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LUPUS ANTICOAGULANT - HYPOPROTHROMBINEMIA SYNDROME IN A 7-YEAR OLD GIRL: A CASE REPORT
Author(s): ,
Turkan Patiroglu
Affiliations:
Pediatric Hematology,Erciyes University Medical Faculty,Kayseri,Turkey
,
Ebru Y?lmaz
Affiliations:
Pediatric Hematology,Erciyes University Medical Faculty,Kayseri,Turkey
,
Ekrem Unal
Affiliations:
Pediatric Hematology,Erciyes University Medical Faculty,Kayseri,Turkey
,
Musa Karakukcu
Affiliations:
Pediatric Hematology,Erciyes University Medical Faculty,Kayseri,Turkey
M. Akif Ozdemir
Affiliations:
Pediatric Hematology,Erciyes University Medical Faculty,Kayseri,Turkey
(Abstract release date: 05/21/15) EHA Library. Patıroglu T. 06/12/15; 102755; PB1700 Disclosure(s): Erciyes University Medical Faculty
Pediatric Hematology
Prof. Dr. Turkan Patıroglu
Prof. Dr. Turkan Patıroglu
Contributions
Abstract
Abstract: PB1700

Type: Publication Only

Background
Lupus anticogulant (LA) can be associated with bleeding as a consequence of hypoprothrombinemia.

Aims
We present the report of a patient with hemorrhagic symptoms in combination of hypoprothrombinemia and LA.

Methods
A 7 year-old girl was admitted with epistaxis for 5 days. On physical examination, she had scleral icterus, echimosis and hepatomegaly. The complete blood count was normal expect hemoglobin of 47 g/l. The coagulation screening showed a prolonged prothrombin time (PT) (60.8 s) and activated partial thromboplastin time (aPTT) (118.6 s). Although a packed fresh frozen plasma was administered to the patient, the prolonge of PT and aPTT continued. Therefore, the mixing study was performed but both PT and aPTT did not improve.

Results
Factor assay revealed a low prothrombin level ( 4,46 % ). The lupus anticoagulant and anti nuclear antibody were found positivity but anti DS-DNA was negativity in advanced laboratory examination of patient respectively. Further evaluation revealed the presence of immunoglobulin (Ig) G an M anticardiolipin antibodies, IgG and IgM anti-β-2-glycoprotein antibodies, IgG and IgM anti phosphotydilserin antibodies. After 5 days of mega dose methylprednisolone (30 mg/kg/d) was administered to the patient, PT( 13.6 s) and a PTT ( 38.4 s) were measured normal and directly Coombs test was found negatively. Therefore, mega dose methylprednisolone treatment was stopped. One month after stopping of the treatment with mega dose methylprednisolone she had again both prolonged PT and aPTT, and the positivity of directly Coombs test. In this period, it was revealed the presence of immunoglobulin (Ig) G an M anticardiolipin antibodies, IgG and IgM anti-β-2-glycoprotein antibodies, IgG and IgM anti phosphotydilserin antibodies. Hence, the patient re-treated with steroid. Her family asked the follow-up in another hospital.

Summary
The low dose immunosuppresive treatment in the patients with LA and hypoprothrombinemia is necessary to continue for the long time

Keyword(s): Anticoagulants, Antiphospholipid antibody

Session topic: Publication Only
Abstract: PB1700

Type: Publication Only

Background
Lupus anticogulant (LA) can be associated with bleeding as a consequence of hypoprothrombinemia.

Aims
We present the report of a patient with hemorrhagic symptoms in combination of hypoprothrombinemia and LA.

Methods
A 7 year-old girl was admitted with epistaxis for 5 days. On physical examination, she had scleral icterus, echimosis and hepatomegaly. The complete blood count was normal expect hemoglobin of 47 g/l. The coagulation screening showed a prolonged prothrombin time (PT) (60.8 s) and activated partial thromboplastin time (aPTT) (118.6 s). Although a packed fresh frozen plasma was administered to the patient, the prolonge of PT and aPTT continued. Therefore, the mixing study was performed but both PT and aPTT did not improve.

Results
Factor assay revealed a low prothrombin level ( 4,46 % ). The lupus anticoagulant and anti nuclear antibody were found positivity but anti DS-DNA was negativity in advanced laboratory examination of patient respectively. Further evaluation revealed the presence of immunoglobulin (Ig) G an M anticardiolipin antibodies, IgG and IgM anti-β-2-glycoprotein antibodies, IgG and IgM anti phosphotydilserin antibodies. After 5 days of mega dose methylprednisolone (30 mg/kg/d) was administered to the patient, PT( 13.6 s) and a PTT ( 38.4 s) were measured normal and directly Coombs test was found negatively. Therefore, mega dose methylprednisolone treatment was stopped. One month after stopping of the treatment with mega dose methylprednisolone she had again both prolonged PT and aPTT, and the positivity of directly Coombs test. In this period, it was revealed the presence of immunoglobulin (Ig) G an M anticardiolipin antibodies, IgG and IgM anti-β-2-glycoprotein antibodies, IgG and IgM anti phosphotydilserin antibodies. Hence, the patient re-treated with steroid. Her family asked the follow-up in another hospital.

Summary
The low dose immunosuppresive treatment in the patients with LA and hypoprothrombinemia is necessary to continue for the long time

Keyword(s): Anticoagulants, Antiphospholipid antibody

Session topic: Publication Only

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