Download to read offline
Rare coagulation disorder
- 1.
- 2. Presenter: DR.Md.Jaki yamani Resident-Phase:B Deptof Haematology,BSMMU Dhaka
- 4. Name:Sumiya Age:13year Sex:Female Address:Daponia,sadar,Mymensingh Religion: Islam Occupation: Class seven student. Date of admission:3-12-2017 Hospital reg: 489/2
- 5. History ofrecurrent bleeding manifestation since childhood.
- 6. According tothe statement of patient she had history of recurrent episodes of abnormal bleeding from minor cut or injury since from her childhood. She mentioned that her First episode of excessive bleeding had occurred from umbilical cord stump during her birth at hospital. After that she had several episodes of excessive bleeding from minor trauma or minor cut in different occasions. Most of the cases Bleeding was mild to moderate and stopped by transfusion of Fresh frozen plasma. Recently she experienced several episodes of epistaxis which was profuse and also subsided by transfusion of fresh frozen plasma.
- 7. For evaluationof the cause of bleeding she got admitted in Hematology department of BSMMU. On query, she gave history of occasional gum bleeding during tooth brushing. She gave no history of excessive menstrual bleeding, haematuria, per rectal bleeding ,haematemesis, haemoptysis, Purpuric rash, joint pain, oral ulceration, hair loss, Raynaud's phenomenon.
- 8. History ofjaundice in September of 2017 which was mild in nature, associated with prodromal period and subsided spontaneously. History of formation of psoas haematoma in 2015 and treated conservatively. Hospital admission for several time but failed to ascertained any definitive cause of her bleeding.
- 9. Tranexamic acid Aminocapryoic acid Ascorbic acid No history of anti platelet, anti coagulant drugs Fresh frozen plasma.
- 10. Came froma middle class family.
- 11. Menstrual flow-Average Menstrual duration-3 to 5 days Menarche – 12 year
- 12. She isimmunized according to EPI schedule.
- 13. No significantfamily history of bleeding disorder. No family history of consanguineous marriage.
- 14. Appearance: Healthy Nutrition: Average Body built: Average Anaemia: Not anaemic Jaundice: Not icteric Clubbing: Absent Temperature: Normal Koilonychia: Absent Leukonychia: Absent Cyanosis: Absent
- 15. Respiratory rate:16/min Pulse: 75/min Blood pressure: 110/60 mmhg JVP: Not raised Lymphadenopathy: No lymphadenopathy Bony tenderness: Absent Oedema: Absent Skin: No purpura,petechiae,ecchymosis,telengiactesia Thyroid: Not enlarged Dehydration: Absent. Decubitus: On choice
- 16. Gastro intestinalsystem: Unremarkable Respiratory system: Unremarkable Neurological system: Unremarkable Cardiovascular system: Unremarkable Musculoskeletal system: Unremarkable Haematological system: Unremarkable
- 17.
- 18.
- 19. Vascular disorder Connective tissue disorder Platelet functional disorder
- 20. Test Result DateNormal range D dimer 0.4µg/ml 3-12-2017 0.00-0.50 µg/ml Fibrinogen 155 mg/dl 3-12-2017 200-400 mg/dl Factor VIII assay 83 % 3-12-2017 50-150 mg/dl Factor IX assay 60 % 3-12-2017 50-150 % Factor XIII assay(Qualitative ) Normal factor XIII activity present 5-12-17 Von Willebrand factor (vWF) 71 % 3-12-2017 50-160% Urea Solubility (2 % acetic acid) Stable 23-11-2010
- 21. Test Result DateNormal range CBC Hb-12.3 gm/dl ESR-30 mm/1st hour RBC -4.39×1012 /L WBC-8.00×109 /L Platelet-230×109/L Differential count- N-63%,L-31%,M-2%, E- 4%,B-00% PCV-0.37 MCV-84fl MCH-28 pg MCHC-33 g/dl 3-12-2017
- 22. Test Result DateNormal range Clot retraction test Normal 24-11-2010 BT 5 min 25 sec 3-12-17 2-7 min CT 7 min 50 sec 3-12-2017 5-11 min TT 14.2 second 23-11-2010 16-20 second PFA-100 Reports pending PT 13.10 second 3-12-2017 12-16 seconds APTT 36 second 3-12-2017 36-40 second
- 23.
- 24.