Hemophilia in Iran

Figures & data

Figure 1 Prevalence comparison of hemophilia with rare bleeding disorders in Iran (WFH 2013 survey).¹⁰ FI, factor I; FII, factor II; FV, factor V; FV-FVII, combined factor V and factor VIII; FVII, factor VII; FX, factor X; FXI, factor XI; FXIII, factor XIII.

Table 1 Number of patients with hemophilia in different parts of Iran^11–17

Study	Origin	Number of patients with HA	Severe (percent)	Moderate (percent)	Mild (percent)	Number of patients with HB
Sharifian et al. (2003)	Throughout Iran	1280	635 (49.6)	277 (21.6)	368 (28.8)	—
Company et al. (2011)	Sanandaj city (Kurdistan Province)	104	82 (78.8)	16 (15.4)	6 (5.8)	—
Mansouritorghabeh et al. (2013)	North eastern	287	143 (49.8)	59 (20.6)	85 (29.6)	92 Severe 43 (46.7%) Moderate 33 (35.9%) Mild 16 (17.4%)
Ziaei et al. (2005)	North western	120	—	—	—	18
Karimi et al. (2009)	Southern	326	38%*	—	—	46
Mehdizadeh et al. (2009)	Tehran Province	885 (1077)	468 (55.5)	101 (12)	275 (32.6)**	162 (211)*** Severe 79 (53.7%) Moderate 30 (20.4%) Mild 38 (25.9%)
Ravanbod et al. (2011)	Throughout Iran	588	473 (80)	115 (20) ****	115 (20) ****	—

*The authors stated that 38% (156) patients with common bleeding disorders had a severe degree of factor deficiency.

**In 41 cases, the severity of disease was not determined.

***In 15 cases, the severity of disease was not determined.

****In 115 cases, the severity of disease was mentioned as mild or moderate.

Table 2 Spectrum of FVIII gene defects among Iranian patients with HA^20–22

			Common gene defects (number)
Study	Origin	Total number	Inversion of intron 22	Inversion of intron 1	Other mutations
Ravanbod et al. (2011)	ICHCC	580	201	5	(1)  51 missense (2)  15 nonsense (3)  45 frame-shift (4)  11 splice-site (5)  1 large duplication
Boekhorst et al. (2008)	ICHCC	199	66	2	(1)  missense in 75 p (2)  Small deletion/insertion in 31 p (3)  Large deletion in 5 p (4)  Nonsense mutation in 13 p (5)  Splice site mutation in 7 p
Haghpanah et al. (2011)	southern Iran	41 p out of 56	23	0	(1)  missense in 2 p (2)  Frameshift in 1 p (3)  Nonsense mutation in 9 p (4)  Polymorphism in 6 p

Table 3 Spectrum of FIX gene defects among Iranian patients with HB^30–33

Study	Origin	Total number	Genetic defects
Kamali et al. (2007)	Isfahan	24	(1)  Missense (70.8%) (2)  Deletion (16.7%) (3)  Nonsense (8.3%) (4)  Insertion (4.2%) (5)  Malmo polymorphism (Ala 148 Thr) in 1 p (6)  Four of mutations were novel
Karimi et al. (2010)	Khorasan	14	(1)  Missense (69.2%) (2)  Nonsense (23.1%) (3)  Insertion (7.7%) (4)  Malmo polymorphism (Ala 148 Thr) in 2 p (5)  Two of the mutations were novel
Karimipour et al. (2009)	Tehran	28	(1)  16 missense (2)  5 nonsense (3)  3 mutations in the promoter region (4)  4 small deletions (5)  One of the mutations was novel
Karimipour et al. (2007)	Tehran	76	(1)  33 missense (2)  2 splice site (3)  11 nonsense (4)  2 frameshift (5)  4 mutations in the promoter region (6)  Seven of mutations were not reported previously

Table 4 Clinical presentations of a number of Iranian patients with HA and HB^11,13

Study	Number of HA patients	First clinical presentations	Clinical presentations	Number of HB patients	First clinical presentations	Clinical presentations
Mehdizadeh et al. (2009)	885	Ecchymosis 103 (13.2%) Hemarthrosis 68 (8.7%) Muscle hematoma 9 (1.2%) Post-dental extraction bleeding 50 (6.8%) Post-circumcision bleeding 113 (14.5%) Cord blood bleeding 4 (0.5%) Gastrointestinal bleeding 5 (0.4%) Menorrhagia 2 (0.3%)	—	162	Ecchymosis 15 (10.9%) Hemarthrosis 11 (8%) Muscle hematoma 5 (3.6) Post-dental extraction bleeding 13 (9.5) Post-circumcision bleeding 17 (12.4) Cord blood bleeding 0 (0) Gastrointestinal bleeding 0 (0) Menorrhagia 1 (0.7)	—
Mansouritorghabeh et al. (2013)	287	—	hemarthrosis 207 (72.6%) epistaxis 160 (55.9%) vast ecchymosis 203 (71%) hematuria 92 (32.3%) umbilical cord bleeding 5 (1.8%) CNS bleeding 7 (2.4%) post-dental extraction bleeding 105 (89%) gastro-intestinal tract bleeding 60 (21%) hematoma 12 (4.2%) gum hemorrhage 15 (5.3%)		—	hemarthrosis 64 (69.9%) epistaxis 51 (55.4%) vast ecchymosis 53 (57.6%) hematuria 31 (33.7%) umbilical cord bleeding 5 (5.4%) CNS bleeding 7 (7.6%) post-dental extraction bleeding 37 (97.4%) gastro-intestinal tract bleeding 22 (23.9%) gum hemorrhage 3 (3.3%)

Table 5 Prevalence of inhibitor among Iranian patients with HA and HB with inhibitor features

				Patients with inhibitor based on severity of disease			Type of inhibitor
Study	Origin	Total number of patients	Patients with inhibitor	Severe	Moderate	Mild	High responding	Low responding
Sharifian et al. (2003)	Throughout the Iran	1280 HA	184 (14.4%)	145 (22.8%)	26 (9.4%)	13 (3.5%)	41 (22.2%)	143 (77.8%)
Company et al. (2010)	Sanandaj city (Kurdistan Province)	104 HA	20 (19.2%)*	10 (10.5%)	4 (3.8%)	2 (1.9%)	4 (20%)	16 (80%)
Boekhorst et al. (2008)	Iran	199 HA	19 (10%)	18 (12%)**	1 (4%***)	0 (0%)	—	—
Haghpanah et al. (2011)	Southern Iran	56 HA	8 (14.28%)	7 (87.5%)****	—	—	—	—
Mansouritorghabeh et al. (2005)	Northeastern Iran	48 HB	3 (6.3%)	2 (9.1%)	0 (0%)	1 (12.5%)	3 (100%)	—
Modaresi et al. (2006)	Northeastern Iran	102 HA	20 (19.6%)	11 (25%)	5 (17.8%)	4 (13.3%)	—	—
Eshghi et al. (2005)	East and south of Iran	355 HA	100 (28.1%)	—	—	—	14 (14%)	86 (86%)
Nesheli et al. (2013).	North	52 HA	9 (17.3%)	—	—	—	6 (66.7%)*****	3 (33.3%)*****

NM, not mentioned.

*In this study, the number of patients with inhibitor was reported to be 20 cases among whom 16 were low responding and 4 were high responding but based on severity of disease, 16 patients were mentioned to have had inhibitor.

**12% of all Iranian patients with severe deficiency had inhibitor.

***4% of 28 Iranian patients with moderate factor deficiency had inhibitor.

****Authors only stated that from eight patients with inhibitor, seven had severe form of HA.

*****Study only mentioned high titer and low titer inhibitor.

Table 6 Prevalence of HCV infection among Iranian patients with hemophilia^50–61

Study	Total number	HCV+
Yazdani et al. (2012)	350	232 (66%)
Mousavian et al. (2011)	1095*	802 (72.3%)
Alavian et al. (2001)	176	106 (60.2%)
Nassiri Toosi et al. (2008)	236	>80%**

*This study assessed 1095 patients with HA, HB, Glanzmann, and other inherited bleeding disorders.

**>80% rate was observed among a total of 236 patients with HA, HB, and other bleeding disorders.

Table 7 Prevalence of HBV, HIV, and HCV infections among Iranian patients with hemophilia^12,62–65

		Total		Total infected patients		HBs+		HIV+		HCV+
Study	Origin	HA	HB	HA	HB	HA	HB	HA	HB	HA	HB
Ziaei et al. (2005)	Northwestern	120	18	52 (43.3%)	7 (38.8%)	2 (1.6%)	0	0*	0	50 (96.1%)	7 (100%)
Sharifi-Mood et al. (2006)	Southeast	74		26 (35%)		3 (4%)		0 (0%)		23 (31%)
Zahedi et al. (2004)	Kerman city	74	23	35 (47.5%)	9 (39.1%)	1 (2.9%)	0	—	—	34 (97.1%)	9 (100%)
Assarehzadegan et al. (2012)	Ahvaz city	87		48 (55.2%)		1 (1.1%)		—		47 (54%)
Mansour-Ghanaei et al. (2002)	Guilan Province	71	21**	NM	NM	19 (26.8%)	7 (33.3%)	—	—	54 (76%)	16 (24%)

*This center had only one HIV+ patient who died six years before that study.

**This study was conducted on 101 patients among whom 9 patients had a coagulation disorder other than HA and HB.

NM, not mentioned.

Report on the WFH Global Survey. Compiled and produced by the World Federation of Hemophilia. 2014.

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