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A YOUNG WOMEN WITH LIGNEOUS MEMBRANES WHO WANTS TO BE PREGNANT
Author(s): ,
Ayse Gonca Kacar
Affiliations:
Pediatric Hematology and Oncology,Cerrahpasa Medical Faculty,Istanbul,Turkey
,
Seyma Fenercioglu
Affiliations:
Gynecology and Obstetrics,Istanbul Fertility Center,Istanbul,Turkey
Tulin Tiraje Celkan
Affiliations:
Pediatric Hematology and Oncology,Cerrahpasa Medical Faculty,Istanbul,Turkey
EHA Library. Gonca Kacar A. 06/09/21; 324503; PB1832
Ayse Gonca Kacar
Ayse Gonca Kacar
Contributions
Abstract

Abstract: PB1832

Type: Publication Only

Session title: Thrombosis and vascular biology - Biology & Translational Research

Background
Plasminogen deficiency is a rare condition with autosomal recessive inheritance. Severe hypoplasminogenemia is associated with ‘ligneous’ lesions on each mucous membranes  especialy eye, ear, mouth, pharynx, respiratory and female genital tract; cervix and endometrium. Furthermore, plasminogen deficient women complained from infertility.  These kind of  patient's diagnosis remained elusive for  years. 

Aims

We want to share this young women in order to take attention and form a greater familiarity with the clinical and histopathologic features of this condition among gynecologists.

Methods

Data from the files of a 32 year -old nulliparous young woman with  intractable gingivitis and dental problems and a  history of persistent conjuctivitis is documented. 

Results

She was refered to a gynaecologist due to infertility and cervicitis. Colposcopic and ultrasound examination of the uterus revealed, woody membraneous lesion which  cover the inner site of the uterine cavity. We decided to give fresh frozen plasma (FFP) infusions both IV and locally to the uterine cavity after diagnosis of plasminogen deficiency.  Heparine added FFP ( 50ml) also 5mg recombinant plazminogen activator ( rt-PA, ActilyseR)remained  in the cavity for 2-4 hours. Although a regular cavity  view was achieved after FFP replacement therapy and local application, freeze embrional transfer was performed without  a proper pregnancy achievement.

Conclusion

Gynecologists and clinicians who are familiar with this bizarre disease’s clinical and histopathologic features will  diagnose  timely and manage properly. 

Keyword(s): Fresh frozen plasma, Heparin, Plasminogen, TPA

Abstract: PB1832

Type: Publication Only

Session title: Thrombosis and vascular biology - Biology & Translational Research

Background
Plasminogen deficiency is a rare condition with autosomal recessive inheritance. Severe hypoplasminogenemia is associated with ‘ligneous’ lesions on each mucous membranes  especialy eye, ear, mouth, pharynx, respiratory and female genital tract; cervix and endometrium. Furthermore, plasminogen deficient women complained from infertility.  These kind of  patient's diagnosis remained elusive for  years. 

Aims

We want to share this young women in order to take attention and form a greater familiarity with the clinical and histopathologic features of this condition among gynecologists.

Methods

Data from the files of a 32 year -old nulliparous young woman with  intractable gingivitis and dental problems and a  history of persistent conjuctivitis is documented. 

Results

She was refered to a gynaecologist due to infertility and cervicitis. Colposcopic and ultrasound examination of the uterus revealed, woody membraneous lesion which  cover the inner site of the uterine cavity. We decided to give fresh frozen plasma (FFP) infusions both IV and locally to the uterine cavity after diagnosis of plasminogen deficiency.  Heparine added FFP ( 50ml) also 5mg recombinant plazminogen activator ( rt-PA, ActilyseR)remained  in the cavity for 2-4 hours. Although a regular cavity  view was achieved after FFP replacement therapy and local application, freeze embrional transfer was performed without  a proper pregnancy achievement.

Conclusion

Gynecologists and clinicians who are familiar with this bizarre disease’s clinical and histopathologic features will  diagnose  timely and manage properly. 

Keyword(s): Fresh frozen plasma, Heparin, Plasminogen, TPA

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