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

WBC, LN disorders Flashcards

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
Q

5q minus syndrome

A

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

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26
Q

CMML

A

Need >1M monocytes to diagnose.

ASXL1 mutations

PDC clusters (CD123+)

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27
Q

JMML

A

RAS-opathy. Associated with Noonan, NF1

Elevated HbF. -7.

Sensitive to GM-CSF, but poor prognosis

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28
Q

Atypical CML

A

BCR-ABL1-negative overlap syndrome. SETBP1 mutations. Has nothing to do with CML.

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29
Q

Castleman disease

A

Hyaline-vascular: Onion-skinning, lollipops, increased FDC meshwork
Plasma-cell: HHV-8 associated. Increased IL-6. Associated with POEMS. May progress to DLBCL

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30
Q

POEMS

A 
Polyneuropathy
Organomegaly
Endocrinopathy
Monogammaglobulinemia
Skin lesions

Associated with plasma-cell multicentric castleman

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31
Q

HIV lymphadenopathy

A

Evolution of follicular hyperplasia, to involution, to depletion

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32
Q

Toxoplasmosis

A

Triad of follicular hyperplasia, monocytoid B cells, and epithelioid granulomas

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33
Q

Infectious mononucleosis

A

Sinusoidal monocytoid B cells.

Paracortical immunoblasts, RS_like cells.

Necrosis and apoptosis OK

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34
Q

Dermatopathic lymphadenopathy

A

Pigment incontinence with increased interdigitating dendritic cells (IDCs): S100+, Fascin+, CD1a-

35
Q

Kikuchi-Fujimoto lymphadenitis

A

Asian patients

Proliferative phase (Crescentic histiocytes, PDCs)&raquo_space; Necrotic phase (no granulocytes)

Self-limiting

36
Q

Cat scratch lymphadenitis

A

Palisading / stellate necrotizing granuloma

37
Q

HLH - Primary, secondary, criteria

A

Primary: PRF1&raquo_space; STX11, UNC13D (AutRec)
Secondary: 5 of 8 - Fever, splenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, decreased NK cell activity, HLH visualized, elevated sCD25

38
Q

CLL

A

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
Q

Follicular lymphoma

A

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
Q

Follicular lymphoma - Subtypes

A

Cutaneous: Neither BCL2+ nor fusion+.
Pediatric: BCL2+, but no fusion.
Duodenal: BCL2+, fusion+.

41
Q

Mantle cell lymphoma

A

CD20+. BCL1/CycD+. SOX11+. CD5+/CD23-.
Histiocytes present. Recall LyPolyp.
FISH is preferable to PCR for CyclinD1 rearr.
Aggressive variants: Pleomorphic, blastoid.

42
Q

Marginal zone lymphomas

A

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
Q

LPL/WM

A

Open sinuses, increased mast cells in BM. IgM paraprotein.

MYD898 L265P (nonspecific)

44
Q

Burkitt

A

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
Q

Hans classifier

A

CD10+&raquo_space; GCB

BCL6-&raquo_space; ABC

MUM+&raquo_space; ABC

None of above true (ie, only BCL6+?)&raquo_space; GCB

46
Q

THRLBCL

A

LBCL that resembles NLP (normal B cell phenotype, maybe CD30). NO ROSETTES.

47
Q

DLBCL, Leg-type

A

Cutaneous DLBCL with poor prognosis.

CD10-, BCL2+, MUM1+ (both poor markers in LBCLs)

48
Q

PMBL

A

Young women, mediastinum

Compartmentalizing fibrosis

B-cell phenotype, often CD30+.

49
Q

IRF4 rearranged DLBCL

A

As name suggests.

CD10+, BCL6+, MUM1+ (all 3!)

High proliferation rate

50
Q

NLPHL

A

Popcorn cells: CD45+, CD20+, often EMA+.

Rosettes of PD1+ helper T-cells

Expanded FDC meshworks

Associated with PTGC

51
Q

CHL types

A

Nodular sclerosing - Mediastinal, often women
Mixed cellularity - Eosinophils, most EBV+
Lymphocyte rich - Good px, few germinal centers
Lymphocyte depleted - Retroperitoneal, poor px

52
Q

AITL

A

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
Q

ALCL

A

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
Q

ATLL

A

T-regs: CD3+, CD4+, CD25+, FOXP3+, CCR4+. CD8-.

Flower cells. Associated with HTLV (+TSP)

Hypercalcemia, lytic bone lesions

55
Q

T-LGLL

A

Need at least 2B/mL?

Associated with RA. Presents with cytopenias.

CD4-, CD8+, toxic markers+.

STAT3 mutations.

56
Q

EATL

A

Celiac disease, europeans

Large cells, usually CD8-.

57
Q

MEITL

A

Asians. Non-mass forming?

Smaller cells. CD8+, CD56+?

58
Q

Hepatosplenic T-cell lymphoma

A

Young men. Common in post-transplant setting.

Usually gamma-delta cells: cCD3, 4-/8-, CD56+.

-7q.

59
Q

Subcuteanous panniculitis-like TCL

A

Lymphoma of gamma-delta T-cells. No involvement of overlying epidermis or dermis.

Note fat rimming by neoplastic T-cells.

60
Q

Mycosis fungoides

A

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
Q

HCL

A

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
Q

vHCL

A

Resembles hairy cell leukemia, but…

CD25-, no BRAF mutations.

63
Q

ETP-ALL

A

Must express CD7, be CD1a- and CD8-.

Cytoplasmic CD3. TdT.

64
Q

ALK+ LBCL

A

Often CD20, PAX5-negative

ALK+, CD138+, often IgA.

65
Q

T-PLL

A

Nuclear irregularity, cytoplasmic blebbing

inv(14) TCL1 abnormalities

66
Q

NK, gamma-delta phenotypes

A

NK: CD4+, CD64+. No CD3.

Gamma-delta: CD3+, 4-/8-.

67
Q

AMLs with megakaryoblastic features

A

t21
t(1;21) RMB-MLK1 - Kids
inv(3) GATA-MECOM - Adults

68
Q

Sezary syndrome

A

Erythrodermic TCL. Loss of epidermotropism.

CD3+, CD4+, CD5/7 often lost, CD8-. Same as MF.

Leukemic, but no BM involvement. Poor survival.

69
Q

DLBCL gene rearrangements

A

BCL6 - Most common (30%). Good Px alone.
BCL2 - 25%. Bad.
MUM-1 - Bad.

Generally, GCB preferable to ABC.

70
Q

WHIM / Myelokathexis

A

CXCR4 mutations, delaying neutrophil maturation & release

Neutropenia, aberrant segmentation, accelerated apoptosis

71
Q

Severe congenital neutropenias

A

HAX1: SCN3 (Kostmann syndrome). Affects mitochondria/apoptosis
ELAN: SCN1. AutDom, Elastase misfolds, mistrafficks.

72
Q

HRS phenotype

A

CD20- (but PAX5 weakly positive)
CD15+, CD30+
CD45-

LMP1+?

Arise from follicle center B-cells

73
Q

Mast cell disorders

A

(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
Q

Plasma cell myeloma - Genetics

A

t(11;14) Cyclin D1 favorable, confers lymphoid morphology
Hyperdiploidy: Odd chromosomes, favorable.

-13 bad, -17 bad. Complex bad. Other IGH translocations bad.

75
Q

Plasma cell paraprotein trivia

A

Flame cells suggest IgA
Plasma cell leukemia often IgD, IgE, or light chain only
Lambda free light chain is more amyloidogenic than kappa

76
Q

Rosai Dorfman

A

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
Q

PMF diagnostic criteria

A

Major: Atypical megakaryocytes, clonality, no other better diagnosis
Minor: Leukoerythroblastosis, anemia, LDH++, splenomegaly

Need all 3 major and 2 minor.

78
Q

Duncan disease

A

X-linked lymphoproliferative syndrome

Associated with SLAM, XIAP gene abnormalities.

Hypogammaglobulinemia. Exaggerated response to EBV infection.

79
Q

TAM

A

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
Q

Core binding factor AMLs

A

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
Q

BPDCN

A

CD4+, CD123+ (normal PDC markers)

CD56+, TdT+ (aberrant)

82
Q

MLNeo

A

PDGFRA/B
FGFR1
NPM-JAK

Responsive to TKIs

83
Q

Most common plasmacytoma sites

A

2 (extraosseous): Upper respiratory tract

Bone #1

Tim's CP Board Review (16 decks)

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