hematology Flashcards
1
Q
different degenrative diseases of back explain
RADUCLOPathy,spondyloarthopathy, spinal metastasis
A
Relieved with rest
• Cervical and lumbosacral
Radiculopathy
• Radiates to leg, positive straight leg raise
test Spinal stenosis
• Pain with standing, relieved with flexion
Spondyloarthropathy
• Relieved with exercise Spinal mets
• Constant pain, worse at night, no
response to position change Osteomyelitis
• Focal tenderness, fever, night sweats
Bone mets PB/KTL
• Prostate, Breast, Kidney, Thyroid, Lung
2
Q
explain pathogeneisis of HEmochromatosis
A
HFE protein normally interacts with
transferrin recognizing Fe uptake from GI
tract > mutation leads to falsely low
detection of Fe > no hepcidin production >
increased DMT1
3
Q
how to ifferentiate between hemophilia and antiphospholipid antibody syndrome
A
use mixing study
4
Q
how to differentiate between pilocytic astrocytoma, and medulloblastoma
A
Pilocytic astrocytomas are the most common brain tumors in children. They frequently arise in the cerebellum and can be differentiated from medulloblastomas by the presence of both cystic and solid components on imaging.
5
Q
histopath of brain tumora
A
Medulloblastoma • 2nd MC (MC malignant) • MC in vermis • Small blue cells with drop mets Ependymomas • Perivascular rosettes • Usually in ventricle causing hydrocephalus Adults GBM • Pseudopalisading Oligodendroglioma
6
Q
why vitk supplemetation necessary in newborn
A
Newborns who don't receive prophylactic supplementation are at risk for bleeding complications. Pts w/ CF are also at risk for vitK defic due to poor absorption of fatsoluble vits.
7
Q
problems with antiphospholipid syndrome
A
Pts w/
antiphospholipid Abs are at risk
for venous and arterial TE and
unexplained, recurrent preg loss
8
Q
what if we give a person B9 with a b12 deficiency how to differentiate between b12 and folate
A
If megaloblastic anaemia due to vitB12 defic is mistakenly Tx w/ vitB9 alone, the neuro dysfxn can worsen. B12 def • High MMA and homocysteine, peripheral
9
Q
how do you diagnose multiple myeloma
A
system). A
bone marrow sample w/ >10%
plasma cells is strongly
suggestive of MM.
10
Q
how does hypothyroid present with
A
Macrocytic RBC without hypersegmentel
11
Q
what are the classic signs seen in hereditary spherocytosis how to diagnose it
A
hemolytic anaemia, jaundice, and splenomegaly ↑ osmotic fragility on acidified glycerol lysis testing confirms the Dx.
12
Q
manifestations of g6pd deficiency
A
Infections, dapsone, antimalarials, TMp
13
Q
explain mechanism of cachexia
A
TNF-a is thought to mediate PNP
cachexia in humans by
suppressing appetite and ↑ BMR
Anorexia, malaise, anemia, weight loss, muscle wasting all driven by TNFalpha (aka cachectin) • Produced by macrophages • Inhibits appetite in hypothalamus, increases BMR • Mediates symptoms of septic shock and causes liver to release acute phase reactants IFNalpha • Antiviral and anti tumor
14
Q
RAS oncogene
ERB1
ERB2
A
cholangiocarcinoma, pancreatic
adenocarcinoma
ERB1 (EGFR): lung adenocarcinoma
ERB2 (HER2): breast cancer
15
Q
explain metalloproteinase
A
Zn containing enzymes • Degrades ECM and BM (laminin and collagen type 3 and 7) Cancer invasion • Tumor cell detach by decreasing e cadherins • Tumor cell ahere to BM (facilitated by laminin) • Tumor cell produce metalloporteinase and cathepsin D protease to invade Random Acid hydrolase: lysosomal enzyme Alk Phos: bone or biliary process issue
16
Q
what are leukoerythroblasts
A
Teardrop cells
• Nucleated RBC
• Marrow fibrosis and mets
17
Q
funtion of tdt
A
Adds nucleotides to V,D and J region to
increase Ab diversity
18
Q
AMl cuses anemia
A
• Myeloblast unable to differentiate >
suppress other cells
• Anemia, thrombocytopenia, neutropeni
19
Q
which diseases have psitive coombs in autoimmune hemolytic anemia
A
SLE, Hodgkin, NHL, mycoplasma, EBV
20
Q
heritditary spherocytosis
A
mchc over 36
21
Q
how to compare LR with CML
A
LR LAP normal or Increased, in cml decreased
22
Q
what are the complication of ebv in hiv
A
Latent EBV can reactive > can cause B cell lymphoma, burkitts and CNS lymphoma BK virus • Typically post transplant nephropathy or hemorrhagic cystitis
23
Q
EBV
A
• CMV, HIV and Toxo:
24
Q
what characterizes a tissue anaplastic
A
- Loss of cell polarity and architecture
- Variation in shape and size
- High N:C ration
- Many mitotic figures
- Giant, multinucleated tumor cells
25
talk about kapsoki sarcoma
```
s. This tumour arises from
primitive mesenchymal cells and
is strongly a/w HHV-8.
Late stage spread to lungs and GI tract
• Spindle and endothelial cell proliferation,
RBC extravasation and inflammation
• HHV8
```
26
aplasticanemia causes
```
Autoimmune
• Parvo and EBV
• Carbamazepine, chloramphenical,
sulfonamides
• Benzene or radiation Dx
• Hypocellular bone marrow composed of
fat
•
```
27
pure red cell aplasia
```
• IgG against erythropoietic precursors and
progenits
• Associated with thymomas and
lymphcytic leukemia and Parvo B19
• Thymectomy usually cures
```
28
fanconi anemia
Causes aplastic anemia
• Short stature
• Absent thumbs
• High risk of malignanc
29
myeodysplasia
Defect in stem cell maturation >
| pancytopenia
30
why reticulocytes are blue
```
↑ bone marrow EPO results in an
accelerated release of immature
RBCs (reticulocytes) into the
bloodstream. Reticulocytes
contain bluish cytoplasm and
reticular precipitates of residual
ribosomal RNA.
```
31
mediastinal mass
Large anterior mediastinal mass can
compress great vessels (SVC syndrome),
cause esophageal dysphagia, compress
trachea (stridor and dyspnea)
32
talk about different spleen patho
```
Splenic hyperplasia/hypertrophy
• Infection
Splenic pressure atrophy
• Tumor
Splenic lipid accumulation
• Lysosomal lipid storage disorder
(Gaucher's)
```
33
b12 deficiency
Give B12 and will see
• Immediate rise and then fall in
reticulocyte coun
34
what does erythroid cells in spleen indicate
```
The presence of erythroid
precursors in organs such as the
liver and spleen is indicative of
EMH, a condition characterised
by EPO-stim, hyperplastic
marrow cell invasion of EM
organs.
```
35
extramedullary hematopoeisis
```
Caused by chronic hemolysis
• Can see "crew cut" on xray or chipmunk
facies
• Intrahepatic extramedullar
hematopoiesis can cause portal HTN
```
36
why normal serum fe in aplastic anemia
because absent HPO stem cells cant use iron
37
what does lead poisoning do to rbc
• Basophilic stippling on hypochromic
microcytic anemia > abnormal degradation
of rRNA
38
what does integrin bind to
```
Adhesion of cells to the ECM
involves integrin-mediated
binding to fibronectin, collagen,
and laminin
s integrins, matric collagen, GAG
```
39
function of GAGS
```
GAG that contributes to
H2O retention in ECM and determines
stiffness of matric
Keraton sulfate: GAG in ECM that
maintains type 1 collagen fibrill
organization (like cornea)
```
40
what are the causes of splenic infarction
• Sickle cell, infectious endocarditis and
| myeloproliferative disorder
41
explain peneerace of VWD
. It has
an AD pattern of inheritance and
variable penetrance.
42
what is dysfibrongeimas
Abnormal fibrinogen causing excessing
bleeding or thrombophilia
• Normal bleeding time
• Abnormal TT, PT, and PTT
43
transglutaminase crosslinking deficiency
=bleeding
44
pathophys of itb
```
Ab to GP2b/3a
• Low platelets
• Normal rest of labs
• Secondary ITP associated with HIV or
Hep C
```
45
hypotyrodisim ffect on rbc
Fe deficiency anemia or pernicious
| anemia
46
whch bacteria sickle cell ppl get
```
Bone necrosis leading to osteomyelitis
(Salmonella and Staph)
• Splenic infarction > scarring, fibrosis and
atrophy
• Infant: splenic sequestration crisis
(pooling of RBC) > low Hb, rapidly
enlarging spleen and hypovolemic shock
```
47
what is sickle cell crisis
• Infant: splenic sequestration crisis
(pooling of RBC) > low Hb, rapidly
enlarging spleen and hypovolemic shock
48
what sort f anemia is seen in sickel cell disease
```
An MCV >110μm3 is highly
suggestive of megaloblastic
anaemia, such as that caused by
vitB9 or vitB12 defic. Pts w/
chronic hemolytic anaemia have ↑
vitb9 req due to ↑ erythrocyte
turnover and are predisposed to
devel macrocytosis.
```
49
can extramedullary hemtoporisi cause macrocyic anemia
Extramedullary erythropoiesis in sickle
cell can cause macrocytic anemia BUT you
must check reticulocyte index (if elevated
retic index > adequate marrow response)
50
exain her 2
```
The HER2 oncogene encodes for
a transmembrane glycoprotein w/
intrinsic tyrosine kinase activity
and is a member of the family of
EGFRs. Overexpression of this
protein is a/w a worse prognosis
and ↑ risk of disease recurrence.
```
51
explain pathogenessi of ebv
```
Binds CD21 infecting B cells
• CD21 (CR2) binds C3d
• Atypical CD8 cells have abundant
cytoplasm, eccentrically placed nucleus,
and skirting
```
52
active plasma cell
wagon wheel apperaence because of perinuclear clearing
53
genetics of non small cell carcinoma
• Chromosome 2 fusion between EML4
| and ALK >
54
mutation in pcv
• V617F mutation involving JAK2 gene
resulting in more sensitive receptor to EPO
and TPO
55
how does hypoxa present in copd and osa
COPD, OSA
| • SaO2 < 92; PaO2 < 65
56
which cancers present with EC epo
• RCC, HCC, pheo, hemangioblasoma,
| leiomyoma
57
in leukmoid reaction what des the peripheral smear look like
```
peri smear can show
↑ bands, early mature neutrophil
precursors (e.g. myelocytes), and
granules (e.g. Dohle bodies) in
the neutroph
Will also see increase bands, toxic
granulations and cytoplasmic vacuoles
```
58
how to treat ttp how o dy/dx from dic
Need hemolytic anemia to dx Tx:
plasmapheresis
pt and ptt
59
classic triad of HSP
• Palpabale purpura, abdominal pain,
arthralgia
• Normal platelet count
60
bone lytic cancers
MM, non small cell of lung, NHL,
| RCC and melanom
61
bone blastic cancer
: prostate (blacks at higher risk),
| small cell of lung, Hodgki
62
how does pnh destroy nephron
• Chronic hemolysis > Fe deposits in
| nephron (especially PCT) > hemosiderosis
63
explain process f cancer cahcexia leading to mucle loss
```
In carelated cachexia, high lvls of proinflammatory cytokines lead to ↑
ubiquitination of sarcomeric
muscle proteins, which in turn
leads to extensive skeletal muscle
loss.
```
64
most likely caus of opc cnacner
hpv
65
explain histopath of melanoam
```
s. Dx is gen made when a
histopath sample shows cellular
atypia w/ cells containing brown
pigment (melanin granules);
immunostaining for
melanin/melanoma markers (e.g.
S-100, HMB-45) is gen (+).
```
66
histopath f mm
Histopath will show
replacement of the normal bone
marrow w/ plasma cells and
blasts.
67
diagnosis of cll based on
```
en CBC reveals dramatic
leukocytosis, and FC
subsequently shows a clonal
population of leukocytes w/
typical B-cell markers such as
CD19, CD20, and CD23.
```
68
what is the histopath in parvo virus infection of cell
pronormoblasts w/ glassy,
| intranuclear viral inclusions.
69
histopath of ewin sarcoma
```
It most
commonly involves the lower
extremity and pelvis and often
metastasizes to the lungs.
Histopathology is Chx by
uniform, small, round, cells;
fibrous septae; and patches of
necrosis and hemorrhage.
```
70
difference between band neutrophil vs segmented
Band neutrophils are slightly less mature than segmented neutrophils and have indented, unsegmented "C" or "S" shaped nuclei. Band neutrophils normally account for approximately 5-10% of peripheral blood leukocytes. An increased proportion of band neutrophils can be seen in infectious and inflammatory conditions.
71
monocyes morph
Here is a monocyte. It is slightly larger than a lymphocyte and has a folded nucleus. Monocytes can migrate out of the bloodstream and become tissue macrophages under the influence of cytokines. Note the many small smudgy blue platelets between the RBC's.
72
what is left shift
A very high WBC count (>50,000) with pronounced left shift that is not a leukemia is known as a "leukemoid reaction".
73
lead posionng basophilic stippling
Basophilic stippling due to inhibition of
| 5' nucleotidase resulting in retained RNA
74
what is hoell olly odies
DNA remnant in RBC with asplenic pt
75
explain pathogensis of warfin induced necrosis
thrombotic occlusion of the
| microvasc w/ skin necrosis.
76
antitrombin3 deficiency
AD
• Presents with venous thrombosis
• No change in PTT with hep > blast with
hep until you can bridge to warfarin
77
pathophys of beta thalessemia
transcription, processing, and
| translation of beta-globin mRNA
78
how are target cells gformed
erythrocytes decrease volme and excessive membrane
79
why do pts with splenectomy develop target cells
```
Pts
who undergo splenectomy usually
develop target cells b/c the spleen
is the 1ary organ that prunes
excessive RBC membrane.
```
80
molecular pathogenesis of MM
```
Myeloma
cells stim osteoclast devel by
secreting RANKL and destroying
OPG, which incr RANK activity
and results in osteoclast diff.
```
81
when do rbc sickle
• RBC sickle when deoxygenated or
dehydrated (causing chain polymerization
via hydrophobic bonds)
82
gardos
Gardos (Ca dependent) K channel
blockers prevent dehydration by prevent K
efflux
83
opiods tolerance symtoms
* Pruritis is mild and tolerance develops
* Nausea develops tolerance
* Can decrease micturation reflex
84
explain the MDR 1
ATp dependent
efflux pump (normal in nephron and BBB)
• Verapamil, diltiazem, ketoconazole:
reduce action of pump
```
he human MDR1 gene codes for
P-gp, a transmembrane ATPdependent efflux pump protein
that has a broad specificity for
hydrophobic compounds. This
protein can both ↓ the influx of
drugs into the cytosol and can ↑
efflux from the cytosol, thereby
```
85
why never give leuovorin
5• Fluoruracil
• Inhibits thymidylate synthetase
• Leucovorin potentiates the cytotoxic
effect
86
how to cancer cells avoid imune suppression
Ca cells avoid immune
recognition by overexpression PDL1, which binds to the PD-1
receptor on Tc cells and inhibs
their response (T-cell
exhaustion). mAbs that block PDL1 and PD-1 are effective against
ca that express high lvls of neoAgs on their surface b/c these
tumours are particularly
susceptible to immune
recognition by Tc cell
87
cdks 4/6
g1-s
88
what is Emicizumab
```
hemophilia A is an XL disorder
a/w a defic of FVIII. Emicizumab
— a bispecific, mAb — mimics
the activity of FVIII by binding to
both FIXa and FX, bringing them
into close proximity to allow for
FX activation.
```
