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112 Cards in this Set

  • Front
  • Back
what is polycythemia

what are the types of polycythemia
Increased RBC. Erythrocytosis, incerased Hb, Hct

1. Relative: Hct is increased bc plasma is decreased (seen in dehydration)
2. Absolute
-Primary: EPO LOW
-Secondary: EPO HIGH. plasma normal, RBC mass increases
what is the cause of relative polycythemia
its when there is increase in HCT bc there is a decrease in plasma

dehydration, diarrhea, cholera, hanta virus
what is the normal breakdown of HCT and Plasma
HCT- 45%
PLasma 55%
what is the Hct and Plasma in:

1. normal
2. secondary Erythrocytosis
3. Polycythemia vera
4. Relative polycythemia
Normal: Hct, 45% PLasma 55%

2. Secondary Erythrocytosis: Hct high, Plasma normal

3. Polycythemia Vera: incerased Hct, Increased Plasma

4. Relative Polycythemia: Hct normal/high (NO increase in RBC mass), PLasma decerased
what is Gaisböck’s syndrome
stress indices polycythemia

associated with HTN, obesity, smoking, stress/anxiety
what is

1. Polycythemia Vera
2. Absolute secondary Polycythemia

**what is EPO level and what are the blood cells doing
1. Polycythemia Vera- "vera" means true. RBC mass, Hct, and PLasma, PMN, platelets all increase. EPO is low. Jak2 Mutation. Myeloproliforative disorder

2. Absolute Secondary Polycythemia- EPO is high at appropriate times ex- high altitude, cyanotic heart disease, pulm disease OR inappropiate bc of renal cell carcinoma, Hepatocellular Ca, cerebellar hemangioblastoma
what type of polycythemia is due to JAK2 mutation. characterize it
Polycythemia vera (absolute primary polycythemia)

myeloproliforative disorder

EPO is LOW

RBC mass, blood volume, PMN, plate ALL INCREASED (myeloid stem cells proliforate)
what is the polycythemia that is EPO independent but occurs bc the BM proliforates in the Absence of GF signal
Polycythemia Vera

*EPO is low, JAK2 mutation makes it myeloproliforative

*absolute primary polycythemia

**RBC mass, plasma, PMN, plate all are increased (myeloid stem cells proliforate)
waht is the classification of polycythemia that occurs when the body is hypoxic and so the kidney sends out more EPO (as is normal)

what if you have polycythemia that has EPO high and you cant figure out why they would have high levels as their O2 is great!
absolute SECCONDARY polycythemia

**recall the primary is "vera" and EPO is low

**can also be a paraneoplastic syndrome- RO renal cell cancer
what is a paraneoplastic syndrome associated with renal cell carcinoma
absoulte secondary polycythemia- renal cell carcinoma sends out lots of EPO
what does hemorrhagic diatheses, what does diathesis mean

what are the 3 categories of bleeding diatheses
makes you more prone or susceptible

1. Increased fragility of BV
2. platelet deficit
3. derangments of coagulation
what are the lab tests for bleeding disorders, what categories of disease can make you bleed
PT, PTT, Platelet count ALWAYS

can also do, bleeding time, platelet studies, vWF

**fragile BV
*platelet problem
*coagulation problem
if PTT is long whats the culprit

what about PT

what about BOTH
PTT- 12, 11, 9, 8

PT- TF, 7

Both: 10,5,2
what test is long when the following is deficient

TF
2
5
7
8
9
10
11
12
PTT- 12,11,9,8
PT- TF, 7
BOTH: 10,5,2

TF
2- BOTH
5- BOTH
7- PT
8- PTT
9- PTT
10- BOTH
11- PTT
12- PTT
PTT stands for...
PT stands for
PTT- thromboplastin time (11, 12, 8,9) intrinsic
PT- prothrombin time (TF, 7) extrinsic
what measures the extrinsic and common path PTT or PT
PT- prothrombin time (TF, 7)
used to monitor coumadin
what measures intrinsic path
PTT- 12,11,8,9
what kinds of things can make you bleed bc the vessel wall is abnormal?

what is the PT- PTT and platelet
vasculitis, bad collagen, defect in vessel, amyloid

PT, PTT, Platelet count all are normal
what are hte causes and consequences of vasculitis
Cause: pathogens (meningiococcemia, rickettsia, DIC, infective endocarditis)
meningiococcemia and rickettsia cause what?
bleeding due to vasculitis (PT, PTT, platelets are normal)

**DIC and infective endocarditis can also cause bleeding bc of BV abnormalities
when might you see a subengual hemmorhage/? what might be another clinical manifestation ocaused by the same thing
infective endocarditis

**they are splinter hemmorhages in the nail bed

**bleeding bc of BV disorder (vasculitis)

**can also get petechiae in the legs and other places
what does meningiococcemia look like
get a rash
*vasculitis that causes hemmorages
tell me about immune/inflammatory vasculitis

causes
immune complex deposition in BV

IC from:
1. RA, SLE
2. hypersensitivity to drug, aka leukocytoclastic vasculitis
3. Unknown: henoch-schonlein purpura
whats leukocytoclastic vasculitis
vasculitis associated with hypersensitvities to drugs
whats Henoch-Schonlein Purpura
-palpable purpura from unknown AG that makes complexes and causes vasculitis

IgA complex deposits in BV

Seen aafter URI
-rash, colicky pain, polyarthralgia, glomerulonephritis
yo man with previous URI in in the hospital with fever, symmetric polyarthritis, rash, abd pain, and hemeuria

waht can be the cause
Vasculitis caused by IgA complex deposition

Henosch Schonelin
The patient, a 54 y/o man with long history of
alcohol abuse, cirrhosis of the liver and
pancreatitis was noted to have a purpuric
macular eruption of the legs.
• Purpura were nontender, 2‐3mm and
associated with hair follicles. Hairs were
twisted into corkscrews.
• His gums were swollen with areas of hemorrhage.
• Fecal occult blood was positive.
• A nutritional deficiency is suspected. Diagnosis?
Alcoholosm associated with thiamine, gums assoc with scurvy- vit C

**purpura with hair follicles, loss of vascular support.
*the collagen is bad
a kid is suspected to be abused bc she AWLAYS is bruised, when the doc takes a look her fingers are hyper mobile, what is a better explaination for the bruising
Ehler Danlos
*also will have stretchy skin
*bruise bc BV are danaged easily
*skeletal deformities

**loss of collagenous support
what 4 conditions are associated with BV damage bc there is a loss of collagenous support
1. Scurvy
2. Ehlers Danlos- collagen 1/3 defective so decreased vascular support --> fragility and easy trauma
3. Cushings: lots of corticosteroid, protein washing and loss of oerivascular support
4. OLD!!!

**keep in mind this type of bleeding (caused by BV fragility) will ahve NORMAL labs
what is the purpura seen in the eldery as a result of normal aging/atrophy
batemen, actinic

**BV fragility leads to hemorrhage, normal labs
Herediatary Hemmorhagic Telangiectasia

AKA
inheritance
Morphology
Clinical
1. called osler weber rendu
2. AD inheritance
3. tortuous thin walled vessels seen in mouth and under nails
4. Clinical- mucosal bleeds (nose, GI) petechiea on mouth, hands, organs
a kid comes in with frequent epitaxis that is NOT due to digital trauma. You note the kid has petechiea in the mouth. The dad also had frequent nose bleeds. what is the problem

(18 y/o male. Suffered from repeated episodes of epistaxis.
Telangiectasias first noticed by a second physician consulted for anemia.
Patient’s father had a milder, undiagnosed form of this disease.)
heriditary telangiectasia (AD inheritance)
also called osler weber rendu

**tortuous thin walled BV in mucosa. lots of hemmorages (BV reason for hemorrhare- normal labs)
A 67 y/o female has multiple telangiectasias on
her hands, arms, legs, lips and nasal mucosa.
• She has had numerous episodes of epistaxis and
GI bleeding since her mid‐20s.
• Telangiectasias are seen endoscopically in her
stomach and colon.
• Her maternal family history is strong for similar
lesions

dx?
inheritance
herideitary telangiectasia- osler weber randeu

AD inheritanec
what are the types. locations and causes of vascular amyloid
1. AL- multiple mylemoa (monoclonal plasma cells)

2. Cerebral Amyloid Angiopathy. ab amyloid accumulation, common in AD. build up in cerebral and cortical vessels
pinch purpura around the eye, nasal bridge is associated with what
amyloid (congo red stain amyloid)

**see periorbital bleeding in systemic amyloidosis
can platelet problems cause bleeding
you bet!

platelet deficit or dysfunction
what is thrombocytopenia
what is the count to be considered thrombocytopenic
when do you have spontaneous bleed
when do you ahve bleed with injury

what is the normal fx of plateles
- bleeding bc of decreased platelets
- platelets <150,000 is thrombocytopenia
- <30,000 spontaneous bleeding
- 30-50,000 post traumatic bleeding
- PT and PTT are normal

PETECNIAE

Normal platelets Fx in primary hemastasis (platelet plug formatin) and as Phospholipid platform for coagulation
what is hypersplenism and dilutional thrombocytopenia
Hypersplenism- when the spleen sequesters lots of platelets

Dilutional- when platelets are diluted by a MASSIVE transfuation
what are the clinical manifestations of thrombocytopenia
Petechiae, ecchymosis, purpura
Mucosal bleeds
Intracranial bleed
what are some cauess of thrombocytopenia
decreased production
decreased survival
sequestration (hypersplenism)
dilutional (massive transfusion)
why might platelet production be decreased? whats in the ddx
1. drug indiced- EtOH, thiazides
2. Infection (HIV) of megakaryocytes


ddx includes pancytopenia
pancytopenia is a decrease in:
platelt, RBC, PMN
what can cause pancytopenia
1. aplastic anemia (no RBC, PMN, plate)
2. myelopthisic disorders: BM is replaced (seen in cancer mets, leukemia/lymphoma, myeloproliforative disease, granuloma)

3. Ineffective magakaryocites: B12/folate defect

4. drug indiced
ok so production of platelets can be low bc of drugs, aplastic anemia, viral infection of megakaryocytes. what can cause decreased survival of platelets
LOTS more things

1. Microangiopathic hemolytic anemia (DIC, TTP, HUS)
2. Giant Hemangiomas
3. Heart valves (prosthetic)
4. HTN
5. Sepsis
IMMUNE MEDIATED
1. AB:AG complex
2. Antiplatelet AB
what is microangiopathic hemolytic anemia
it includes DIC, TTP, and HUS and is a category od diseases that decrease platelet survival through mechanical (non immune) mechanism
what are the mechanical causes of decreased platelet survival
1. Microangiopathic Hemolytic Anemia (DIC, TTP, HUS)
2. Giant hemangiomas
3. prosthetic heart valves
4. Malignant HTN
5. sepsis
what are some immune mediated reasons platelet survival can be decreased
1. immune complexes circulation can cause platelets to form thrombi

2. Antiplatelet AB
pts with SLE get thrombocytopenia why
1 type II reaction against BV
A 24 y/o P1G2 patient with uneventful pregnancy
and labor gives birth to a term infant, Apgars 9,
10 at 2 and 5 minutes.
Neonatal exam of the infant demonstrates
extensive petechiae and scattered purpura and
ecchymoses.
What lab tests would you order?
petechiae and purpura indicated bleeding get PTT, platelets and PT for ALL BLEEDERS

PT normal
PTT normal
Platelet LOW

**can be caused by mom AB attacking baby platelets- neonatal thrombocytopenia
what is the pathogenesis of neonatal thrombocytopenia
Maternal AB against HPA (human platelet AG) on the baby that were inherited from dad

Ab crosses placenta to baby

Immune mediated platelet destruction by alloAB

**can affect FIRST preg and others.
what are the causes of secondary autoimmune thrombocytopenia
1. drugs- heparin, quinidine, sulfa
2. Infections- HIV, CMV, mono
3. Autoimmune disease- SLE (type II HS rxn)
4. Chronic Lymphocytic Leukemia
whats going on in primary idiopathic thrombocytopenia (IPT)
IgG targets non specific epitopes on platelets (perhaps AB to GPIIBIIIa)

splenectomy will work as tx bc you are removing the source of platelet removal
campare acute ITP (idiopathic thrombocyto) and chronic ITP
ACUTE: common, childhood, M=W, self limited!

CHRONIC: males, less than 40, petechia/ecchymoses w/o truauma, hx of nose bleed, increased risk for intracranial hemorrhage, normal spleen, EXCLUDE SECONDARY CAUSE of bleedign. Tx with immunosuppression (cortocosteroids, IVIg, anti CD20). common to relapse
whats the BM and clinical presentation of idiopathic thrombodytopenia (primary)
BM: increased megakaryocytes, they may be immature, they are atypical

Petechiae
large thrombocytes seen on peripmeral smear. Abrupt onet after viral infection. self limited. pupura, mocosal bleeding. Tx with IV Ig, steroids

Spleen is normal in size (compared to how huge it is in spherocytosis nad chronic)
what does it mean when you see megathrombocytes
that there are young platelets

can be ITP
A 27 year old woman presents with petechial
rash over the legs and epistaxis.
• She had delivered of two babies uneventfully
and had no other hospitalizations.
• Three weeks before presentation she suffered
an influenza‐like illness that lasted 5 days. She
recovered and then became fatigued 4 days ago
at which time she noticed the “rash”.
• She took no medication
• Petechiae and ecchymoses were noted.
Influenza tells us its AB mediated- ACUTE PRIMARY IDIOPATHIC thrombocyto

petechiae, test for PT PTT and platelet (ALL bleeders get this)

PT, PTT normal
PLatelts low

Immune mediated primary idiopathic

test for HIV, SLE, adn BM to RO other cause. exclude causes of secondary ITP
Child brought by mother after pickup from day
care where she feared that he had been abused.
• His only abnormalities were superficial bruises.
• Platelet count was 1000/μL (normal >150,000).
• Increased megakaryocytes were present in
his otherwise normal bone marrow aspiration.
• Past history is treatment for sinusitis,
conjunctivitis and bronchitis 2 weeks earlier.
• What is the diagnosis?
• Prognosis? How does prognosis compare with
previous case.
platelets- low
Hx of infection-- AB mediated destruction of platelets

Likely to get over it (often self limited), tx with corticosteroids and IVIg
what are the labs in ITP
PT, PTT normal
platelet decreased
BM megakaryocytes increased
Antiplatelet AB present

EXCLUDE causes of secondary ITP
what is the pathogenesis of HIT type II
Heparin Indiced thrombocyto

heparin is given and the body makes AB to heparin, the AB activates platelets adn forms intravascular thrombi, all of these thrombi are life threatening
what are the clinical features of HIT type II
heparin indiced thrombocyto

-onset 5-14 days after heparin is given
- 50% decrease in platelets
- AB to hepatin is found
- AB activates platelets and we get thrombi formed, this clot fomration is why our platelet count is low
what is type I HIT
most common, seen with unfractionated heparin
platelets aggregate but dont form clots :)
A 67 y/o man was admitted with diagnosis of
deep vein thrombosis and pulmonary embolus.
He c/o SOB, chest pain, and hemoptysis.
• Diagnosis of PE was established by V‐Q scan.
• Patient was started on unfractionated heparin,
6000 units q4h after loading dose of 20,000 units.
He was continued on heparin and oozing was
noted from venipuncture sites on the
9th day

What is the cause of thrombocytopenia
what additional labs are needed
what is the treatment
heparin, get PT, PTT, platelets as baseline before therapy

9th day means its type II HIT


platelets are normal on admission but 10,000 on day 9
PTT/PT prolonged on day 9


HIT type II
Labs: BM?
Tx: STOP heparin
what is the most common blood problem in HIV, whats the mech
thrombocytopenia

HIV infects megakaryocytes and so decreased production
increased destruction bc of immune dysregulation
Auto AB to gpIIbIIIa cross react with HIV gp120
what causes thrombocytopenia bc of thrombotic microangiopathies
TTP, HUS

CLinical: thrombocytopenia, intravascular thrombi, microangiopathic hemolysis
what is the pathogenesis of microangiopathic hemolytic anemias

what are clinical, lab, and morphological findings
1. endo damage--> vWF released and makes platelets stick

2. platelets are tied up and so we get thrombocytopenia, the platelet aggregations for intravascular thrombi

3. mechanical lysis of RBC leads to microangiopathic hemolytic anemia

TTP, and HUS see schistocytes
what is seen on smear that makes you think HUS and TTP (microangiopathis hemolytic anemai)
schistocytes (decapitated RBC)
what are schistocytes
decapitated RBC seen in microangiopathic hemolytic anemia (HUS, TTP)
what ar ehte 2 trpes of thrombotic microangiopathies

what are some common features
o Thrombotic Thrombocytopenic Purpura (TTP)
o Hemolytic Uremic Syndrome (HUS)

FEATURES
oHyaline thrombi in any blood vessels in the body
oShould see fragmentation of RBC’s (schistocytes) on peripheral smear due to hemolysis causes elevated bilirubin
oMacrocytes with polychromasia
oIncreased unconjugated bilirubin
Decreased haptoglobin
oEndothelial activation of von Willebrands factor
how are fragmented cells (schistocytes) formed in HUS nad TTP
decapitated by fibrin
waht is TTP
Thrombotic Thrombocytopenic Purpura

Deficit in ADAMTS 13 (protease for vWF)

sx: FAT RN goes crazy -->
Fever
Anemia (schistocytes/hemolysis)
THrombocytopenia
Renal Failure
Neurologic signs

FATAL if you dont do plasmaphoresis
thrombotic thrompocytopenic purpura are seen when?
Following febrile illness. Its when ADAMT 13, the vWF protease, is broken and we get lots of pletelet thrombi form in vasculature. there are also schistocytes formed.

FAT RN
Fever, Anemia (hemolysis/schistocytes) Thrombocytopenia, Renal failure, neurologic

Needs plasmaphoresis to take out the clots
This patient was seen for menometrorrhagia.
She was anemic and underwent a diagnostic
D&C at which time leiomyomata were
discovered.
Patient recovered uneventfully.
• The next day, she suddenly developed a
severe headache and became unable to
speak although she understood simple
commands.
A right facial droop was noted. Lumbar
puncture yielded xanthochromic,
hemorrhagic CSF. She was febrile with
blood pressure 150/80.
• Petechial hemorrhages and multiple
splinter hemorrhages were present.
• CT of the head showed a tiny hemorrhage
of the right parietal lobe and infarctions of
the left frontal lobe and right cerebellar cortex

UA: protein, 0-5 hyaline casts, RBC, WBC

CBC: low Hb, platelets low, RBC morph- schistocytes, reticulocytosis 1 day later

PT, PTT, fibrinogen- normal, ro DIC

CHemistry- renal disease, mm degradation (AST, LDH, CPK)

Whats the Dx
Whats the treatment
HA, speach problems- Neurologic (FAT RN)

Xanthochromic- blood, protein

Hemorrhages- low platelets

UA- indicated renal disease (FAT RN)

Thrombotic Thrombocytopenic Purpura (TTP) (Fever, anemia, thrombocytopenia, renal, neurologic, schistocytes)

Treatment: START ASAP, can be life threatening. Plasmaphoresis to take out vWF complexes

TTP- ADAMT 13 (vWFprotease) is deficient so we get lots of platelet thrombi
what is the clinical feature of HUS and cause
1 E coli O157H7- shiga toxin is toxic to endo

2. Sx: bloody diarrhea, acute gastroenteritis, then renal failure

3. NO AB!!! it makes it worse

4. Renal Failure predominated
in HUS and TTP we have renal failure, what is a good wat to tell them apart
TTP has neufrologic signs

HUS- some E coli with shiga toxin
who gets HUS commonly?
is there a genetic predisposition
HUS- E coli, shiga toxin

Common in kids and old
Genetics: compliment regulation

HUS- blood diarrhea, gastroenteritis, then its RENAL FAILURE

**in HUS the shiga toxin damages endo so vWF is released and we get lots of intravascular thrombi formed, this is what clots up the glomerulus and leads to renal failure
After eating potato salad, a 71 y/o man
develops bloody diarrhea. In a few days,
he is in acute renal failure with thrombocytopenia,
and hemolytic anemia. His PT,
PTT, are normal. He dies of myocardial
infarction. Hyaline microthrombosis is noted
in multiple organs including the renal glomeruli.
Extensive petechiae are present in the
epicardium.
HUS- potato salad indicated E coli O157H7

MI- bc of thrombi

Shiga toxin damages BV and so vWF makes clots.
what are some causes of HUS besides E coli with shiga
radiation
drugs

**will still get irreversible kidney damage
how are the microangiopathies treated

PPT
HUS
PPT- plasmaphoresis ASAP

HUS- NO AB!!! supportive
inheritance of clotting factors

X linked
AD
AR
x- 8 9
dom- vWF
recessive- ALL OTHERS

**genetic deficit usually one factor affected
**if more than 1 factor is affected its usually acquired (liver damage, vit K-7 9 10 2 protein C deficit, DIC)
what factors are affected with vit K deficit
7 9 10 2 protein C
**coumadin can cause vit K deficit

**in acquired deficits like this several factors are affected! if genetic usually a single factor

other acquired:
liver disease
DIC
what are the causes of clotting factor deficits (4)
1. Vit K deficit. 7 9 10 2 protein c (coumadin induced)

2. Liver disease

3. Genetic
X linked- 8 9
AD- vWF
Recessive- all others

4. DIC
what coagulation factor travels with vWF
8, it extends the life of 8

**when vWF is deficient (AD) it can look like an 8 deficit (8 deficit is hemophilia A)
vonWillebrain Disease

1. Inheritance
2. Clinical features
3. labs
4. treatment
1. autosomal dom

2. Clinical: spontaneous bleed from mucosa, excess blood from wounds, heavy menses,

3. LABS: NORMAL platelet count, PROLONGED bleed time

4. Tx: desmopressin, cryoprecipitate, NO aspirn
what is type I vWF diseae
what about type 3
1. most common, AD inheritance, mild bleeding. vWF decreased but PTT is normal. its a partial deficit, one allele is normal

3. SEVERE- autosomal recessive. its TOTALLY different acts as factor 8 deficit (vWF binds 8 to extend t 1/2) PTT is prolonged
This 5 year old boy was seen for a marked
tendency for bruising of the arms and legs.
• He had an episode of epistaxis at age 3 that
required hospitalization 1 unit transfusion with
packed cells.
• Family history: The mother, maternal
grandfather and maternal greatgrandmother
had episodes of abnormal bleeding‐epistaxis,
easy bruising and menorrhagia. The mother
had required 10 units of RBCs at delivery

The family history suggests what pattern of
inheritance?
• Does the pattern of clinical bleeding suggest an
abnormality of primary or secondary hemostasis?
• What additional lab tests are indicated?
• If the patient requires surgical intervention, what
would be the treatment of choice for a bleeding
complication
inheritance suggests AD bc its in all generations

vWF disease, type I

superficial bleeds, primary hemostasis

PT, PTT, platelet, bleed time (expect bleed time to be long)

Tx with desmopressin
what are the characteristics of clotting factor deficiencies (except vWF, its a bit different)
1. large ecchymoses/hematoma
2. hemarthrosis
3. prolonged bleeding
4. GI GU bleed

**deep bleeds (recall vWF deficit was superficial mucosal bleeds)
An individual with hemophilia A is unable to
solidify the platelet plug formed following
endothelial damage; this leads to abnormal
bleeding. This condition is caused by a defect
in the coagulation cascade. Which of the
following elements of the coagulation cascade
is defective in persons with hemophilia A?

A) Factors II, VII, IX, and X
B) Factor VIII
C) Factor IX
D) Lack of protein C
E) Mutations in antithrombin III
factor 8

A) Factors II, VII, IX, and X. VIT K
B) Factor VIII- Hemophilia A
C) Factor IX- hemophilia B "christmas disease"
D) Lack of protein C- Thrombosis
E) Mutations in antithrombin III- Thrombosis
Factor 8.9 deficit

1. Clinical
2. Inheritance
3. Labs
8- hemophilia A **more common**
9, hemophilia B

1. Clinical: hemarthrosis, deep hematomas, "deep bleed", spontaneous hemorrhage, NO PETECHIAE

2. Inheritance: x linked recessive

3. LABS: PTT long in both. B will also have abnormal facoor 9 assay
wht is christmas disease
hemophilia B, factor 9
what is a factor 8 inhibitos
anti factor 8 AB that develops in some pts with hemophilia A

x linked inheritance: seen in males with no fx
if 20-30 times a year you have spontaneous bleeds and deep hematomas with NO petechiae what can be the problem
hemophilia A or B

**no petechiea bc platelets are normal
in what disease can you see a pseudotumor 2 to hematoma
hemophilia, this gets DEEP bleeds and no petechais (nothing wrong with platelts)

can also get cystic remodling of glenohumeral joint (bone cyst bc of a hematoma in the shoulder)
tell me about hemarthrosis seen in hemophilia a and b
deep bleed into joints

cyctic deformity, hemosiderin makes it dark- lack of blood
This 7 day old boy is seen for evaluation for a
bleeding disorder.
• He was the product of a normal delivery to a
healthy 22 year old primagravida. On the
second day after delivery, he was circumcised
and bled for 4 days, but received no specific
treatment.
• A maternal uncle was diagnosed with
hemophilia A at age 18 months when a fall
produced hematoma of cheek and eye.

The uncle’s record shows a PTT of 88 sec (<42)
and Factor VIII activity of <1%.
• Patient received factor concentrates and was
discharged. He continued to have frequent
episodes of bleeding and required factor VIII
concentrates twice a month.
• Patient’s lab PT 12 seconds (10‐12)
PTT 111 seconds (<42)
• Factor VIII activity <1%
• Mother‐Factor VIII activity 19%
PTT is long in hemophilia

Platelet and PT are normal
how is hemophilia A treated
recombinant factor 8 is given prophylactically, not just when you start to bleed
A 49 y/o male was seen for large hematoma
over anterior surface of tibia where he had
been accidentally struck.
• Past history: Hemorrhoidectomy, required
5 unit blood transfusion.
• GI bleeding 2 yrs. later due to duodenal ulcer;
‐ gastrectomy, 11 units blood transfused.
• GI bleeding due to ulcer at operative site;
further surgery with 20 units transfused.
• Bleeds profusely from dental extractions. Younger brother diagnosed with factor IX
deficiency.
‐ History of recurrent GI bleeding with
surgery; multiple transfusions.
• One brother with no history of bleeding
• One sister with excessive bleeding following
nasal polypectomy that did not require
transfusions.

what do you think the labs are (PT, PTT, Platelet)

what is the most likely dx

what is the inheritance

whats the confirmatory test
PT, platelet normal
PTT long

most likely: factor 8, 8 is more common than 9

X linked recessive (both 9 and 8)

Confirm with factor 8.9 assay
whats the difference in clinical presentation btwn factor 8 adn 9 hemophilia
nothng

8 and 9 are BOTH x linked recessive
Hemophliaa B

1. deficit
2. another name
3. inheritance
4. lab
1 9
2. christmas disease
3. x linked recessive
4. long PTT, 9 assay abnormal
what ist he most common serious autosomal factor deficit?


wat are the labs
factor 11 (vWF is a little more common)
common in ashkenazi jews

PTT long,
A 65 year old woman bled for 10 days after a
dental extraction. One sibling died from postop
hemorrhage; there is a history of bleeding in a
number of relatives. PTT is markedly prolonged
and bleeding time w/in normal limits. Diagnosis?
a. factor VIII deficiency
b. factor XI deficiency
c. factor XII deficiency
d. factor IX deficiency
 e. von Willebrand’s disease
factor 11

Female so not 8 or 9
Bleed time normal so not vWF
an Ashkenazi Jewi has increased PTT, what is a common autosomal coagulation factor deficit they might have
11
tell me about DIC
thrombo-hemorrhagic disorder

**systemic activation of coagulation leads to thrombi throuought microcitculation

**fibrinolysis is activated, d dimer is high

**common as a secondary complication of lots of diseases
what are 2 mechs that trigger DIC (systemic activation of coagulation cascade)
1. Release of tissue factor or thromboplastic substances into circulation
-released from placenta, cancer, mucin,

2. Eidespread endo damage
-sepsis
- endotoxins (LPS) exotoxins activate endo

also heat stroke
DIC

1. clinical
2. lab
3. cause
1. Clinical: thrombic/hemorrhage. ischemia, microangiopathic hemolytic anemia

2. Lab: FDP and d Dimer increased

3. Cause: sepsis, obstretics, cancer, trauma, snake bite, vasculitis, liver disease hemolytic transfusion reaction
what is one of th emost common causes of DIC
obstretics (premature seperation of placenta, amniotic embolus, retained dead featus)

sepsis

cacner, trauma, snake bite, vasculitis, liver disease, hemolytic transfusion reaction
when do you see Purpura fulminans
DIC of meningiococcemia (N meningitidis)
what is the pathogenesis of DIC
massive tissue injury, sepsis, or endo injury all cause release of tissue factor

tissue factor leads to widespread microvascular thrombosis

this leads to:
1. plasmin activation --> fibrinolysis --> icnreased d dimer --> bleeding
2. vascular occlusion --> ischemic tissue damage

BLEEDING
what might be the reason someone has micothrombi in arterioles and capillaries of kidney, adrenal, brain, heart

waterhouse Friderichensen syndrome
DIC

Waterhouse Friderichesen- BL hemorrhage of adrenals
what is chronic DIC due to

what is Acute DIC due to
CHRONIC: cancer, recurrent DVT, no bleeding diathesis

ACUTE: lots of bleeding that wont clot, can lead to shock, acute renal failure, ARDS, circulatory failure
76 y/o female admitted with fever and vomiting.
She was aphasic from a previous CVA (stroke)
and fed via a PEG tube. She had been treated by
her caretaker daughter for a urinary tract infection.
• She was cold and clammy on admission to the
hospital. Her temperature was 97oF, BP 101/42,
respirations 18 and heart rate 133.
• She had no urinary output and remained oliguric
despite IV fluids. Hemodialysis started.
She experienced respiratory distress later on the
day of admission and was placed on a ventilator.
Acrocyanosis developed.
• She continued to deteriorate and was pronounced
dead of multiple organ system failure due to septic
shock within 48 hours of hospitalization.

Blood pH 7.05 (7.2‐7.4)
• Coagulation tests revealed prolonged PT, PTT
and decreased platelets
• AST, ALT, BUN, Creatinine, Bilirubin, and CK were all
elevated
• WBC 28,000 5000‐10,000 /dL
• UA showed heavy branched and budding yeast,
2+ bacteria, > 200 WBCs/hpf, 10‐12 RBCs/hpf
What is the cause of her abnormal coagulation tests?
What is her diagnosis?
COagulation: acute DIC that progressed into septic shock
This 15 y/o boy presented with fever, tachycardia,
and tachypnea that rapidly progressed to
unconsciousness within hours.
• He was admitted to intensive care unit in septic
shock with a temperature of 40.2oC, pulse180/min.
and BP 70/45 mmHg. PT was 90 seconds (~12 sec.).
• Petechia and purpura developed and spread on his
face, arms and legs. Hands and feet were cool and
cyanotic.

Neisseria meningitidis was subsequently
confirmed by biopsy of skin lesions and blood
culture.
• What is the diagnosis?
• What serious complication is associated with
this disease?
• He recovered but required amputation of both
forefeet and seven fingers. With reconstructive
surgery and orthopedic shoes, he is able to walk
and use remaining fingers in a pincer grip.
meningiococcemia

**can loose a finger or toe
what is it called when your adrenal glands die in meningiococcemia
waterhouse friderichsen
A 57 year old alcoholic male was admitted to the
hospital for non‐life threatening injuries sustained
in a fall. He was known to have cirrhosis of the
liver.
• He developed a fever on his second hospital day
and blood cultures were positive for S. aureus.
He was placed on antibiotics. Shortly thereafter,
oozing was noted from venipuncture sites and
the wounds from his fall.

Admission 2nd Day Reference
PT 14 seconds 17 seconds (10‐12 seconds)
PTT 39 seconds 43 seconds <42
Platelet count 90,000/mm3 35,000 >150,000
Confirmatory tests:
Fibrinogen 100mg/dl 170‐410mg/dl


• Septicemia
 Associated endothelial damage
 Macrophage activation: release of tissue
 factor
• Liver unable to clear activated coagulation factors
• Decreased production of coagulation factors
FDP >100μg/ml <10 μg/m
PT, PTT increase on 9th day adn plalet droped

fibrinogen increases and FDP dropped