Krafts - Bleeding Disorders

Front 1

von Willebrand disease
Hemophilia A and B
Hereditary platelet disorders
These are all what type of bleeding disorders?

Back 1

Hereditary Bleeding Disorders

Front 2

The following are all ________ bleeding disorders:
DIC
ITP
TTP/HUS
Others

Back 2

Acquired

Front 3

Patients with platelet disorders tend to bleed from ______ __________ and ____, show petechiae, and happens spontaneously usually.

Back 3

mucous membranes
skin
(nosebleeds, GI bleeds, menorrhagia)

Front 4

Patients with coagulation disorders tend to bleed into ______ and ____ ______ (they get large _________ bleeds), usually after some memorable trauma.

Back 4

joints
soft tissue
intramuscular

Front 5

Patients with ____________ (low platelets) present with spontaneous bleeding from mucous membranes and skin and increased risk of intracranial bleeding

Back 5

thrombocytopenia

Front 6

Term for a confluence of petechiae

Back 6

Purpura

Front 7

- Most common hereditary bleeding disorder
- Autosomal dominant
- certain factor decreased (or abnormal)
- Variable severity (nosebleeds, bruising to severe bruising)

Back 7

von Willebrand disease

Front 8

von Willebrand factor:
- Glues _______ to endothelium
- Carries factor ___
- Huge multimeric protein
- Made by __________ and endothelial cells

Back 8

platelets
VIII (free factor VIII is destroyed)
megakaryocytes

Front 9

What disease/condition is this?
-Bleeding time: prolonged
-PTT: prolonged (“corrects” with mixing study)
-INR: normal
-vWF level decreased (normal in type 2, low in type 1, absent in type 3)
-platelet aggregation studies abnormal

Back 9

von Willebrand disease

Front 10

Treatment for von Willebrand Disease:
DDAVP (_____ VIII and vWF levels)
Cryoprecipitate (_______ vWF and VIII)
Factor VIII

Back 10

raises
contains

Front 11

- Most common factor deficiency
- X-linked recessive in most cases (30% are random mutations)
- Factor VIII level decreased (gene mutation)
- Variable amount of “factor” bleeding

Back 11

Hemophilia A

Front 12

What does this these test results indicate?
- INR, TT, platelet count, bleeding time: normal
- PTT: prolonged (“corrects” with mixing study)
- Factor VIII assays: abnormal
- DNA studies: abnormal

Back 12

Hemophilia A or B

Front 13

What 2 things can you use to treat Hemophilia A?

Back 13

DDAVP (stimulates patient's endothelial cells to release factor VIII)
Factor VIII (for patients with severe hemophilia and can't make any factor VIII. Want to give little amounts so they don't make antibodies to it)

Front 14

Hemophilia B
- Factor __ level decreased
- Much less common than hemophilia A
- Same inheritance pattern which is what?
Same clinical and laboratory findings

Back 14

Factor IX
X-linked recessive (30% random mutations)

Front 15

2 other Factor Deficiences that are rare:
XI defeciency - bleeding only after ____
Factor __ deficiency (cross-links fibrin): severe neonatal bleeding, bad

Back 15

trauma
Factor XIII

Front 16

The only way to tell Hemophilia A and B apart is by doing what assays?

Back 16

factor VIII and factor IX assays

Front 17

Bernard-Soulier Syndrome (hereditary platelet disorder) has abnormal ___ leading to abnormal adhesion, big platelets, and severe bleeding

Back 17

Gp1b (vWf binding site)

Front 18

Glanzmann Thrombasthenia (hereditary platelet disorder)
No ____ so no aggregation and severe bleeding

Back 18

GpIIb-IIIa (binds fibrinogen)

Front 19

Gray Platelet Syndrome (hereditary platelet disorder) has no _-______, so big empty platelets with mild bleeding

Back 19

no alpha-granules

Front 20

Delta granule deficiency can be part of what syndrome?

Back 20

Chediak-Higashi

Front 21

- Lots of underlying disorders
- Something triggers coagulation, causing thrombosis
- Platelets and factors get used up, causing bleeding
- thrombo-hemorrhagic
- Microangiopathic hemolytic anemia (fragmented RBC)

Back 21

Disseminated Intravascular Coagulation

Front 22

Causes of DIC:
__ complications
Malignancy - especially _________
sepsis
major _______

Back 22

OB complications
adenocarcinoma

Front 23

What would these tests indicate?
-INR, PTT, TT prolonged
-FDPs: increased
-Fibrinogen: decreased

Back 23

DIC

Front 24

The severity of DIC is reflected on what? Clinicians order them daily to see trend of schistocytes (fragmented RBC)

Back 24

Blood smear

Front 25

To treat DIC you need to diagnose and treat the underlying cause of the DIC, and support the patient with:
fresh frozen plasma (FFP) - contains ________ factors
cryoprecipitate - contains ________
platelets
RBCs

Back 25

coagulation
fibrinogen

Front 26

Idiopathic Thrombocytopenic Purpura (ITP)
Anti________ antibodies
Acute and chronic types
Diagnosis of exclusion (rule out all others first)
Steroids or __________

Back 26

platelet
splenectomy (normalizes the platelet count)

Front 27

ITP causes autoantibodies to GP ____ or __. These bind to platelets and cause them to be eaten by macrophages in the _______.

Back 27

IIb-IIIa or Ib
spleen

Front 28

ITP has chronic and acute types:
Children, abruptly following viral illness =
Adult women, primary or secondary, insidious, danger of bleeding into the brain =

Back 28

acute ITP
chronic ITP

Front 29

These tests indicate what condition?
Signs of platelet destruction:
-thrombocytopenia
-normal/increased
-megakaryocytes
-big platelets
-INR/PTT normal
-No specific diagnostic test for ITP

Back 29

Idiopathic Thrombocytopenic Purpura

Front 30

Other causes of thrombocytopenia besides ITP:
1. _____ effect
2. Aplastic anemia
3. Bone marrow replacement (e.g., by tumor)
4. Big ______
5. Non-immune-related platelet destruction (e.g., DIC, TTP, HUS)

Back 30

drug
spleen

Front 31

Treatment for ITP includes:
Glucocorticoids
Splenectomy
IV _______

Back 31

immunoglobulin

Front 32

How is thrombotic microangiopathies different from DIC?

Back 32

NOT coagulation system screwed up, usually just the platelets

Front 33

Thrombotic Thrombocytopenic Purpura
-Pentad: MAHA, thrombocytopenia, fever, neurologic defects, renal failure
-Deficiency of enzyme _______
-Big ___ multimers trap platelets
-Plasmapheresis or plasma infusions treatment

Back 33

ADAMTS13
vWF

ADAMTS13 cleaves UL vWF into less active bits!

Front 34

What condition is this?
- MAHA and thrombocytopenia
- Epidemic (E. coli) vs. non-epidemic
- Toxin (or ?) damages endothelium
- Treat supportively

Back 34

Hemolytic Uremic Syndrome

Front 35

Epidemic HUS is caused by ____ O157:H7, makes toxin, injures ______ cells, children/elderly, bloody diarrhea -> ____ failure, fatal in 5% cases

Back 35

E. Coli
Endothelial
Renal

Front 36

Non-epidemic HUS is a defect in complement factor _, inherited or acquired, ____ failure, relapsing-remitting course, fatal 50%

Back 36

H
Renal

Front 37

In DIC, the INR and PTT are _______ . In TTP and HUS they are _______!

Back 37

prolonged, normal

Front 38

Treat Hemolytic Uremic Syndrome with supportive care (not much) and dialysis if needed, but NOT ________

Back 38

antibiotics - they may increase toxin release

Front 39

Bleeding caused by deficiency or abnormality of vit K dependent factors caused by a drug: ________, poor diet, malabsorption, _______ disease of the newborn

Back 39

coumadin
hemorrhagic

Front 40

What do these all have in common: II, VII, IX, X, and proteins C and S

Back 40

Vitamin K dependent factors

Shortest half-life = VII

Front 41

Bleeding associated with liver failure:
_____ obstruction causing impaired absorption of vitamin K
Hepatocytes make less ________ factors
Portal hypertension causes a large _____
Decreased thrombopoietin production by liver contributes to low _____ count

Back 41

Biliary
coagulation
spleen (decreases platelet count)
platelet

Front 42

What factor normally binds to vWf?

Back 42

VIII

Front 43

MOST of the time, what 4 things cause DIC?

Back 43

Malignancies (adenocarcinoma)
Ob complications
Sepsis
Trauma