Bleeding diathesis

Bleeding diathesis
Classification and external resources
Specialty	hematology
ICD-10	D69.9
ICD-9-CM	287.9
DiseasesDB	1442
MeSH	D006474

In medicine (hematology), bleeding diathesis (or bleeding tendency or predisposition or h(a)emorrhagic diathesis) is an unusual susceptibility to bleed (hemorrhage) mostly due to hypocoagulability, in turn caused by a coagulopathy (a defect in the system of coagulation). Several types are distinguished, ranging from mild to lethal. Also, bleeding diathesis can be caused by thinning of the skin or impaired wound healing.

Hypocoagulability

Hypocoagulability is a bleeding diathesis caused by coagulopathy.

Causes

While there are several possible causes, they generally result in excessive bleeding and a lack of clotting.

Acquired

Acquired causes of coagulopathy include anticoagulation with warfarin, liver failure, Vitamin K deficiency and disseminated intravascular coagulation.

Additionally, the haemotoxic venom from certain species of snakes can cause this condition, for example Bothrops, rattlesnakes and other species of viper. Viral hemorrhagic fevers include dengue hemorrhagic fever and Dengue Shock Syndrome

Leukemia may also cause coagulopathy. Furthermore, cystic fibrosis has been known to cause bleeding diathesis, especially in undiagnosed infants, due to malabsorption of fat soluble vitamins like Vitamin K.

Autoimmune causes of acquired coagulation disorders

There are autoimmune causes of coagulation disorders. They include acquired antibodies to coagulation factors, termed inhibitors of coagulation. The main inhibitor is directed against clotting Factor VIII. Another example is antiphospholipid syndrome an autoimmune, hypercoagulable state.

Genetic

Some people lack genes that typically produce the protein coagulation factors that allow normal clotting. Various types of Hemophilia and von Willebrand disease are the major genetic disorders associated with coagulopathy.

Rare examples are Bernard-Soulier syndrome, Wiskott-Aldrich syndrome and Glanzmann's thrombasthenia.

Symptoms

Symptom	Disorders
Petechiae (red spots)	Wiskott-Aldrich syndrome, where they may resemble a few bruises^[1] Acute leukemia^[2] Chronic leukemia^[2] Vitamin K deficiency^[3]
Purpura and ecchymoses	Acute leukemia^[2] Chronic leukemia^[2] Vitamin K deficiency^[3]
Blood in stool	Wiskott-Aldrich syndrome, especially in infancy^[1] Acute leukemia^[2]
Bleeding gingiva (gums)	Wiskott-Aldrich syndrome^[1] Acute leukemia^[2] Chronic leukemia^[2]
Prolonged nose bleeds	Wiskott-Aldrich syndrome^[1]

Complications

Following are some complications of coagulopathies, some of them caused by their treatments:

Complication	Disorders
Soft tissue bleeding, e.g. deep-muscle bleeding, leading to swelling, numbness or pain of a limb.	Hemophilia^[4] Von Willebrand disease ^[5]
Joint damage, potentially with severe pain and even destruction of the joint and development of arthritis	Hemophilia^[4] Von Willebrand disease ^[5]
Retinal bleeding	Acute leukemia^[2]
Transfusion transmitted infection, from blood transfusions that are given as treatment.	Hemophilia^[4]
Adverse reactions to clotting factor treatment.	Hemophilia^[4]
Anemia	Von Willebrand disease ^[5]
Exsanguination (bleeding to death)	Von Willebrand disease ^[5] Acute leukemia^[2] Vitamin K deficiency^[3]
Cerebral hemorrhage	Wiskott-Aldrich syndrome^[1]

Comparing coagulation tests

Laboratory findings in various platelet and coagulation disorders (V - T)
Condition	Prothrombin time	Partial thromboplastin time	Bleeding time	Platelet count
Vitamin K deficiency or warfarin	Prolonged	Normal or mildly prolonged	Unaffected	Unaffected
Disseminated intravascular coagulation	Prolonged	Prolonged	Prolonged	Decreased
Von Willebrand disease	Unaffected	Prolonged or unaffected	Prolonged	Unaffected
Haemophilia	Unaffected	Prolonged	Unaffected	Unaffected
Aspirin	Unaffected	Unaffected	Prolonged	Unaffected
Thrombocytopenia	Unaffected	Unaffected	Prolonged	Decreased
Liver failure, early	Prolonged	Unaffected	Unaffected	Unaffected
Liver failure, end-stage	Prolonged	Prolonged	Prolonged	Decreased
Uremia	Unaffected	Unaffected	Prolonged	Unaffected
Congenital afibrinogenemia	Prolonged	Prolonged	Prolonged	Unaffected
Factor V deficiency	Prolonged	Prolonged	Unaffected	Unaffected
Factor X deficiency as seen in amyloid purpura	Prolonged	Prolonged	Unaffected	Unaffected
Glanzmann's thrombasthenia	Unaffected	Unaffected	Prolonged	Unaffected
Bernard-Soulier syndrome	Unaffected	Unaffected	Prolonged	Decreased or unaffected
Factor XII deficiency	Unaffected	Prolonged	Unaffected	Unaffected
C1INH deficiency	Unaffected	Shortened	Unaffected	Unaffected

Causes other than coagulation

Bleeding diathesis may also be caused by impaired wound healing (as in scurvy), or by thinning of the skin, such as in Cushing's syndrome .

References

1 2 3 4 5 Wiskott-Aldrich Syndrome The International Patient Organisation for Primary Immunodeficiencies (IPOPI).
1 2 3 4 5 6 7 8 9 Disorders of thrombosis and hemostasis: clinical and laboratory practice. Page Rodger L. Bick. Edition 3, illustrated. ISBN 0-397-51690-8, ISBN 978-0-397-51690-2. 446 pages
1 2 3 Vitamin K Deficiency eMedicine. Author: Pankaj Patel, MD. Coauthor(s): Mageda Mikhail, MD, Assistant Professor. Updated: Dec 18, 2008
1 2 3 4 Hemophilia Complications By Mayo Clinic staff. May 16, 2009
1 2 3 4 Von Willebrand disease --> Complications By Mayo Clinic staff. Feb. 7, 2009

Diseases of clotting (D50–69,74, 280–287)

Coagulation/
coagulopathy

↑

Hyper- coagulability	primary: Antithrombin III deficiency Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden Prothrombin G20210A Sticky platelet syndrome acquired:Thrombocytosis essential DIC Purpura fulminans autoimmune Antiphospholipid

↓

Hypo-
coagulability

Thrombocytopenia	Thrombocytopenic purpura: ITP Evans syndrome TM TTP Upshaw Schulman syndrome Heparin-induced thrombocytopenia May–Hegglin anomaly

Platelet function	adhesion Bernard–Soulier syndrome aggregation Glanzmann's thrombasthenia platelet storage pool deficiency Hermansky–Pudlak syndrome Gray platelet syndrome

Clotting factor	Hemophilia A/VIII B/IX C/XI von Willebrand disease Hypoprothrombinemia/II XIII Dysfibrinogenemia Congenital afibrinogenemia

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