Variable Manifestations of Severe Hypoprothrombinemia (Factor II Deficiency): 2 Cases
Sirisha Rani S*, Darshak Makadia, Lokesh Lingappa, Nikit Shah and Ramesh Konanki
Department of Hematology Oncology, Department of Paediatric Neurology, Rainbow Children’s Hospital and Perinatal Centre, Hyderabad, India
- Corresponding Author:
- Sirisha Rani S
Department of Hematology Oncology
Department of Paediatric Neurology
Rainbow Children’s Hospital and Perinatal Centre
E-mail: [email protected]
Received Date: December 06, 2013; Accepted Date: December 27, 2013; Published Date: December 31, 2013
Citation: Sirisha Rani S, Makadia D, Lingappa L, Shah N, Konanki R (2013) Variable Manifestations of Severe Hypoprothrombinemia (Factor II Deficiency): 2 Cases. J Blood Disord Transfus 5:192. doi:10.4172/2155-9864.1000192
Copyright: © 2013 Sirisha Rani S, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Case 1 is a 28 months female child, who has been symptomatic from 8 month of age with multiple, painful bruises over legs once in 5 to 6 weeks. Her complete blood picture was normal.PT and APTT were prolonged with normal fibrinogen and liver function. Case 2, became symptomatic from day 2 of life. He was treated for blood stained vomiting and black coloured stool and severe anaemia. Second episode was subdural hemorrhage and seizures. Investigations revealed abnormal PT and APTT with normal fibrinogen and liver function. Hereditary prothrombin deficiency is one of the rare congenital coagulation defect encountered in clinical practice. High index of suspicion is required to diagnose this condition with systematic approach as the facility to check factor 2 levels are not freely available in many centres. Bleeding manifestations are dependent on factor level. Children with severe deficiency are prone for life threatening bleeds. We report couple of children who had severe form of hereditary prothrombin deficiency with variable clinical manifestations. In both the cases, coagulation profile was suggestive of common pathway defect, PT and APTT were prolonged. Fibrinogen and Liver function tests were normal. No evidence of sepsis, no response to vitamin K. Further evaluation revealed low prothrombin activity (<1%). Factor v and x were normal.