cases; also disrupted follicular networks

45
Q

Morphology of Nodal Pediatric Marginal Zone?

Immunophenotype?

Stain to DDx from pediatric follicular?

A

M>F; large officles with expantension of mantle zone B into GCs (mimics PTGC)
Same as adult; CD10 tends to be negatlive. BCL2+ (50%); light chain restricted

46
Q

What feature defines diffuse follicular lymphoma/translocation?

What needs reporting?

A

Lymphoma cells but not CD21/23 positive cells to indicate a follicule; t(14;18) IGH/BCL2

Diffuse and nodular patterns (25% increments)

47
Q

Is t(14;18) IGH-BCL2 seen in testicular follicular lymphoma; prognosis?

Immunophenotype?

A

No; good prognosis

Usually surface Ig+, Pan B-cell+, Usually BCL2, 6, and CD10+, Cd5-, CD43-; high grade (3B) can lose CD10 and keep BCL6+

48
Q

Follicular lymphomoa can transform to?

A

DLBCL, or B-ALL (even if “MYC and BCL2; do not called it High-grade/double hit)

49
Q

One way to separate duodenal MALT from duodenal FL?

A

FL tends to have t(14;18) IGH-BCL2 and FL like staining. Can overalp on H and E

50
Q

Pediatric follicular lymphoma criteria?

T/F: IHC–BCL2+?

Morphology?

A

False: BCL2 negative (rare weak) and lack t(14;18) translocation

Tend to be BCL6+. CD10+, Pan B+

Head and neck nodes, high grade cytology; good prognosis

51
Q

Large B-cell with IRF4/MUM1 occurs where/patterns?

IHC?

A

Waldeyer’s ring (but could be anywhere); diffuse, follicular, or mixed

Mature B-cell markers, 66% BCL2+ and CD10+, can be BCL6+

52
Q

Do primary cutaneous follicular lymphoma show BCL2 rearrangement?

IHC?

A

No!

BCL6+, possible light chain expression, CD10- (may be positive in follicular pattern/ negative in diffuse), BCL2-, CD20+, CD79a+, CD5-, CD43-

53
Q

Criteria for in situ mantle cell lymphoma vs mantle cells lymhpoma?

CD5 status in in situ?

A

Cyclin D1 positive cells in mantle zone in background of follicular hyperplasia, favors in situ; if mantle expanded and replaced by Cyclin D1+ cells then overt mantle cell with mantle zone growth apttern

In situ: tends to be CD5-, and SOX11+ (some -)

54
Q

Cutanious diffuse large: How to seperate primary cutaneous follicular from DLBCL leg type

A

Leg type: Tend to have MYD88, no CD21/23 mesh work, MUM1/IRF4+, BCL2+, BCl6+, CD10-, lacks follicular pattern

Follicular type: Follicular pattern, some mesh work, MUM1/IRF4-, CD10+, BCL6+, BCL2- (most cases)

55
Q

TCHRBCL (T-cell bkg) vs NLPHL (B-cell bkg)?

A

TCHRBCL: CD15-, CD30-, Pan B-cell + (19, 20, 79a), BCL6+, 2- background CD68/163+, and CD3/5+, lacks FDC network, EBV-, Cd45+, more elderly

NLPHL: CD20+, CD22+, Cd79a+, EMA+ (50%), MUM1+ (50%), CD15-, CD30-, EBV-, CD45+, BCL6+, more in kids, CD57+ rosettes, FDC network present

56
Q

DLBCL Leg Type IHC?

2 Genes with translocations commonly?

A

Sheets of cells of centroblast and immunoblasts:
Cd20+, CD79a, BCL2+ (90%), IFR4/MUM1+ (90%), FOXP1+, MYC+, cIgM+, IgD+ (50%); 10% BCL6+ (dim), CD10-

MYC and BCL6; also MYD88 L265P (60%)

57
Q

DLBCL associated with chornic inflammation, sites and associated with?
Staining?

DDx from primary effusion lymphoma?

A

EBV, pleura is common
CD20+, CD79a+; some can be plasmacytid losing 20 and 79a and gaining MUM and CD138; some grain T-cell markers

Primary effusion is HHV8+ and EBV-

58
Q

Extranodal NK/T-cell vs Lymphatoid granulomtosis?

A

Both are EBV and angiodestructive: NK/T is CD2+, CD56+ and CD3+

LYG: has a more mixed background, B-cells, associated with immunodeficency, Grade 3 has clonal evolution-dx DLBCL

59
Q

Primary Mediastinal Large B-cell lymphoma histology?

IHC?

A

Variable and can mimic RS cells/CHL; scleorsis common; clear cells can be seen

IHC: CD20+, CD45+, MUM1+/-, CD30+ (weak), More suggestive: CD23+, MAL+, c-Rel(+), TRAF+, Surface Ig-, BCL2+, BCL6+, CD54+, FAS+

60
Q

ALK Large B-cell Lymphoma; ALK staing pattern; morphology?
IHC?

A

Granular cytoplasmic (CLTC-ALK) vs. nuclear in NPM1-ALK (T-cell); larget cells with prominent nucleoli

More plasmacytic: MUM1+, CD138+, CD20/79a -ve (rare positive), CD45-, weak to negative CD30, cIg+, maybe CD4+, CD56+

61
Q

Plasmablastic lymphoma population, viral association?

IHC?

A

HIV patients, head and neck, EBV+

CD138+, CD38+, CD30+, EMA+, Ki-67 >90%, BCL2/6-, CD10- (rare +)
CD20, CD79a, CD45 mostly - (can be weakly positive)

62
Q

Burkitt Lymphoma IHC?

What chrm has an alteration that causes Burkitt like morphology?

A

B-cell marker +, Germinal Center Phenotype: CD10+, BCL6+
TdT-, BCl2-

MYC tends to be +

11q!; BL Like with alteraiton of 11q

63
Q
A
64
Q 
AML t(8;21) RUNX-RUNX1T1 morphology?
IHC?
A

Large cells with azourophilic granules/possible auer rods and Psuedo-CH granules and Hofs; neutrophil dysplasia

High CD34, HLA-DR, MPO, CD13; possible low CD33; CD19/PAX5 rare +, cCD79a rare+

65
Q

AML Inv(6); t(16;16) CGFB-MYH1 features?

IHC?

A

Abnormal Eos with dense granules; Eos increased; neuts decreased

CD34+, CD117+, Myeloid markers positive (CD13, CD33); Mono’s can be positive (CD14, CD64, etc); some are CD2+

66
Q

Hypogranular APL Flow phenotype?

A

HLA-DR-/lo, CD34-lo, CD11a/b/CD180/lo, Bright CD33+, Heterogenous CD13+, CD117 can be variable, CD64+, CD56+ (10% poor prognosis)

67
Q

Variant APL’s and their responses to retinoic acid?

A

t(11;17): ZBTB16-RARA: Responds; regular nuclei iwth grans, lacks auer rods and pelgeroid neuts; resistant

t(5;17); NPM1-RARA: hyper cells>hypogranular; responds

STAT5-RARA: resistant

68
Q

AML t(9;11) KMT2A–MLLT3 associated with; marker of poor prognosis?

Phenotype?

A

Monocytes; MECOM+

CD33+, Cd65+, CD4+, HLA-DR+, CD13lo, CD34lo, Cd14lo, CSPG4+; adults have monocytic markers (CD14, Cd4, Cd64, and lysozyme), CD34/117 variable

69
Q

AML t(6;9); DEK-NUP214 associated with?

IHC?

A

Lower WBC counts (12k/uL); multilineage dysplasia, ring sideroblasts (in some cases), pancyto; poor prognosis

MPO+, CD13+, CD33+, CD123+, CD34+, CD117+, CD15+ (some cases), TdT+ 50%, CD64 occasionally
Basos seen as: CD123, CD33, CD38 triple+; HLA-DR-

70
Q

Inv(3); t(3;3) is associated with what genes and blood/BM findings?

Phenotype?

A

GATA2 and MECOM; normal–elevated plts, dysplastic megs and multilineage dysplasia

71
Q

AML t(1;22) RBM15-MKL1 associated with?

Phenotype?

A

Down’s; megakaryocytic maturation; seen in children
Morphology mimics acute megakaryooblastic leukemia

72
Q

AML with NPM1 mutations has what NPM staining pattern?

Associated with?

A

Cytoplasmic NPM1

AML with myelomonocytic features; cells tend to be CD34-, Higher WBC and plt counts vs most AMLs; some Acute monocytic leukemias have NPM1

73
Q

AML with biallelic mutation has what morphologic features?

Phenotype?

A

No specific ones; assoicated iwth AML iwth or without maturation;

Higher HLA-DR, CD7 and CD16 expression and low/- CD56; CD14-, CD64-

74
Q

AML-MRC criteria; % needed dysplasitc for moprhologic diagnosis?

If based on morphology what needs to be excluded?

A

Greater or equal to 50% in 2 lines

AML with NPM1 or bialleic CEBPA

75
Q

Therapy replated MDS, AML, MN, have what common cytogenetic findings?

A

Many have loss of chrm 5q, 7q or all of 7; TP53 mutation associated with poor prognosis

76
Q

How to ddx AMML from acute monoblastic leukemia?

A

Both have >20% blasts, AMML has between 20-79% monocytic cells and monoblastic leukemia 80+% monocytic cells

77
Q

Pure erythroid leukemia criteria (still an AML, NOS)?

IHC?

DDx from?

A

≥ 30% proerythroblasts; no significant myeloblast population, ≥ 80% erythroid lineage in marrow

CD71+ (many times), but almost always E-Cad+, CD34-, CD117+ (mostly), CD36+, CD41-, CD64-
MDS-EB2

78
Q

Acute megakaryoblastic leukemia criteria?

IHC?

A

Greater or = to 20% blasts of which 50+% are megakaryoblasts

CD61+, CD41+, Myeloid: CD13/33 can be +, CD45-, HLA-DR-, CD34-, CD36+, MPO-, TdT-, CD7 can be+

79
Q

Acute basophilic leukemia associated with?

DDx from mast cells?

IHC?

A

Hyperhistenemia; blasts have corse basophilic granules

CAE- vs mast cells

Myeloid: CD13/33+, CD203c+, CD123+, CD11b+, CD34 maybe +, HLA-DR+, CD117-, Maybe CD7+, CD9+, Maybe TdT+ and CD22+

80
Q

Acute panmyelosis with myelofibrosis criteria?

Prognosis?

BM findings?

A

20% blasts BM/PB–not seen in MDS or primary fibrosis; with marrow fibrosis; pancytopenia;

Bad prognosis

Hypercellular, hard to aspirate, trilineage increased with sheets of blasts and atypical megs

81
Q

What type of AML is associated with Downs?

TAM shows up at what age, mimic what; risk of AML?

A

Acute Megakaryoblastic Leukemia

About 3-7 days, lasts days/months; AML; 10-30% 1-3 years later

82
Q
A
83
Q

Myeloid leukemia associated with Downs includes what 2 entities; prognosis?

IHC?

Pathopneumo gene?

A

MDS and AML (50% megalokaryoblastic leukemia); good

Most blasts MPO-, CD117+, CD13+, CD33+ CD7+, CD4+, CD41+, CD61+, CD71+, CD34-( 50% of cases; unlike TAM)

Mutations in GATA1; trisomy 8 common

84
Q

Hepatosplenic T-cell associated with?

IHC Markers?

Chromosome marker?

A

Chron’s and immunosuppression

CD3+, CD4 and 8 can be + or -, CD56+ (commonly), Gamma/Delta(+), TIA-1 (+), tends to lack Granzyme B (+ in LGL leukemia)

Isochrome 7q

85
Q

Agressive NKL vs. Chronic lymphoproliferative disorder NK cell phenotypes?

ANKL is associated with?

A

Aggressive NKL: CD56bright/CD16dim/CD57- vs CLPD-NK: CD56dim/CD16+/CD57+

EBV+ (90%); EBV- (10%)

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