DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20162148

Combined protein C and protein S deficiency with pregnancy

Hemangi K. Chaudhari, Parmanand K. Shah, Keshav R. Pai, Natasha B. D’Souza

Abstract


A 35 year old female patient, married since 8 years, G6P1L1SA4 was registered with our unit since 8th week of gestation. She was a known case of protein C and protein S deficiency diagnosed 7 years ago (thrombotic profile- protein C: 70% normal: 70-100%, protein S: 55% normal: 70/140%, AT-III: 116% normal 70/140%, factor V leiden: no mutation detected) which was detected on evaluation for her bad obstetric history. ACLA was also positive. She was started on injection low molecular weight heparin 0.6 mg s/c OD, in addition to continuing Tb. Aspirin 75 mg which had been started when the pregnancy was registered. Foleys induction was done at 39 weeks of gestation in view of previous LSCS with pre-eclampsia. Emergency LSCS was done in view of previous LSCS with non-progress of labour. Male child weighing 2.4 kg was born which is doing well. A patient having combined deficiency of both factors protein C and protein S is very rare. Anticoagulation therapy is the cornerstone in the management of patients with inherited coagulation defects.

Keywords


Protein C deficiency, Protein S deficiency, Recurrent miscarriage, Deep vein thrombosis

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References


Hayashida M, Yamada H, Yamazaki S, Nomura H, Yoshimura K, Kitahara O, et al. Combined protein C and protein S deficiency in a family with repetitive thromboembolism and segregated gene mutations. Intern Med. 2003;42(3):268-72.

Formstone CJ, Hallam PJ, Tuddenham EG, Voke J, Layton M, Nicolaides K, et al. Severe perinatal thrombosis in double and triple heterozygous offspring of a family segregating two independent protein S mutations and a protein C mutation. Blood. 1996;87:3731-7.

Plutzky J, Hoskins JA, Long GL, Crabtree GR. Evolution and organization of the human protein C gene. Proc Natl Acad Sci USA. 1986;83:546-50.

Schmidel DK, Tatro AV, Phelps LG, Tomczak JA, Long GL. Organization of the human protein S genes. Biochemistry. 1990;29:7845-52.

Reitsma PH, Bernardi F, Doig RG, Gandrille S, Greengard JS, Ireland H, et al. Protein C deficiency: a database of mutations, 1995 update. On behalf of the subcommittee on plasma coagulation inhibitors of the scientific and standardization committee of the ISTH. Thromb Haemost. 1995;73:876-89.

Gandrille S, Borgel D, Ireland H, Lane DA, Simmonds R, Reitsma PH, et al. Protein S deficiency: a database of mutations. For the Plasma Coagulation Inhibitors Subcommittee of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost. 1997;77:1201-14.

Cuker A, Pollak SE. Protein C deficiency. eMedicine. 2009. Available at http:// emedicine.medscape.com/article/205470.

Martinez HR, Rangel-Guerra RA, Marfil LJ. Ischemic stroke due to deficiency of coagulation inhibitors. Report of 10 young adults. Stroke. 1993;24(1):19-25.

Barinagarrementeria F, Cantú-Brito C, De La Peña A, Izaguirre R. Prothrombotic states in young people with idiopathic stroke. A prospective study. Stroke. 1994;25(2):287-90.

Pabinger I. Thrombophilia and its impact on pregnancy. Thromb Res. 2009;123(3):16-21.

Vormittag R, Pabinger I. Thrombophilia and pregnancy complications. Hamostaseologie. 2006;26(1):59-62.

Regan L, Rai R. Thrombophilia and pregnancy loss. J Reprod Immunol. 2002;55:163-80.