Awidi et al. (1993) conducted a four-year prospective study on patients admitted or referred with thromboembolic disease to Jordan University Hospital or to the Thrombosis/Haemostasis Laboratory at the University of Jordan. Hereditary thrombophilia was diagnosed in the absence of an acquired cause of thrombophilia in addition to two of the following: 1) positive family history of thrombophilia, 2) confirmed same deficiency in a closely related family member, 3) the deficient protein is constantly below 2 SD of the normal mean on repeated testing. There were a total of 217 patients (102 males and 115 females) with confirmed thromboembolic disease. Their mean age was 34 years. A total of 49 patients (26 males and 23 females) fulfilled the criteria of hereditary thrombophilia. There were 17 cases of protein C deficiency (PC), 15 protein S deficiency (PS), 10 antithrombin III deficiency (ATIII), 3 dyfibrinogenemia, 2 heparin cofactor II deficiency, and 2 plasminogen defects. In this group most of the thrombosis was venous. A positive family history was obtained in 65.3% of patients with hereditary thrombophilia. Twenty-seven additional relatives with deficiency were identified upon family studies. Awidi et al. (1993) calculated the prevalence of hereditary thrombophilia in Jordan to be at 1/25,000.

Aboobacker et al. (2004) described a Kuwaiti infant diagnosed with homozygous severe protein C deficiency. The child was the first born to a consanguineous couple; the father was a Kuwaiti, while the mother was a Saudi Arabian. At 4-weeks of age, she presented with a spontaneous painful swelling over the left leg, which was treated with antibiotics. At 2-months of age, a similar erythematous swelling which turned blackish and necrotic. Investigations revealed absent protein C antigen and activity. Assay of protein C in both parents revealed deficiency, indicating heterozygous states, and the patient was diagnosed with homozygous protein C deficiency. She was treated with I.V. fresh frozen plasma, antibiotic, and oral warfarin. However, she continued to get episodes of purpura fulminans and thrombotic lesions, following which she was put on a long-term prophylaxis of subcutaneous infusion of protein C concentrate along with oral warfarin. This procedure dramatically reduced the episodes of purpura fulminans. Lensectomy and vitrectomy revealed irreversible total retinal detachment in the left eye causing blindness. She was found to have no adverse effects from the protein C infusion. At two-years she showed normal growth patterns, except for the blindness in the left eye.

[Aboobacker KC, Al-Matar ER, Bourahma MH. Homozygous severe protein C deficiency type I: long-term prophylactic intermittent therapy with subcutaneous purified protein C concentrate: first case report from Kuwait. Kuwait Med J. 2004; 36(2):137-9.]

Pathare et al. (2006) studied the prothrombotic risk factors in Omani patients who presented with their first thrombophilic event. All Omani patients (39 patients with 24 females and 15 males) who were admitted between 2001 to 2003 with a first episode of deep venous thrombosis (DVT) with or without pulmonary embolism (PE) or with thrombosis in an unusual site were included in the study with exclusion of those with past history of stroke, myocardial infarction, malignancy, severe illness, surgical operation, or trauma requiring bed rest for more than one week in the six weeks period before admission. The median age at the time of the thrombolic event was 37.9 years, and the mean age of onset was lower in females (34.6 years) than in males (43.2 years). Most of the cases presented with DVT (46.1%), while PE was diagnosed in 20.5% and 33.3% had thrombosis at unusual site. In 27.3%, an underlying clinical condition was detected which included central venous catheter, infection, venous thrombosis, cardiac thrombus, sickle cell disease, and lupus anticoagulant. Low levels of protein C were detected in six patients (15.38%). One patient was found to have a combination of both MTHFR and CBS 844ins68 mutations with low protein C level. Pathare et al. (2006) suggested the conduction of a larger study to relate the hereditary markers for thrombophilia to the actual event.

Seligsohn et al. (1984) studied an Arab family in which 2 sibs with first-cousin parents died with massive venous thrombosis in the neonatal period. Protein C antigen was undetectable by immunologic assays of plasma available from one infant. Both parents and 12 of 25 relatives were heterozygous. None of these (age range, 4-70 years) had had thrombotic episodes.

Abu-Amero et al. (2003) described a nine-month-old Saudi boy with protein C deficiency with purpura fulminans.  He was born to consanguineous parents after an uneventful pregnancy at 39 weeks.  At birth, he had multiple bullae on the left lobar region of the abdomen, right cheek, right elbow, and an occipital region filled with fluid.  He was well at the age of 3 months, with no neurological deficit, but blind.  At the age of 4 months, he was on warfarin and the FFP was stopped.  Two months later he was admitted to the hospital due to discoloration of the scrotum, so the FFP was given again.  At the age of one year, the patient remained free of new lesions with protein C concentrate and warfarin treatments.