Things I can't remember Flashcards
CMML Criteria?
CMML 0, 1, 2 criteria?
What other cell type is common in biopsies?
Atypical CML Criteria (BCR-ABL1 Negative)?
Are the megs normal in this entity?
JMML Criteria?
Associated with what syndrome?
What Hb is increased/ MC Chr abnormality/ Other Gene?
Associated with NF1 and Noonan (RASopathy)
Hb F/ Monosomy 7/ CBL
MDS-RS-T criteria?
If prior MDS that goes to RS-T, call it RS-T?
Findings in RBCs and Megs
Genes?
No; it is an evolution of MDS
RBCs atypical and macrocytic; ET like (large)
SF3B1 (60%), JAK2 V617F (<10%), CALR or MPL W515 Rare
MDS/MPN U Criteria
CML Accelerated Phase Criteria?
Blast phase criteria?
Marker of poor prognosis?
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
CML p210 has what features?
p230?
p190?
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)
CNL Criteria?
Gene associated?
CSF3R and ssociated with SETBP1 and ASXL1 (poor prognosis)
PV criteria?
What do megs look like?
Megs are highly variable in size
Post PV myelofibrosis criteria?
Primary myelofibrosis criteria?
PMF overt fibrotic stage criteria?
What lineage looks really weird?
Megs are highly atypical and have various sizes and “Cloud nuclei”
ET Criteria?
T//F: ET has dense meg clusters?
F: PMF tends to have denser clusters
Post ET MF Criteria?
Chronic Eosinophilic Leukemia Criteria?
Differences from hyperesosinophilic syndrome?
HES: No end organ damage or dysfunction. Both > 1.5k eos
Mastocytosis criteria?
Mutation?
Aberrant markers?
KIT D816V
Mastocytosis is associated with other entities?
Mast cell leukemia has what kind of KIT mutaitons?
Can be associated with CMML; KIT mutation still common
Atypical; may not have D816V but others; sequence gene
FIP1L1-PDGFRA eosinophilia criteria?
Associated with what?
Responds to what drug?
Mastocytosis (CD2-, CD25+) and can have elevated tripase
Imatinib
ETV6-PDGFRA Eosinophilia Criteria/Findings
Transolocation?
Associated with; responds to what drug?
t(5;12)(q32;p13.2)–classic; many others
Mastocytosis (CD2+ and CD25+); imatinib if translocation present
MPN with FGFR1 Criteria/Findings?
Gene on what chr?
Associated with?
8p11; copious partners
T-LL (may be clue to dx); B-LL, Mixed phenotype and CEL–>AML
Myeloid/Lymphoid Neoplasm with PCM1-JAK2 Criteria/Findings?
Provisional: t(8;9)
Eosinphilia, neutrophil precursors in blood, Monocytosis rare, Basophilia can be seen, maybe dysgran and erythropoiesis
Blastic Plasmacytoid Dendritic Cell Neoplasm Criteria?
Associated with/Rule-out?
DDX from mature plasmacytoid dendritic cell proliferation?
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
Does ALL havea lower limit of blasts to diagnose?
B-ALL markers?
PAX5 can be positive in what other entity?
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
Most common translocaiton in mixed phenotype acute leukemia?
Second most common translocation?
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)
Markers of centroblasts and germinal center cells?
As B-cells mature to plasma cells what happens to BCL6 and MUM1/IRF4?
BCL6+, BCL2-, CD10+, LMO2+, and HGAL+ (seen in germinal center malignancies)
BL6 goes down and MUM1 becomes +
T/F: Gamma-Delta T-cells show CD4+, CD8+ phenotype?
T follicular helper cell phenotype?
Markers on Treg cells?
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+
What is the most common rearrange in kids <1 yr with ALL?
Prognosis?
ETV6-RUNX1 t(12;21) phenotype and prognosis?
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
B-ALL Hyperdiploid prognosis?
Hypodiploid prognosis?
What B-ALL transolocation has eosinophilia?
Good
Poor; near haploid the worst (23-29 chrm)
t(5;14); IGH/IL3
T-ALL can be hard to differentiate from what?
NK cell ALL; T-ALL can have CD56+ , and early T-cell markers CD7, Cd2 CD5 and cCD3e can be on early NK cells
ETP-ALL phenotype?
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
NK-LL/LBL hard to ddx from?
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
What percentage of Prolymphs are needed for PLL?
Increased prolymphs in CLL are associated with?
What is LEF1 used for in CLL?
>55% is PLL
Trisomy 12
Identifies CLL/SLL cells in tissue (normal B-cells negative)
CLL Poor Prognsis?
ZAP70+, CD38+, TP53 Mutations (poor Flutarabine response), Lacks IGV hyper mutation, Complex Karyotype
B-PLL needs what percentage to call it and need to lack?
Is there a good phenotype?
Greater than 55%; need to lack SOX11 and t(11;14)
No; highly variable in what B-cell markers are present
Spelenic Marginal Zone has what in circulation?
Are node affected?
Phenotype?
Villous lymphs
No! Marrow is affected
CD20+, CD79a+, Cd5-, CD10-, CD23-, CD43-, Annexin A1-, Cyclin D1-, LEF1-, BCL6-
How does Hairy Cell look in the marrow?
How does it appear in the spleen?
Stains/IHC pattern?
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-
IgM MGUS Criteria/ progresses to?
What percentage have monoclonal B-cells?
Do PC’s have CD56?
Progresses to LPL, B-cells dx, and amylodosis, not myeloma
75%; phenotype is non-specific
No; CD56-
Myeloma and smoldering myeloma criteria?
MGUS to MM rate, what about Light chain MGUS to MM?
1% for heavy chain/year, 0.3% for Light chain MGUS
IgA MGUS highest (1.5%/yr)
Small cell myeloma and lymphoplasmacytic can be confused with?
Non-secretory myeloma needs to be ddxed from?
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)
Solitary plasmacytoma and minimal bone marrow involvement criteria?
POEMS Criteria?
POEMS stands for?
Polyneuropathy, organomegaly, ednocrinopathy, monoclonal gammopathy (G and A; lambda most common), and skin changes (not all need to be present
MALT Phenotype?
What new marker might be useful?
Not a great one: CD20+, CD79a+, CD5-, CD10-, CD23-, CD43-/+, CD11c-/+, rare CD10+, BCL6-
CD21, 23, and 35 show disrupted follicules
IRTA1
Stains to DDX Nodal Marginal Zone from Follicular
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
Morphology of Nodal Pediatric Marginal Zone?
Immunophenotype?
Stain to DDx from pediatric follicular?
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
What feature defines diffuse follicular lymphoma/translocation?
What needs reporting?
Lymphoma cells but not CD21/23 positive cells to indicate a follicule; t(14;18) IGH/BCL2
Diffuse and nodular patterns (25% increments)
Is t(14;18) IGH-BCL2 seen in testicular follicular lymphoma; prognosis?
Immunophenotype?
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+
Follicular lymphomoa can transform to?
DLBCL, or B-ALL (even if “MYC and BCL2; do not called it High-grade/double hit)
One way to separate duodenal MALT from duodenal FL?
FL tends to have t(14;18) IGH-BCL2 and FL like staining. Can overalp on H and E
Pediatric follicular lymphoma criteria?
T/F: IHC–BCL2+?
Morphology?
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
Large B-cell with IRF4/MUM1 occurs where/patterns?
IHC?
Waldeyer’s ring (but could be anywhere); diffuse, follicular, or mixed
Mature B-cell markers, 66% BCL2+ and CD10+, can be BCL6+
Do primary cutaneous follicular lymphoma show BCL2 rearrangement?
IHC?
No!
BCL6+, possible light chain expression, CD10- (may be positive in follicular pattern/ negative in diffuse), BCL2-, CD20+, CD79a+, CD5-, CD43-
Criteria for in situ mantle cell lymphoma vs mantle cells lymhpoma?
CD5 status in in situ?
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 -)
Cutanious diffuse large: How to seperate primary cutaneous follicular from DLBCL leg type
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)
TCHRBCL (T-cell bkg) vs NLPHL (B-cell bkg)?
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
DLBCL Leg Type IHC?
2 Genes with translocations commonly?
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%)
DLBCL associated with chornic inflammation, sites and associated with?
Staining?
DDx from primary effusion lymphoma?
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-
Extranodal NK/T-cell vs Lymphatoid granulomtosis?
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
Primary Mediastinal Large B-cell lymphoma histology?
IHC?
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+
ALK Large B-cell Lymphoma; ALK staing pattern; morphology?
IHC?
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+
Plasmablastic lymphoma population, viral association?
IHC?
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)
Burkitt Lymphoma IHC?
What chrm has an alteration that causes Burkitt like morphology?
B-cell marker +, Germinal Center Phenotype: CD10+, BCL6+
TdT-, BCl2-
MYC tends to be +
11q!; BL Like with alteraiton of 11q
AML t(8;21) RUNX-RUNX1T1 morphology? IHC?
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+
AML Inv(6); t(16;16) CGFB-MYH1 features?
IHC?
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+
Hypogranular APL Flow phenotype?
HLA-DR-/lo, CD34-lo, CD11a/b/CD180/lo, Bright CD33+, Heterogenous CD13+, CD117 can be variable, CD64+, CD56+ (10% poor prognosis)
Variant APL’s and their responses to retinoic acid?
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
AML t(9;11) KMT2A–MLLT3 associated with; marker of poor prognosis?
Phenotype?
Monocytes; MECOM+
CD33+, Cd65+, CD4+, HLA-DR+, CD13lo, CD34lo, Cd14lo, CSPG4+; adults have monocytic markers (CD14, Cd4, Cd64, and lysozyme), CD34/117 variable
AML t(6;9); DEK-NUP214 associated with?
IHC?
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-
Inv(3); t(3;3) is associated with what genes and blood/BM findings?
Phenotype?
GATA2 and MECOM; normal–elevated plts, dysplastic megs and multilineage dysplasia
AML t(1;22) RBM15-MKL1 associated with?
Phenotype?
Down’s; megakaryocytic maturation; seen in children
Morphology mimics acute megakaryooblastic leukemia
AML with NPM1 mutations has what NPM staining pattern?
Associated with?
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
AML with biallelic mutation has what morphologic features?
Phenotype?
No specific ones; assoicated iwth AML iwth or without maturation;
Higher HLA-DR, CD7 and CD16 expression and low/- CD56; CD14-, CD64-
AML-MRC criteria; % needed dysplasitc for moprhologic diagnosis?
If based on morphology what needs to be excluded?
Greater or equal to 50% in 2 lines
AML with NPM1 or bialleic CEBPA
Therapy replated MDS, AML, MN, have what common cytogenetic findings?
Many have loss of chrm 5q, 7q or all of 7; TP53 mutation associated with poor prognosis
How to ddx AMML from acute monoblastic leukemia?
Both have >20% blasts, AMML has between 20-79% monocytic cells and monoblastic leukemia 80+% monocytic cells
Pure erythroid leukemia criteria (still an AML, NOS)?
IHC?
DDx from?
≥ 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
Acute megakaryoblastic leukemia criteria?
IHC?
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+
Acute basophilic leukemia associated with?
DDx from mast cells?
IHC?
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+
Acute panmyelosis with myelofibrosis criteria?
Prognosis?
BM findings?
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
What type of AML is associated with Downs?
TAM shows up at what age, mimic what; risk of AML?
Acute Megakaryoblastic Leukemia
About 3-7 days, lasts days/months; AML; 10-30% 1-3 years later
Myeloid leukemia associated with Downs includes what 2 entities; prognosis?
IHC?
Pathopneumo gene?
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
Hepatosplenic T-cell associated with?
IHC Markers?
Chromosome marker?
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
Agressive NKL vs. Chronic lymphoproliferative disorder NK cell phenotypes?
ANKL is associated with?
Aggressive NKL: CD56bright/CD16dim/CD57- vs CLPD-NK: CD56dim/CD16+/CD57+
EBV+ (90%); EBV- (10%)
