It is estimated that about 1000 individuals with bleeding disorders exist in Khorasan Razavi province.23
We could obtain phone numbers of 552 cases in the region. Among them, 462 were males with common and rare bleeding disorders, 423 persons had underwent circumcision (91.5%) and 39 persons (8.5%) did not circumcise (). They included 284 cases with HA, 89 with HB, 23 with von Willebrand disease, 12 with factor V deficiency, 10 with factor VII deficiency, 2 with factor X deficiency, 19 with combined factor V and VIII deficiency, 16 with platelets disorders, 4 with factor XIII deficiency, 1 with afibrinogemia and 2 with factor XI deficiency.
A review of total number of male individuals with various inherited bleeding disorders and numbers who experienced post circumcision bleeding in each group.
Overall among 423 cases who circumcised 240 cases (56.7%) had passed the procedure with bleeding experience, while 183 cases (43.3%) had passed it successfully and without noticeable bleeding experience. Post circumcision bleeding has been known as early complication after the circumcision procedure. Its prevalence has been reported as 1–23% in normal infants and children and is depending on used method and experiences of practitioners.24
In HA group, 284 cases were identified and entered the study, among them cases 20 (7%) cases had not circumcised. Overall 264 individuals with HA had been circumcised (). Among them 117 cases (41.2%) had passed circumcision without bleeding. Their mean of age was 25.07 ± 1.44 year old with minimum 3 years, maximum 88 year and mean 23 year. The mean of factor VIII plasma level was 4.19 ± 5.88 % with minimum <1%, maximum 35%, median 1%. They include 89 cases (31.3%) with infusion of factor VIII concentrate, 11 cases (3.9%) using infusion of cryoprecipitate and 17 cases (6%) without usage any coagulation products.
In HA group, 147 cases (51.8%) had passed circumcision with bleeding episodes. Their mean of age was 31.16 ± 1.59 year with minimum 3 and maximum 91 and median 28 year old. Their mean of factor VIII level was 5.26 ± 5.63 % with minimum 1 and maximum 30 and median 3%. They include 132 cases (46.5%) who did not infuse any coagulation products, 12 (4.2%) had infused factor VIII concentrate and 3 (1.1%) had infused cryoprecipitate.
Chi-square test showed there is relation between severity of disease and post circumcision bleeding (p=0.029). The linear association was 0.008 that emphasizes on the lower factor VIII is associated with higher bleeding risk.
In HB group, 89 cases entered the study, 8 cases (9%) had not circumcised and 81 cases had been circumcised (91%). Among them 31 cases (34.8%) had passed circumcision without bleeding (). Their mean age was 21.90 ± 8.70 year with minimum 6, maximum 45 and median 22 years old. Also their mean of factor IX was 2.61 ± 3.57 % with minimum 1%, maximum 20% and median 1%. There was no relation between severity of disease and post circumcision bleeding, but there was linear by linear association 0.082. They include 29 cases (32.6%) that infused factor IX concentrate pre and post procedure, 1 case (1.1%) had infused FFP and 1 case (1.1%) claimed not to infuse any coagulation products.
On the other hand 50 cases (56.1%) had passed the procedure with bleeding episodes. Their mean of age was 28.26 ± 1.30 year, minimum 5 and maximum 67 and median 27 year old. The mean of factor IX level was 3.97 ± 4.82 %, minimum 1%, maximum 30% and median 2%. They include 43 cases (48.3%) that did not infused any coagulation products, 6 cases (6.7%) that infused factor IX concentrate and 1 case (1.1%) who infused fresh frozen plasma.
von Willebrand disease (vWD)
Generally 23 male cases with vWD with mean age of 29.04 years old and the youngest was a 11 year old boy and the oldest a 63 year man. They entered the study and all of them had been circumcised (). There is no data on subtypes of vWD in our patients due to lack of facilities and an expert researcher. Among them 11 cases (47.8%) had experienced bleeding that included 10 cases (43.5%) who did not infused cryoprecipitate or FVIIIC and 1 case (4.3%) who had bleeding despite infusion of cryoprecipitate. Also 12 cases (52.2%) had passed the procedure without bleeding and included 4 cases (17.4%) that infused cryoprecipitate, 4 cases (17.4%) that infused FFP and 4 cases (17.4%) that declared that infused no coagulation products.
Combined factor V and VIII deficiency (CF5F8D)
19 males with CF5F8D entered the survey. Two of them (10.5%) had not circumcised. Among 17 cases (89.5%) who had been circumcised, 13 cases (68.4%) had bleeding episodes and had not infused any coagulation products and 4 cases (21.1%) had passed circumcision using infusion of FVIIIC and FFP without any obvious haemorrhage ().
Platelet disorders (Plt)
16 males with Plt disorders entered the survey, of them 3 persons (18.7%) had not been circumcised (). We do not have exact subtypes of Plt disorders due to lack of aggregometry and related diagnostic facilities in our region. Among 13 remaining persons (82.4%) who underwent circumcision practice, 7 persons (57.14%) had not bleed and included 6 individuals who had infused Plt bags and a case who claimed had passed the procedure without Plt infusion. Also 6 persons (42.85%) had experienced bleeding that included 5 individuals who had circumcised without infusion of Plt and a case that had bleeding despite infusion of Plt.
Factor V deficiency
Overall 12 cases with factor V deficiency with mean age of 32 years old came into the study. The younger one was 17 year old and the oldest one was 61 year old. All of them had been circumcised (). Their mean of factor V level was 6.83% and six of them had severe form. Seven cases (58.3%) had post circumcision haemorrhage that include 6 persons (50%) who had not infuse FFP and a case (8.3%) who had bleeding despite infusion FFP.
Also 5 individuals (41.7%) had passed circumcision without bleeding episodes that included 3 cases (25%) that had not infused FFP and 2 persons (16.7%) that had infused FFP before the procedure.
Factor VII deficiency (FVIID)
There were 10 males with FVIID with mean age of 18.5 years old that 2 of them (20%) had not circumcised (). Among 8 circumcised cases the mean of factor FVII was 8.1% and there were 3 cases with severe form (FVII<1%).
Among 8 circumcised cases, five cases had experienced post circumcision bleeding. They included 2 persons (25%) who did not infuse factor VII concentrate or FFP before and after the procedure and had noticeable bleeding. Also 3 individuals (37.5%) who had infused factor VII or FFP concentrate before and after the procedure. One case of current group who had infused factor VII concentrate and experienced bleeding, reported 7 days lasting of haemorrhage after circumcision.
Also among 8 circumcised ones, 3 cases (37.5%) were the patients who had not infused factor VII concentrate and had passed circumcision without noticeable bleeding.
Factor XIII deficiency (FXIIID)
There were 4 cases with FXIIID with mean of 10.5 years old and 3 of them (75%) had not circumcised. The only individual with FXIIID, who had been circumcised, was a 9 years old child who stated that had passed circumcision with infusion of cryoprecipitate concentrate without bleeding. His factor XIII level was <1% ().
Factor X deficiency (FXD)
There were 2 males with FXD with 9 and 56 years old. The younger one with 1% of plasma level of factor X had not circumcised. The older one with factor X level ≈6% had circumcised without infusion of coagulation factor and no bleeding episodes ().
Factor XI deficiency
There were 2 brothers with factor XI deficiency without consanguineous marriage in the parents. The younger one was 10 years old with 2% of factor XI level and older was 13 years old with 11% of factor XI. Both of them had passed circumcision practice with infusion of FFP and without obvious bleeding. The discrepancy between plasma levels of factor XI in them is due to inadequate laboratory facilities in our area ().
There was a 31 years old case with afibrinogemia (factor XIII < 60 unit) without consanguine marriage in the parents. He underwent circumcision without infusion of any coagulation factor and had bleeding after the procedure ().