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International Practices in Managing Preconception, Pregnancy and Childbirth in Women With Glanzmann Thrombasthenia: A Survey From the European Association of Haemophilia and Allied Disorders (EAHAD). [PDF]

Haemophilia
ABSTRACT Background Glanzmann thrombasthenia (GT) is an inherited platelet disorder resulting in severely reduced platelet aggregation and increased bleeding tendency. Pregnancy and childbirth in women with GT present significant challenges for both mother and child.
Rutten KHG, Schutgens REG, d'Oiron R, Fiore M, Castaman G, Gomez K, Mathias M, Lavin M, Elfvinge P, Kadir RA, van Galen KPM, Glanzmann Thrombasthenia and Women and Girls with Bleeding Disorders Working Groups of the EAHAD.   +11 more
europepmc   +2 more sources

The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes of non-surgical bleeding episodes in patients with Glanzmann thrombasthenia

Haematologica, 2015
Standard treatment for Glanzmann thrombasthenia is platelet transfusion. Recombinant activated factor VII has been shown to be successful in patients with Glanzmann thrombasthenia with platelet antibodies or who are refractory to platelet transfusions ...
Giovanni Di Minno, Rainer B Zotz, Roseline d’Oiron, Niels Bindslev, Matteo Nicola Dario Di Minno, Man-Chiu Poon   +5 more
doaj   +1 more source

The international, prospective Glanzmann Thrombasthenia Registry: treatment and outcomes in surgical intervention

Haematologica, 2015
Standard treatment for Glanzmann thrombasthenia, a severe inherited bleeding disorder, is platelet transfusion. Recombinant factor VIIa is reported to be effective in Glanzmann thrombasthenia with platelet antibodies and/or refractoriness to platelet ...
Man-Chiu Poon, Roseline d’Oiron, Rainer B. Zotz, Niels Bindslev, Matteo Nicola Dario Di Minno, Giovanni Di Minno   +5 more
doaj   +1 more source

Association of autosomal‐recessive‐type distal renal tubular acidosis and Glanzmann thrombasthenia as a consequence of runs of homozygosity

Clinical Case Reports, 2022
We report the case of a Filipino girl with autosomal‐recessive‐type distal renal tubular acidosis and Glanzmann thrombasthenia caused by homozygous variants in the genes SLC4A1 and ITGA2B within the long homozygous DNA region on chromosome 17q21.31. This
Keiko Aso, Takehiko Soutome, Mari Satoh, Takako Aoki, Hiromi Ogura, Toshiyuki Yamamoto, Hitoshi Kanno, Hiroyuki Takahashi   +7 more
doaj   +1 more source

Glanzmann thrombasthenia

Orphanet Journal of Rare Diseases, 2006
Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet aggregation.
Nurden Alan T
doaj   +1 more source

Management of Glanzmann's Thrombasthenia – Guidelines based on an expert panel consensus from gulf cooperation council countries

Journal of Applied Hematology, 2019
Glanzmann thrombasthenia is a rare disorder, due to quantitative and/or qualitative abnormalities of the platelet integrin αIIbβ3 and/or αIIbβ3. Although it is considered a rare disorder with a global incidence of 1/1,000,000 population, the case is ...
Ahmad Tarawah, Tarek Owaidah, Naima Al-Mulla, Muhammad Faisal Khanani, Jehan Elhazmi, Muneer Albagshi, Yaser Wali, Shikah AlMohareb, Abdulkareem Almomen   +8 more
doaj   +1 more source

Glanzmann thrombasthenia complicated by frequent myeloproliferative neoplasm‐related thromboembolism: thrombosis occurring regardless of αIIbβIII integrin deficiency

Clinical Case Reports, 2021
A patient with Glanzmann Thrombasthenia developed recurrent venous thrombosis with a JAK2 positive myeloproliferative neoplasm. This indicates that the platelet aIIbβIII integrin has no role in venous thrombosis. We discuss the other potential mechanisms
Roger E. G. Schutgens, Rolf T. Urbanus
doaj   +1 more source

Trombastenia de Glanzmann. Histerectomia vaginal de urgência tratada com factor VIIa recombinante.

Acta Médica Portuguesa, 2004
In the present article, regarding a clinical case of Thrombasthenia of Glanzmann with severe anaemia by menometrorrhagia, the authors propose the vaginal hysterectomy and the administration of the recombinant factor VIIa on the immediate pre and ...
João S Mairos, Ana C Bernardo, Anabela R Neves, Ana P Campos, Filomena P Nunes, João S Saraiva, Maria C Trinidade, Alcides H Pereira, Ana Marques Pereira, Carlos B Falcão, Hélio Retto   +10 more
doaj   +1 more source

Leukocyte adhesion defects Type III: A rare association of primary immunodeficiency and platelet functional defect

Journal of Applied Hematology, 2022
Leukocyte adhesion defect (LAD) Type III is an extremely rare disease, which presents with severe infections and a Glanzmann thrombasthenia-like bleeding phenotype.
Anand Prakash
doaj   +1 more source

A rare case report on Glanzmann thrombasthenia

National Journal of Physiology, Pharmacy and Pharmacology, 2017
Glanzmann thrombasthenia (GT) is a rare inherited blood clotting disorder characterized by the impaired function of platelets that are essential for proper blood clotting and can lead to prolonged bleeding time. Patient with GT may experience menorrhagia,
Somy Cherian, Preenumol Thomas, Roshni PR   +2 more
doaj   +1 more source