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Flashcards in CP Deck (81):
1

Niemann-Pick Disease

sphingomyelinase

Foamy

2

Gaucher Disease

glucocerebrosidase

Wrinkled paper

3

Factor deficiency with no symptoms
with dramatic PT prolongation

XII

4

Which MLL in adults vs Kids

9;11 kids (9 months baby)
4;11 in adults

5

DEK NUP2

MLD and basophilia

6

Factor ? deficiency in Ashkanizi kews?

11

7

Renal Medullary Carcinoma in who

Sickle cell trait

8

CBC in sickle cell trait

completely normal

9

G6PD pathway?

pentose phosphate (hexose monophophate)

10

Pyruvate Kinase pathway?

Glycolytic

11

Sucrose lysis test

PNH screen

12

Acidified Serum (Ham's)

old PNH test

13

False Neg G6PD test

recent attack (too many retics) or female heterozygote

14

Fe def anemia vs Beta thal trait

inc RDW in Fe def anemia (both microcytic/hypochromic)

15

Reticulocyte index formula

= reticulocytes × patient HCT/normal HCT

16

2 RBC types with inc RNA

basophilic stippling and retics

17

Alkaline gel

origin --> CSF-A

CEOA2, SDGLe

18

hgb absorbance

340 nm

19

met hgb absorbance

340+ 200 (cyan+ met) = 540 nm

20

met hgb charge

M=3+
M flip

21

Monitor TTP response

Plt counts, NOT ADAMTS13 (remains low)

22

Abciximab target
pattern in aggre

targets GPIIb/IIIa
looks just like glanzmann's (only ristocetin response)

23

Ticlodipine

target ADP receptor

24

Reverse warfarin effect

Prothrombin gene concentrate + vitamin K

25

A Mono L Flow Phenotype
Consistent?
Negative?

vs

CMML Flow Phenotype
Decreased?
Aberrant?

Consistent = CD33 (BRIGHT) and CD64
Other = CD11b, CD13, HLA DR
Negative = CD34
vs

in CMML
Decreased = CD36, CD14, CD13, CD64
Aberrant = CD56

26

Acute Promyelocytic Leukemia Flow Phenotype
Positive:
Absent:
Aberrant:

Positive: Bright CD33, variable CD13, CD117
Absent: CD34 and HLA-DR
Aberrant: CD2 and CD56

27

AEL markers?

CD71 and Glycophorin A

28

Marker TCRHRLBCL vs NLPHL

NLPHL = EMA+
CD4 and CD57+ T cell rossettes (FHTcell)

29

Cold agglutinins
What Ab kind?
Situations?

Auto-anti-I
1) Cold agglutinin disease
2) Mycoplasma pneumoniae infection

Auto-anti-i
1) Associated with infectious mononucleosis
2) Less often a problem than auto-anti-I

iadult phenotype
1) Uncommon persistence of i antigen in adults

30

Beta vs Alpha Thal MCV

Beta = bad = 55-65
alpha = 65-75

31

Alpha thal trait HPLC

all normal (normal F and A2)

32

Sickle Cell Trait HPLC

Hgb A (50-60%) and Hb S (35-45%)*********

If with alpha-globin gene deletion(s), Hb S < 35%

If β⁺ thalassemia, % Hb S > % Hb A

If with β⁰ thalassemia, almost all hemoglobin is Hb S, same phenotype as sickle cell disease (~ Hb SS)

33

Diamond-Blackfan Anemia

Normal** bone marrow cellularity

Macrocytic anemia with erythroblastopenia*****

Well-preserved granulocytic and megakaryocytic maturation

34

Fanconi Anemia tests

Chromosomal breakage (typically tests peripheral blood lymphocytes); Cells are stimulated and exposed to diepoxybutane &/or mitomycin C

35

Bernard soulier vs VWD

BSS = giant plts

36

Hairy Cell Leukemia phenotype

Annexin-A1, CD123, TRAP, CD103, BRAF, CD200

bright: CD103, CD25, CD11c, and CD22

37

KMT2A (a.k.a. MLL) rearrangement is most common in __________, who often present with _____________

infants
very high white blood cell count and higher rate of CNS involvement

38

Testing for red cell G6PD activity - when?

best performed when patient not hemolyzing; false negative results because ofadequate G6PD activity in reticulocytes

39

Serum and plasma tubes

Serum
Red: No anticoagulant --> clots --> all fibrinogen used up

Plasma
Lavender: Treated with EDTA.
Blue: Treated with citrate.
Green: Treated with heparin.

40

Serum

Plasma minus fibrinogen

41

What RBC parameter unaffected in CAIHA

Hb, WBC, plts all unaffected

clumps counted as one, so RBC goes down, so MCH and HCT abnormal

42

Not included in DIC panel

PTT

43

HLH criteria

only molecular needed if present
otherwise
5 of 8 clinical and laboratory criteria required for diagnosis

Fever
Splenomegaly
Cytopenias
Hyper triglyceridemia &/or hypo fibrinogenemia
Serum ferritin > 500 µg/L
Hemophagocytosis
Low or absent NK-cell activity
Soluble CD25 (sIL-2 receptor) > 2,400 U/mL

44

SLE cells

Neuro Lupus

S LE --> LE cells

NPSLE due to cytokines (IL6)

45

EBV pos lung lymphoma

lymphomatoid granulomatosis
more EBV pos cells --> higher grade

46

RDD histiocyte

S100 and CD68

47

Null lymphomas

PEL and ALCL

48

serous atrophy of BM aka

gelatinous transformation

49

ATLL immunophenotype

mature T cells
CD2(+), CD3(+), CD5(+), TCR-α/β(+)
CD7(-) or dim
~ 90% of cases are CD4(+)

4 and 25 coexpression

50

AITL immunophenotype

CD2(+), CD3(+), CD5(+), βF1/TCR-αβ(+)

± aberrant loss or reduced expression of CD7

Usually CD4(+)
T cells have follicular helper T-cell immunophenotype in most cases

CD10(+), Bcl-6(+), CXCL13(+), CXCR5(+), PD-1(+)

51

Hepatosplenic T-cell lymphoma (HSTCL)

Most cases TCR-γδ(+)
TCR-γδ(+) most reliably determined by flow cytometry
CD2(+), CD3(+), CD7(+), CD16(-/+), CD56(+/-)
KIR(+), CD94 (dim + or -)

CD4(-), CD8(-),CD5(-), AND CD57(-)

Unusual variations include CD5 expression

CD57+ in TLGL-L

52

Acanthocytes

equally spaced
A--> Abeta lipoproteinemia

53

Arterial Thrombosis

A --> ACA (anticardiolipin antiobody) syndrome
A and V

LAC --> only venous

54

thrombin time

bypasses everything before thrombin (only prolonged in heparin or thrombin inhibitor)

55

Warfarin OD

Factor V unaffected, only in liver failure

56

Childhood Myelodysplastic Syndrome (Refractory Cytopenia of Childhood)

Persistent cytopenia(s) during childhood

Refractory neutropenia or thrombocytopenia most common; isolated refractory anemia rare

Blasts: < 2% in peripheral blood; < 5% in bone marrow

Dysplasia in 2 lineages (erythroid, granulocytic, megakaryocytic) or ≥ 10% in single lineage

57

iron storage in BM and reflected in the serum

ferritin
elevated in ACD (since there is trapping in the BM)
dec in IDA

58

Type II vWD: _____________ defects of vWF

Qualitative defects of vWF

59

Type 2A vWD

2A --> Absent HMWMs and IMWMs

Most common type II variant
Mutation affects platelet to platelet adhesion
Impaired vWF multimer assembly; results in decreased HMWMs and IMWMs

60

Type 2B vWD

Increased binding 2BGpIb

Desmopressin contraindicated in type 2B vWD

Spontaneous binding of vWF to platelets leads to depletion of HMWMs and formation of platelet aggregates

Platelet aggregates are removed from circulation, leading to thrombocytopenia

61

Type 2M vWD

M: multimers still present

Decreased vWF-dependent platelet adhesion

No deficiency of HMWMs and IMWMs

62

Type 2N vWD

N: nearly hemophiliac

Markedly decreased binding affinity for FVIII
Mutation leads to impaired interaction between vWF and FVIII --> enhanced clearance and low circulating levels of FVIII

63

Platelet-type or pseudo-vWD

Defect on Platelet

similar phenotype as type 2B vWD

Decreased platelet count

Decreased vWF activity out of proportion to decrease in vWF:Ag
Decreased vWF antigen
vWF:RCo/vWF:Ag ratio < 0.5-0.7

Absence of HMWM on vWF multimer analysis

Increased response to low-dose ristocetin-induced platelet aggregation (RIPA)

64

Mega Marker

CD61

65

Erythro Marker

CD71
PAS (cytoplasmic)

66

B-PLL

Monoclonal B cell, lacks distinctive immunophenotypic features

67

Primary Cutaneous Follicle Center Lymphoma

Bcl-6(strong +)
CD10 and Bcl2-2 can be negative and often negative

PCFCL that are CD10(+) and Bcl-2(+) likely carry t(14;18)(q32;q21)

68

One Symptom of AITL

Follicular helper T cells upregulate CXCR5 and CXCL13
CXCL13 promotes B-cell recruitment through adherence of B cells on high endothelial venules (HEV)
CD21(+) follicular dendritic cells expand around HEV
Leads to B-cell expansion, plasmacytic differentiation, and hypergammaglobulinemia

69

AITL morphology

Marked proliferation of arborizing HEV with Increased proliferation of follicular dendritic cells (FDC), usually around HEV

Partial or complete effacement of architecture; perinodal infiltration common

Cells with clear to pale cytoplasm & distinct cell membranes

Often form small clusters around follicles and HEV

Small reactive lymphocytes, plasma cells, eosinophils, and histiocytes
± immunoblasts of B-cell lineage; can be prominent
± Reed-Sternberg + Hodgkin (RS+H)-like cells of B-cell lineage; usually EBV(+)

70

AITL Pts at increased risk for?

developing another lymphoma
Diffuse large B-cell lymphoma (DLBCL) is most common
Usually EBV(+)
EBV(+) DLBCL can precede diagnosis of AITL

71

AITL Phenotype

aberrant loss or reduced expression of CD7

Usually CD4(+) and CD8(-)

CD10(+), Bcl-6(+), CXCL13(+), CXCR5(+), ICOS(+), &/or PD-1(+)

Normal CD4:CD8 ratio is common
Due to reactive T cells that outnumber neoplastic T cells

72

Pattern of HCL, SMZL, and GDHSTCL in BM

interstitial and sinusoidal
esp sinusoidal --> SMZL, and GDHSTCL

73

APL

HLA DR neg
CD34 Neg

CD33 Pos

74

refrac cytopenia of childhood vs aplastic anemia

lack of erythroids + adipocytosis of marrow = aplastic anemia

75

CLL immunophenotype

Weak monotypic surface Ig, weak CD20 --> absence of FMC7
Pos: CD23, CD5

76

Argatroban

direct thrombin (IIa) inhibitor

77

Fondaparinux

indirect Xa inhibitor

78

Rivaroxaban, apixaban and edoxaban

direct Xa inhibitor

79

PCV of castlemann with increased?

IL6

80

Types of LyP

Type A (mixed infiltrate)
-Few scattered large cells (RS or multinuc), polymorphous inflammatory cells

Type B (mycosis fungoides-like)
-Cannot be separated from MF by histology or immunohistochemistry
-Type B LyP spontaneously regresses, unlike MF

Type C (ALCL-like)
-Cannot be separated from ALCL by histology or immunohistochemistry

Type D (cytotoxic T-cell variant)
-Marked epidermotropism and CD8(+)
-Mimics aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma
-MUM1 focal to diffusely (+)

Type E (angioinvasive variant)
-Angioinvasive infiltrates of medium-sized atypical lymphocytes

81

one main diff between cut ALCL and LyP Type C?

clinical, if it regresses --> LyP