Bleeding Disorders

Question 1

what are bleeding disorders?

Answer 1

conditions in which the blood doesn’t clot properly, making it hard for a person to stop bleeding

Normally, platelets and clotting factors work together to stop bleeding; platelets form a soft plug and fibrin anchors it. But if there is a shortage of platelets, or if there are abnormalities in the clotting system, clotting can be impaired and excessive bleeding can occur

Question 2

what are the two causes of bleeding disorders?

Answer 2

1. lack of platelets = thrombocytopenia (primary hemostasis)

2. abnormalities in the clotting system (secondary hemostasis)

Question 3

which bleeding disorders result from a lack of platelets?

Answer 3

1. Idiopathic thrombocytopenic purpura (ITP)
2. thrombotic thrombocytopenic purpura (TTP)
3. hemolytic uremic syndrome (HUS)

Question 4

which bleeding disorders result from problems with the clotting system?

Answer 4

1. Coagulation disorders; like hemophilia
2. Platelet disorders; like Bernard-Soulier syndrome

• Disorders that involve both coagulation and platelets; like von Willebrand
disease and disseminated intravascular coagulation

Question 5

what are some types of coagulation disorders?

Answer 5

hemophilia

Question 6

what is a type of platelet disorder?

Answer 6

Bernard-Soulier syndrome

Question 7

what disorders involve both coagulation and platelets?

Answer 7

1. von Willebrand
   disease
2. disseminated intravascular coagulation

Question 8

how do you get a bleeding disorder?

Answer 8

inherited or acquired

Question 9

why do people get petechiae?

Answer 9

spontaneous skin and mucous membrane bleeding

normally, the platelets lining the vessel walls provide physical protection against blood seepage between endothelial cells

if there are very few platelets around, or if the platelets are there but are not functioning properly, there is less protection, and blood seeps from capillaries, forming little red dots (or petechiae) on the skin

seen in patients with thrombocytopenia and patients with platelet disorders (causing intrinsic platelet abnormalities)

Question 10

what is mucous membrane bleeding?

Answer 10

seen in platelet bleeding

includes things like nosebleeds, heavy periods, or oral/gingival bleeding

Question 11

what is factor bleeding?

Answer 11

patients with coagulation disorders tend to bleed after a traumatic incident – but not right away

they can form a platelet plug just fine, so right after an injury they are able to achieve temporary hemostasis. But they are not able to make fibrin well, so the platelet plug isn’t sealed up, and it just washes away after some time

Question 12

what are the types of bleeding that can happen if you have a bleeding disorder?

Answer 12

1. platelet bleeding

bleeding is due to platelet abnormalities (problems with primary hemostasis)

2. factor bleeding

factor abnormalities (problems with secondary hemostasis)

Question 13

what types of diseases are factor bleeding?

Answer 13

• severe factor deficiencies like severe hemophilia
• hemarthrosis = bleeding into joints
• hematoma = bleeding into deep soft tissue

Question 14

How do patients with platelet problems present, compared to patients with coagulation problems?

Answer 14

Patients with platelet problems tend to have superficial, spontaneous bleeding, whereas patients with coagulation problems tend to have delayed, deep bleeding related to trauma.

Question 15

which bleeding disorders are hereditary?

Answer 15

1. von Willebrand disease
2. Hemophilia A
3. Hemophilia B
4. hereditary platelet disorders

Question 16

which bleeding disorders are acquired?

Answer 16

1. disseminated intravascular coagulation (DIC)
2. Idiopathic thrombocytopenic purpura (ITP)
3. Thrombotic microangiopathies: thrombotic thrombocytopenic purpura (TTP)
4. hemolytic-uremic syndrome (HUS)

Question 17

what is von Willebrand disease?

Answer 17

caused by a decreased amount of functional von Willebrand factor (that protein that helps platelets bind to the subendothelium)

most common hereditary bleeding disorder

occurs in about 1 in 100 people, many of whom are totally asymptomatic

Question 18

what is the most common hereditary bleeding disorder?

Answer 18

von Willebrand disease

Question 19

what is Hemophilia A?

Answer 19

X-linked disorder in which patients are deficient in factor VIII

most common factor deficiency - NOT the most common hereditary bleeding disorder!

Question 20

what is the most common factor deficiency?

Answer 20

Hemophilia A

Question 21

what is Hemophilia B?

Answer 21

x-linked deficiency in factor IX

more rare than hemophilia A

Question 22

what are hereditary platelet disorders?

Answer 22

the platelets are missing some important structure, like glycoprotein IIb/IIIa, resulting in loss of platelet function

ex. Bernard-Soulier syndrome

Question 23

what is disseminated intravascular coagulation

Answer 23

sometimes called “Death is Coming” because it can be such a dangerous problem

the whole thing starts when something kicks off the coagulation cascade causing rampant, widespread clot formation

then, as platelets and coagulation factors are depleted, bleeding becomes a problem

Question 24

what is

Idiopathic thrombocytopenic purpura?

Answer 24

patients make antibodies against their platelets for some unknown (idiopathic) reason, marking them for destruction in the spleen

eventually, the platelet count drops so low that patients begin to get symptoms, such as purpura or easy bruising

Question 25

what is thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS)?

Answer 25

these disorders are characterized by widespread platelet aggregation

coagulation system isn’t activated, though, so these disorders are different from DIC

Question 26

Why are hemophilia A and hemophilia B seen almost exclusively in males?

Answer 26

They are both X-linked disorders

Question 27

Which symptom is most consistent with a coagulation factor deficiency?

Answer 27

Bleeding into joints

Question 28

A 23-year-old female presents to clinic with excessive menstrual bleeding and easy bruising. Which disorders is the most likely cause of her symptoms?

Answer 28

von Willebrand Disease