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Tim's CP Board Review > WBC, LN disorders > Flashcards

WBC, LN disorders Flashcards

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1
Q

Chediak Higashi

A

LYST

Large granules in neutrophils

Associated with albinism, bleeding, and HLH-like EBV infection

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2
Q

May-Hegglin anomaly

A

MYH9 abnormality

Dohle-like granulocyte inclusions

Macrothrombocytopenia

Associated with deafness, cataracts, nephritis

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3
Q

Alder-Reilly phenomenon

A

PAS+ granules in ALL WBCs.

Associated with mucopolysaccharidoses.

Normal granulocyte function.

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4
Q

Pelger-Huet anomaly

A

Lamin B receptor abnormality.

Hypolobated neutrophils.

No clinical significance.

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5
Q

Niemann-Pick

A

Sphingomyelinase deficiency.

Foamy vacuolated macrophages. “Sea-blue”? Mucin negative.

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6
Q

Gaucher cells

A

Glucocerebrosidase deficiency

Tissue paper wrinkled macrophages. Mucin-negative.

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7
Q

Hematogones

A

Blast-like B-cell precursors.

CD34+, TdT+. CD10+, which fades as CD20 is acquired.

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8
Q

Blast morphologies and markers

A

Monoblasts: Grey-blue cytoplasm, irregular and prominent nuclear folds. CD4/CD16
Megakaryoblasts: Blebbing. CD41/42/61
Erythroblasts: Dark, with vacuoles. CD71/E-Cad/GlycophorinA

Myeloblasts generally: MPO, CD13, CD15, CD33

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9
Q

“Block PAS positivity”

A

Buzzword for ALL

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10
Q

Cytochemistry, generally

A

Alpha-naphthyl esterase (non-specific) - Monocytes
Chloroacetate esterase (CAE) - Granulocytes
*Both of above in Combined esterase

MPO, Sudan Black - Myeloid

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11
Q

Acute monocytic leukemia
Pure erythroid leukemia
Megkaryoblastic leukemia

A

Need >80% of each respective blast morphology and phenotype. Rule out other specific diagnoses (eg, for Megakaryoblastic, must rule out inv(3), t(1;22))

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12
Q

APL

A

t(15;17) PML-RARa ; variant translocations often confer resistance to ATRA therapy

Hypergranular or hypogranular (butterfly lobed nuclei, higher count).

CD33+, CD117+, CD34 dim, HLA-DR -

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13
Q

t(9;11) AML

A

MLL rearrangement

Usually seen in children. Intermediate prognosis.

Monocytic morphology

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14
Q

t(6;9) AML

A

DEK-NUP.

Basophilia. Poor prognosis.

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15
Q

inv(3) or t(3;3) AML

A

MECOM

Megakaryoblasts

Hypolobation, dysplasia

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16
Q

t(1;22) AML

A

RMB15-MLK1

Megakaryoblastic.

Associated with t21.

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17
Q

Chemotherapy types and t-MN

A

Alkylating agents: MDS-like AML with -5, -7, etc. Dismal prognosis. Slow onset.
Topoisomerase inhibitors: MLL-rearrangement, monocytic. Still poor prognosis. Fast onset.

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18
Q

AML with normal karyotypes

A

FLT3 ITD - Poor px (especially D835 - Confers resistance to FLT3 inhibitors)
Biallelic CEBPA - Good px
NPM1 - Good px
IDH - ???

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19
Q

B-ALL - Molecular profiles

A

t(12;21) ETV6-RUNX1 - Good
Hyperdiploidy (>52chr) - Good
(Age 1-10 good)

Hypodiploidy (<40chr) - Bad
t(9;22) or BCR-ABL1-like - Bad
MLL-rearranged - Bad. Infants.
RUNX1 amplified - Bad
Complex karyotype - Bad
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20
Q

Features of MPNs

A 
Extramedullary hematopoiesis
Organomegaly
NO DYSPLASIA
Basophilia
Progression to fibrosis and leukemia
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21
Q

MPN genetics

A

JAK2 (V617F > Exon 12 mut)
CALR
MPL
BCR-ABL

CSF3R in CNL

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22
Q

CML

A

Basophilia, marked left shift. Diagnosable on smear and molecular (but get BMBx for blast count)

Low LAP score. Pseudo-Gaucher cells. Dwarf megakaryocytes

Phases: Chronic, accelerated (10-19% blasts), blast crisis (+Ph, i17q, +8, +19)

p210 transcripts

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23
Q

MDS genetics

A

Good: -Y, del(5q), del(20q)

Poor: -7, -17p, complex karyotypes

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24
Q

MDS class flowchart

A
  1. Excess blasts
  2. Mildilineage dysplasia
  3. Ringed sideroblasts
  4. Isolated del(5q)
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25
5q minus syndrome
Usually seen in older women. MDS with isolated del(5q) Presents with macrocytosis and THROMBOCYTOSIS BMBx shows small megakaryocytes Associated with RPS14 abnormalities. Treatable with lenalidomide
26
CMML
Need >1M monocytes to diagnose. ASXL1 mutations PDC clusters (CD123+)
27
JMML
RAS-opathy. Associated with Noonan, NF1 Elevated HbF. -7. Sensitive to GM-CSF, but poor prognosis
28
Atypical CML
BCR-ABL1-negative overlap syndrome. SETBP1 mutations. Has nothing to do with CML.
29
Castleman disease
Hyaline-vascular: Onion-skinning, lollipops, increased FDC meshwork Plasma-cell: HHV-8 associated. Increased IL-6. Associated with POEMS. May progress to DLBCL
30
POEMS
``` Polyneuropathy Organomegaly Endocrinopathy Monogammaglobulinemia Skin lesions ``` Associated with plasma-cell multicentric castleman
31
HIV lymphadenopathy
Evolution of follicular hyperplasia, to involution, to depletion
32
Toxoplasmosis
Triad of follicular hyperplasia, monocytoid B cells, and epithelioid granulomas
33
Infectious mononucleosis
Sinusoidal monocytoid B cells. Paracortical immunoblasts, RS_like cells. Necrosis and apoptosis OK
34
Dermatopathic lymphadenopathy
Pigment incontinence with increased interdigitating dendritic cells (IDCs): S100+, Fascin+, CD1a-
35
Kikuchi-Fujimoto lymphadenitis
Asian patients Proliferative phase (Crescentic histiocytes, PDCs) >> Necrotic phase (no granulocytes) Self-limiting
36
Cat scratch lymphadenitis
Palisading / stellate necrotizing granuloma
37
HLH - Primary, secondary, criteria
Primary: PRF1 >> STX11, UNC13D (AutRec) Secondary: 5 of 8 - Fever, splenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, decreased NK cell activity, HLH visualized, elevated sCD25
38
CLL
CD20+. CD5+/CD23+. CD200+, LEF1+. Need 5k lymphs, else call MBL. Proliferation foci. Paraimmunoblasts. Cytogenetics: del(13q) good. t12, -11q, -17p bad. CD38, ZAP70, CD49d, IGHV unmut bad.
39
Follicular lymphoma
CD20+. CD10+, BCL2+. Tends also to express BCL6, HGAL, LMO2 Back-to-back follicles. Buttock cells. Grading: <15 centroblasts per hpf (Gr 1-2), any residual centrocytes 3a, purely centroblasts 3b.
40
Follicular lymphoma - Subtypes
Cutaneous: Neither BCL2+ nor fusion+. Pediatric: BCL2+, but no fusion. Duodenal: BCL2+, fusion+.
41
Mantle cell lymphoma
CD20+. BCL1/CycD+. SOX11+. CD5+/CD23-. Histiocytes present. Recall LyPolyp. FISH is preferable to PCR for CyclinD1 rearr. Aggressive variants: Pleomorphic, blastoid.
42
Marginal zone lymphomas
General: Dutcher/russell bodies. NMZL: Associated with IRTA abnormalities EMZL: Often t(11;18) API2-MALT1 SMZL: Bipolar villous projections. Associated with autoimmune disorders, H. Pylori, Chlamydia
43
LPL/WM
Open sinuses, increased mast cells in BM. IgM paraprotein. MYD898 L265P (nonspecific)
44
Burkitt
3 types: Endemic (EBV+, jaw), sporadic (abdomen), immunosupp (EBV++). Starry sky histology. Vacuolated cells on smear. EXTRANODAL! CD10+, MYC+. Also BCL6+. Ki67 100% t(8;14) > t(2;8) (kappa) or t(8;22) (lambda)
45
Hans classifier
CD10+ >> GCB BCL6- >> ABC MUM+ >> ABC None of above true (ie, only BCL6+?) >> GCB
46
THRLBCL
LBCL that resembles NLP (normal B cell phenotype, maybe CD30). NO ROSETTES.
47
DLBCL, Leg-type
Cutaneous DLBCL with poor prognosis. CD10-, BCL2+, MUM1+ (both poor markers in LBCLs)
48
PMBL
Young women, mediastinum Compartmentalizing fibrosis B-cell phenotype, often CD30+.
49
IRF4 rearranged DLBCL
As name suggests. CD10+, BCL6+, MUM1+ (all 3!) High proliferation rate
50
NLPHL
Popcorn cells: CD45+, CD20+, often EMA+. Rosettes of PD1+ helper T-cells Expanded FDC meshworks Associated with PTGC
51
CHL types
Nodular sclerosing - Mediastinal, often women Mixed cellularity - Eosinophils, most EBV+ Lymphocyte rich - Good px, few germinal centers Lymphocyte depleted - Retroperitoneal, poor px
52
AITL
Fh T-cells: CD3+, CD4+, CD8- ; CD10+, PD1+, ICOS+, BCL6+, CXCL+ Arborizing vessels in LN with PAS+ material. Polyclonal hypergammaglobulinemia (rheumatoid factors, ASMA, cryoglobulins) Expanded FDC meshworks May have incidental EBV+ B cells
53
ALCL
Sinusoidal involvement by CD30+ Hallmark/Wreath cells. Granule markers more reliable than CD30? EMA+, ALK variable. t(2;5) has nuclear and cytoplasmic expression. Perivascular rosettes ALK- often DUSP22, IRF4, TP63 rearranged (poor px)
54
ATLL
T-regs: CD3+, CD4+, CD25+, FOXP3+, CCR4+. CD8-. Flower cells. Associated with HTLV (+TSP) Hypercalcemia, lytic bone lesions
55
T-LGLL
Need at least 2B/mL? Associated with RA. Presents with cytopenias. CD4-, CD8+, toxic markers+. STAT3 mutations.
56
EATL
Celiac disease, europeans Large cells, usually CD8-.
57
MEITL
Asians. Non-mass forming? Smaller cells. CD8+, CD56+?
58
Hepatosplenic T-cell lymphoma
Young men. Common in post-transplant setting. Usually gamma-delta cells: cCD3, 4-/8-, CD56+. -7q.
59
Subcuteanous panniculitis-like TCL
Lymphoma of gamma-delta T-cells. No involvement of overlying epidermis or dermis. Note fat rimming by neoplastic T-cells.
60
Mycosis fungoides
Patch / Plaque / Tumor stages. Indolent course, but poor overall prognosis? Epidermotropism, Pautrier microabscesses, cerebriform nuclei. CD4+, CD8-. Often loss of CD5, CD7. TCR rearrangements.
61
HCL
Leukemic disorder of B-cells: CD20+, CD11c+, CD13+, CD103+, TRAP+, Annexin A1+. CYCLIN D1? BRAF V600E, sometimes MAP2K1. Monocytopenia, marrow fibrosis with interstitial infiltration. Blood lakes in spleen. Fried eggs. No LN involvement. Circumferential projections.
62
vHCL
Resembles hairy cell leukemia, but... CD25-, no BRAF mutations.
63
ETP-ALL
Must express CD7, be CD1a- and CD8-. Cytoplasmic CD3. TdT.
64
ALK+ LBCL
Often CD20, PAX5-negative ALK+, CD138+, often IgA.
65
T-PLL
Nuclear irregularity, cytoplasmic blebbing inv(14) TCL1 abnormalities
66
NK, gamma-delta phenotypes
NK: CD4+, CD64+. No CD3. Gamma-delta: CD3+, 4-/8-.
67
AMLs with megakaryoblastic features
t21 t(1;21) RMB-MLK1 - Kids inv(3) GATA-MECOM - Adults
68
Sezary syndrome
Erythrodermic TCL. Loss of epidermotropism. CD3+, CD4+, CD5/7 often lost, CD8-. Same as MF. Leukemic, but no BM involvement. Poor survival.
69
DLBCL gene rearrangements
BCL6 - Most common (30%). Good Px alone. BCL2 - 25%. Bad. MUM-1 - Bad. Generally, GCB preferable to ABC.
70
WHIM / Myelokathexis
CXCR4 mutations, delaying neutrophil maturation & release Neutropenia, aberrant segmentation, accelerated apoptosis
71
Severe congenital neutropenias
HAX1: SCN3 (Kostmann syndrome). Affects mitochondria/apoptosis ELAN: SCN1. AutDom, Elastase misfolds, mistrafficks.
72
HRS phenotype
CD20- (but PAX5 weakly positive) CD15+, CD30+ CD45- LMP1+? Arise from follicle center B-cells
73
Mast cell disorders
(Rule out other hematolymphoid malignancy first) Must show clustering, abnormal spindling CD2, 25 are abnormal. CD117 is expected. KIT mutational analysis (817). Increased mast cell tryptase.
74
Plasma cell myeloma - Genetics
t(11;14) Cyclin D1 favorable, confers lymphoid morphology Hyperdiploidy: Odd chromosomes, favorable. -13 bad, -17 bad. Complex bad. Other IGH translocations bad.
75
Plasma cell paraprotein trivia
Flame cells suggest IgA Plasma cell leukemia often IgD, IgE, or light chain only Lambda free light chain is more amyloidogenic than kappa
76
Rosai Dorfman
Self-limiting histiocytosis of especially cervical and occipital lymph nodes. Emperipolesis. S100+. CD1a-. Often has increased plasma cells and polyclonal hypergammaglobulinemia. Rule out hemophagocytosis.
77
PMF diagnostic criteria
Major: Atypical megakaryocytes, clonality, no other better diagnosis Minor: Leukoerythroblastosis, anemia, LDH++, splenomegaly Need all 3 major and 2 minor.
78
Duncan disease
X-linked lymphoproliferative syndrome Associated with SLAM, XIAP gene abnormalities. Hypogammaglobulinemia. Exaggerated response to EBV infection.
79
TAM
Develops in 25% of t21 infants, with acquired GATA-1 mutation Increased circulating blasts (10%?) up to a few months out from birth. Resolves spontaneously, but predicts later AML.
80
Core binding factor AMLs
t(8;21) RUNX1-RUNX1T1 - Salmon-colored granules. CD19. inv(16) CBFB-MYH11 - Abnormal eosinophils Both are definable by translocation (do not need 20% blasts) Both are worsened with co-occurrence of KIT mutation
81
BPDCN
CD4+, CD123+ (normal PDC markers) CD56+, TdT+ (aberrant)
82
MLNeo
PDGFRA/B FGFR1 NPM-JAK Responsive to TKIs
83
Most common plasmacytoma sites
Bone #1 #2 (extraosseous): Upper respiratory tract

Tim's CP Board Review (16 decks)

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