関: bleeding tendency、hemorrhagic diathesis

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constitutional predisposition to a particular disease or abnormality

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/05/14 18:11:18」(JST)

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In medicine (hematology), bleeding diathesis (or bleeding tendency or predisposition or h(a)emorrhagic diathesis) is an unusual susceptibility to bleeding (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.

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.

Generally, if one parent has a type of Bleeding Diathesis, there is a 95% chance of passing it onto their offspring, since most genetically acquired coagulopathies are sex-linked, X chromosome disorders.

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
Hemophilia	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

^ ^a ^b ^c ^d ^e Wiskott-Aldrich Syndrome The International Patient Organisation for Primary Immunodeficiencies (IPOPI).
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ 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
^ ^a ^b ^c Vitamin K Deficiency eMedicine. Author: Pankaj Patel, MD. Coauthor(s): Mageda Mikhail, MD, Assistant Professor. Updated: Dec 18, 2008
^ ^a ^b ^c ^d Hemophilia Complications By Mayo Clinic staff. May 16, 2009
^ ^a ^b ^c ^d Von Willebrand disease --> Complications By Mayo Clinic staff. Feb. 7, 2009

UpToDate Contents

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1. 出血性素因を有する成人患者へのアプローチ approach to the adult patient with a bleeding diathesis
2. 出血症状を有する小児に対するアプローチ approach to the child with bleeding symptoms
3. 不正子宮出血がみられた閉経前の女性に対する初期アプローチ initial approach to the premenopausal woman with abnormal uterine bleeding
4. 急性腎障害（急性腎不全）のマネージメントの概要 overview of the management of acute kidney injury acute renal failure
5. 女性における生殖器出血の原因の概要 overview of causes of genital tract bleeding in women

English Journal

Altered resting state effective connectivity in long-standing vegetative state patients: An EEG study.

Varotto G1, Fazio P2, Rossi Sebastiano D1, Duran D1, D'Incerti L3, Parati E4, Sattin D5, Leonardi M5, Franceschetti S1, Panzica F6.Author information 1Dept. of Neurophysiology and Diagnostic Epileptology, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy.2Dept. of Neurophysiology and Diagnostic Epileptology, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy; Dept. of Medical and Surgical Sciences of Communication and Behaviour, Neurology Section, University of Ferrara, Italy.3Dept. of Neuroradiology, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy.4Dept. of Cerebrovascular Diseases, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy.5Unit of Neurology, Public Health, Disability Unit, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy; Coma Research Centre, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy.6Dept. of Neurophysiology and Diagnostic Epileptology, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy. Electronic address: panzica@istituto-besta.it.AbstractOBJECTIVE: Recent evidence mainly based on hemodynamic measures suggests that the impairment of functional connections between different brain areas may help to clarify the neuronal dysfunction occurring in patients with disorders of consciousness (DOC). The aim of this study was to evaluate effective EEG connectivity in a cohort of 18 patients in a chronic vegetative state (VS) observed years after the occurrence of hypoxic (eight) and traumatic or hemorrhagic brain insult.
Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology.Clin Neurophysiol.2014 Jan;125(1):63-8. doi: 10.1016/j.clinph.2013.06.016. Epub 2013 Aug 6.
OBJECTIVE: Recent evidence mainly based on hemodynamic measures suggests that the impairment of functional connections between different brain areas may help to clarify the neuronal dysfunction occurring in patients with disorders of consciousness (DOC). The aim of this study was to evaluate effecti
PMID 23927942

Diagnosis and treatment of cerebral venous and sinus thrombosis.

Weimar C.Author information Department of Neurology and Stroke Center, University of Duisburg-Essen, Hufelandstr. 55, 45122, Essen, Germany, christian.weimar@uk-essen.de.AbstractCerebral venous and sinus thrombosis is a still underdiagnosed cause of stroke, with an incidence of about 2.8 events per 100,000 person-years in young women and about 1.3 events per 100,000 person-years in the general population. Puerperium, oral hormonal contraception, and coagulation disorders remain the most frequently identified risk factors. Initial treatment with heparin is the only proven therapy, although the evidence is based on only two randomized placebo-controlled trials which together included 79 patients. In the case of clinical deterioration under anticoagulation, local thrombolysis and mechanical thrombectomy may be considered, but clinical efficacy is supported only by case reports. Patients with imminent lateral herniation due to large hemorrhagic infarctions should be treated with prompt surgical decompression. Following the acute phase, oral anticoagulation is recommended for 3-12 months, and only patients suffering from a severe coagulopathy or with recurrent cerebral venous and sinus thrombosis should be considered for long-term anticoagulation. Only insufficient experience is available for novel anticoagulants such as thrombin inhibitors or factor Xa antagonists.
Current neurology and neuroscience reports.Curr Neurol Neurosci Rep.2014 Jan;14(1):417. doi: 10.1007/s11910-013-0417-5.
Cerebral venous and sinus thrombosis is a still underdiagnosed cause of stroke, with an incidence of about 2.8 events per 100,000 person-years in young women and about 1.3 events per 100,000 person-years in the general population. Puerperium, oral hormonal contraception, and coagulation disorders re
PMID 24264471

Use of thromboelastography and thrombin generation assay to predict clinical phenotype in patients with severe FVII deficiency.

Tran HT, Tjønnfjord GE, Holme PA.Author information Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway; Department of Haematology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.AbstractBleeding tendency is weakly correlated with the activity of factor VII (FVII) in the plasma of patients with FVII deficiency. A laboratory method for predicting bleeding risk in patients with this coagulation disorder is lacking. We investigated whether global coagulation assays, specifically thromboelastography (TEG) and thrombin generation assay (TGA), could be used to predict bleeding risk. We also sought to identify factors that may explain the differences in bleeding phenotype observed among individuals with severe FVII deficiency. The study comprised 12 patients with severe FVII deficiency (FVII activity <1%). Eleven patients were homozygous for the Gln100Arg mutation and one patient was compound heterozygous. Clinically, 10 patients had increased haemorrhagic diathesis, whereas two patients were asymptomatic. Blood sampling was performed at baseline for TEG and TGA analyses. The platelet aggregation assay was performed and the plasma level of anticoagulation inhibitors and thrombophilic risk factors assessed. No difference in the TEG and TGA results was observed in all FVII-deficient individuals. The level of free tissue factor pathway inhibitor was within the normal range and similar in symptomatic and asymptomatic subjects. None of the participants had the FV Leiden mutation, prothrombin gene mutation, or abnormal anticoagulant inhibitor levels. Asymptomatic subjects showed normal platelet aggregation. These data suggested that TEG and TGA were not suitable methods for predicting the clinical phenotype in FVII-deficient subjects.
Haemophilia : the official journal of the World Federation of Hemophilia.Haemophilia.2014 Jan;20(1):141-6. doi: 10.1111/hae.12256. Epub 2013 Aug 28.
Bleeding tendency is weakly correlated with the activity of factor VII (FVII) in the plasma of patients with FVII deficiency. A laboratory method for predicting bleeding risk in patients with this coagulation disorder is lacking. We investigated whether global coagulation assays, specifically thromb
PMID 23992369

Japanese Journal

Prostatic carcinoma presenting as a haemorrhagic diathesis after dental extraction

Br Dent J 166, 295-296, 1989
NAID 10012587252

肝疾患におけるカリクレイン‐キニン系の変動について

血液と脈管 15(2), 128-134, 1984
NAID 130004260937

血小板容積に関する臨床的検討

血液と脈管 10(1), 140-144, 1979
NAID 130004092350

Related Pictures

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関連記事	「diathesis」「haemorrhagic」

「diathesis」

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

　　[★]

n.

(ある病気にかかりやすい)素質、体質、素因

The combination of dipyridamole and warfarin can be used to inhibit thrombus formation on prosthetic heart valves, while the combination of dipyridamole and aspirin can be used to reduce the likelihood of thrombosis in patients with a thrombotic diathesis(PPC.401)

関: diathetic, disease susceptibility, ground, predisposition

「haemorrhagic」

　　[★]

adj.

出血性の

関: haemophilic、hemophilic、hemorrhagic

[was-1] Wiskott-Aldrich Syndrome The International Patient Organisation for Primary Immunodeficiencies (IPOPI).

[bick-2] ^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ 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

[emedicine-3] Vitamin K Deficiency eMedicine. Author: Pankaj Patel, MD. Coauthor(s): Mageda Mikhail, MD, Assistant Professor. Updated: Dec 18, 2008

[mayo-hemophilia-4] Hemophilia Complications By Mayo Clinic staff. May 16, 2009

[mayo-vwd-5] Von Willebrand disease --> Complications By Mayo Clinic staff. Feb. 7, 2009

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案内

haemorrhagic diathesis