CP Flashcards
1
Q
Niemann-Pick Disease
A
sphingomyelinase
Foamy
2
Q
Gaucher Disease
A
glucocerebrosidase
Wrinkled paper
3
Q
Factor deficiency with no symptoms
with dramatic PT prolongation
A
XII
4
Q
Which MLL in adults vs Kids
A
9;11 kids (9 months baby)
4;11 in adults
5
Q
DEK NUP2
A
MLD and basophilia
6
Q
Factor ? deficiency in Ashkanizi kews?
A
11
7
Q
Renal Medullary Carcinoma in who
A
Sickle cell trait
8
Q
CBC in sickle cell trait
A
completely normal
9
Q
G6PD pathway?
A
pentose phosphate (hexose monophophate)
10
Q
Pyruvate Kinase pathway?
A
Glycolytic
11
Q
Sucrose lysis test
A
PNH screen
12
Q
Acidified Serum (Ham’s)
A
old PNH test
13
Q
False Neg G6PD test
A
recent attack (too many retics) or female heterozygote
14
Q
Fe def anemia vs Beta thal trait
A
inc RDW in Fe def anemia (both microcytic/hypochromic)
15
Q
Reticulocyte index formula
A
= reticulocytes × patient HCT/normal HCT
16
Q
2 RBC types with inc RNA
A
basophilic stippling and retics
17
Q
Alkaline gel
A
origin –> CSF-A
CEOA2, SDGLe
18
Q
hgb absorbance
A
340 nm
19
Q
met hgb absorbance
A
340+ 200 (cyan+ met) = 540 nm
20
Q
met hgb charge
A
M=3+
M flip
21
Q
Monitor TTP response
A
Plt counts, NOT ADAMTS13 (remains low)
22
Q
Abciximab target
pattern in aggre
A
targets GPIIb/IIIa
looks just like glanzmann’s (only ristocetin response)
23
Q
Ticlodipine
A
target ADP receptor
24
Q
Reverse warfarin effect
A
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
