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Word association > CP > Flashcards

CP Flashcards

1
Q

Niemann-Pick Disease

A

sphingomyelinase

Foamy

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2
Q

Gaucher Disease

A

glucocerebrosidase

Wrinkled paper

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3
Q

Factor deficiency with no symptoms

with dramatic PT prolongation

A

XII

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4
Q

Which MLL in adults vs Kids

A

9;11 kids (9 months baby)

4;11 in adults

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5
Q

DEK NUP2

A

MLD and basophilia

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6
Q

Factor ? deficiency in Ashkanizi kews?

A

11

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7
Q

Renal Medullary Carcinoma in who

A

Sickle cell trait

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8
Q

CBC in sickle cell trait

A

completely normal

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9
Q

G6PD pathway?

A

pentose phosphate (hexose monophophate)

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10
Q

Pyruvate Kinase pathway?

A

Glycolytic

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11
Q

Sucrose lysis test

A

PNH screen

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12
Q

Acidified Serum (Ham’s)

A

old PNH test

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13
Q

False Neg G6PD test

A

recent attack (too many retics) or female heterozygote

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14
Q

Fe def anemia vs Beta thal trait

A

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

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15
Q

Reticulocyte index formula

A

= reticulocytes × patient HCT/normal HCT

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16
Q

2 RBC types with inc RNA

A

basophilic stippling and retics

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17
Q

Alkaline gel

A

origin –> CSF-A

CEOA2, SDGLe

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18
Q

hgb absorbance

A

340 nm

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19
Q

met hgb absorbance

A

340+ 200 (cyan+ met) = 540 nm

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20
Q

met hgb charge

A

M=3+

M flip

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21
Q

Monitor TTP response

A

Plt counts, NOT ADAMTS13 (remains low)

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22
Q

Abciximab target

pattern in aggre

A

targets GPIIb/IIIa

looks just like glanzmann’s (only ristocetin response)

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23
Q

Ticlodipine

A

target ADP receptor

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24
Q

Reverse warfarin effect

A

Prothrombin gene concentrate + vitamin K

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25
Q

A Mono L Flow Phenotype
Consistent?
Negative?

vs

CMML Flow Phenotype
Decreased?
Aberrant?

A

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

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

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26
Q

Acute Promyelocytic Leukemia Flow Phenotype
Positive:
Absent:
Aberrant:

A

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

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27
Q

AEL markers?

A

CD71 and Glycophorin A

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28
Q

Marker TCRHRLBCL vs NLPHL

A

NLPHL = EMA+

CD4 and CD57+ T cell rossettes (FHTcell)

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29
Q

Cold agglutinins
What Ab kind?
Situations?

A

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

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30
Q

Beta vs Alpha Thal MCV

A 
Beta = bad = 55-65
alpha = 65-75
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31
Q

Alpha thal trait HPLC

A

all normal (normal F and A2)

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32
Q

Sickle Cell Trait HPLC

A

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)

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33
Q

Diamond-Blackfan Anemia

A

Normal** bone marrow cellularity

Macrocytic anemia with erythroblastopenia*****

Well-preserved granulocytic and megakaryocytic maturation

34
Q

Fanconi Anemia tests

A

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

35
Q

Bernard soulier vs VWD

A

BSS = giant plts

36
Q

Hairy Cell Leukemia phenotype

A

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

bright: CD103, CD25, CD11c, and CD22

37
Q

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

A

infants

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

38
Q

Testing for red cell G6PD activity - when?

A

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

39
Q

Serum and plasma tubes

A

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

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

40
Q

Serum

A

Plasma minus fibrinogen

41
Q

What RBC parameter unaffected in CAIHA

A

Hb, WBC, plts all unaffected

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

42
Q

Not included in DIC panel

A

PTT

43
Q

HLH criteria

A

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

Fever
Splenomegaly
Cytopenias
Hyper triglyceridemia &amp;/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
Q

SLE cells

Neuro Lupus

A

S LE –> LE cells

NPSLE due to cytokines (IL6)

45
Q

EBV pos lung lymphoma

A

lymphomatoid granulomatosis

more EBV pos cells –> higher grade

46
Q

RDD histiocyte

A

S100 and CD68

47
Q

Null lymphomas

A

PEL and ALCL

48
Q

serous atrophy of BM aka

A

gelatinous transformation

49
Q

ATLL immunophenotype

A

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

4 and 25 coexpression

50
Q

AITL immunophenotype

A

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
Q

Hepatosplenic T-cell lymphoma (HSTCL)

A

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
Q

Acanthocytes

A

equally spaced

A–> Abeta lipoproteinemia

53
Q

Arterial Thrombosis

A

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

LAC –> only venous

54
Q

thrombin time

A

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

55
Q

Warfarin OD

A

Factor V unaffected, only in liver failure

56
Q

Childhood Myelodysplastic Syndrome (Refractory Cytopenia of Childhood)

A

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
Q

iron storage in BM and reflected in the serum

A

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

58
Q

Type II vWD: _____________ defects of vWF

A

Qualitative defects of vWF

59
Q

Type 2A vWD

A

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
Q

Type 2B vWD

A

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
Q

Type 2M vWD

A

M: multimers still present

Decreased vWF-dependent platelet adhesion

No deficiency of HMWMs and IMWMs

62
Q

Type 2N vWD

A

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
Q

Platelet-type or pseudo-vWD

A

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
Q

Mega Marker

A

CD61

65
Q

Erythro Marker

A

CD71

PAS (cytoplasmic)

66
Q

B-PLL

A

Monoclonal B cell, lacks distinctive immunophenotypic features

67
Q

Primary Cutaneous Follicle Center Lymphoma

A

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
Q

One Symptom of AITL

A

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
Q

AITL morphology

A

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
Q

AITL Pts at increased risk for?

A

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

71
Q

AITL Phenotype

A

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
Q

Pattern of HCL, SMZL, and GDHSTCL in BM

A

interstitial and sinusoidal

esp sinusoidal –> SMZL, and GDHSTCL

73
Q

APL

A

HLA DR neg
CD34 Neg

CD33 Pos

74
Q

refrac cytopenia of childhood vs aplastic anemia

A

lack of erythroids + adipocytosis of marrow = aplastic anemia

75
Q

CLL immunophenotype

A

Weak monotypic surface Ig, weak CD20 –> absence of FMC7

Pos: CD23, CD5

76
Q

Argatroban

A

direct thrombin (IIa) inhibitor

77
Q

Fondaparinux

A

indirect Xa inhibitor

78
Q

Rivaroxaban, apixaban and edoxaban

A

direct Xa inhibitor

79
Q

PCV of castlemann with increased?

A

IL6

80
Q

Types of LyP

A 
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
Q

one main diff between cut ALCL and LyP Type C?

A

clinical, if it regresses –> LyP

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