thrombasthenia


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thrombasthenia

 [throm″bas-the´ne-ah]
1. decreased platelet function; called also thromboasthenia.
Glanzmann thrombasthenia a hereditary platelet abnormality characterized by defective clot retraction, prolonged bleeding time, and related symptoms such as epistaxis and inappropriate bleeding. Clinically there is abnormal glass adhesion and impaired aggregation to ADP, collagen, and thrombin. Called also thrombasthenia and Glanzmann's disease.

throm·bas·the·ni·a

(throm'bas-thē'nē-ă),
An abnormality of platelets characteristic of Glanzmann thrombasthenia.
See also: Bernard-Soulier syndrome.
Synonym(s): thromboasthenia
[thromb- + G. astheneia, weakness]

thrombasthenia

/throm·bas·the·nia/ (throm″bas-the´ne-ah) a platelet abnormality characterized by defective clot retraction and impaired ADP-induced platelet aggregation; clinically manifested by epistaxis, inappropriate bruising, and excessive posttraumatic bleeding.
Glanzmann thrombasthenia  thrombasthenia.

thrombasthenia

[throm′basthē′nē·ə]
Etymology: Gk, thrombos, lump, a + sthenos, not strength
decreased platelet function. See Glanzmann thrombasthenia.

thrombasthenia

Glanzmann's disease, see there.

throm·bas·the·ni·a

(throm'bas-thē'nē-ă)
An abnormality of platelets; to date, several varieties have been categorized; often finding characteristics of Glanzmann thrombasthenia.
Synonym(s): thrombo-aesthenia.
[thromb- + G. astheneia, weakness]

thrombasthenia

An obsolescent term for the condition in which blood platelets are present in normal numbers but do not function normally in their role in blood clotting. The condition may be inherited but is more commonly acquired. With the rapid growth in understanding of platelet function this term is likely to disappear.

throm·bas·the·ni·a

(throm'bas-thē'nē-ă)
Abnormality of platelets.
Synonym(s): thromboasthenia.
[thromb- + G. astheneia, weakness]

thrombasthenia (throm´basthē´nēə),

n a hemorrhagic diathesis associated with qualitative abnormalities of the platelets.

thrombasthenia

a platelet abnormality characterized by defective clot retraction and impaired ADP-induced platelet aggregation. There are mild bleeding tendencies. Inherited as an autosomal recessive disorder in Otterhounds. Called also Glanzmann's disease, Glanzmann-Naegeli syndrome.
References in periodicals archive ?
Glanzmann Thrombasthenia is a genetic platelet disorder in which platelet glycoprotein [alpha]IIb/[beta]3 the major integrin complex (fibrinogen receptor) is either deficient or present but dysfunctional.
Criteria for diagnosis of Glanzmann's thrombasthenia (GT) were normal PT, aPTT, platelet count, and morphology; no curve with ADP, epinephrine, or collagen; and normal curve with ristocetin in platelet aggregation by lumiaggregometry.
Nurden AT Didry D, Kieffer N, McEver RP Residual amounts of glycoproteins IIb and IIIa may be present in the platelets of most patients with Glanzmann's thrombasthenia.
For example, Glanzmann thrombasthenia is a deficiency of the IIb and/or IIIa subunits of the GP IIb/IIIa receptor.
Mr Justice Astill said although Mrs Wood was suffering from a rare genetic blood disorder known as Glanzmann's Thrombasthenia, the hospital should have done more to prevent her death.
EPISTAXIS CASES ENCOUNTERED IN THE SHEBA MEDICAL CENTER FROM 1983 TO 1986 Number Associated Problem of cases Hypertension 19 Leukemia 4 Postrhinoseptoplasty 3 Nasal trauma 2 Hereditary hemorrhagic telangiectasia 2 Anticoagulant treatment 3 Deficiency in coagulation factors 2 Idiopathic thrombocytopenic purpura 2 Thrombotic thrombocytopenic purpura 1 Thrombasthenia 1 Chronic renal failure 2 Hepatic cirrhosis 1 Hepatic tumor 1 Infectious hepatitis 1 Idiopathic 30 Total 74
Intensive and Successful Transfusion of Pathogen Inactivated INTERCEPT Platelet Concentrates for Major Gynecological and Obstetrical Surgery in Glanzmann Thrombasthenia Type 1 with Gypsy Mutation documented the ability of INTERCEPT-treated platelets to support two patients with a recessive autosomal severe bleeding disorder during surgical procedures.
Glanzmann thrombasthenia with bleeding or as prophylaxis before surgery Therapeutic Bleeds from oral cavity, mucous membrane or any other site with platelet dysfunction irrespective of the platelet count
Hemarthrosis is rarely seen in disorders of platelet function and occurs even more rarely in Glanzmann thrombasthenia (GT), whereas episodes of hemarthrosis can be frequent in hemophilia (1).
Bernard-Soulier's syndrome, Glanzmann's thrombasthenia and storage pool disease has become easy through flow cytometry.
Glanzmann thrombasthenia is the diagnosis which should be considered primarily in patients with normal platelet count and unusual cutaneous and mucosal bleedings starting from birth and early childhood.
The CBC in individuals with Glanzmann's thrombasthenia is usually normal, with an abnormal PFA-100.