Does the Coinheritance of Sickle Cell Trait and Non-O Blood Groups Provide Synergistic Protection against Spontaneous Bleeding in Severe Haemophilia ? = هل توريث سمة الخلايا المنجلية ومجموعات الدم غير التابعة لفصيلة O، تعطي حماية تآرزية ضد النزيف التلقائي في حالات الهيموفيليا سيولة الدم ؟

2018 ◽  
Vol 54 (2) ◽  
pp. 141-149
Author(s):  
Sagir G. Ahmed ◽  
Modu B. Kagu ◽  
Umma A. Ibrahim
2016 ◽  
Vol 3 (2) ◽  
pp. 50-54 ◽  
Author(s):  
Sagir G. Ahmed ◽  
Umma A. Ibrahim ◽  
Modu B. Kagu ◽  
Usman A. Abjah

Abstract Haemophilia A is an X-linked recessive disorder associated with deficiency of coagulation factor VIII and lifelong bleeding diathesis. Sickle cell trait (SCT) is the heterozygous state for the sickle β-globin gene. The frequency of SCT is up to 30% in Africa, wherein it confers survival advantage by providing resistance against severe malaria. SCT does not cause vaso-occlusive crisis, but is associated with high risk of venous thromboembolism as variously reported in the literature. We consider SCT as a hypercoagulable prothrombotic state and hypothesise that coinheritance of SCT may ameliorate the clinical phenotype of severe haemophilia. We conducted a retrospective analysis of frequencies of spontaneous bleeding among severe haemophiliacs with SCT (Hb AS phenotype) and their counterparts with normal Hb phenotype (Hb AA phenotype) in order to determine the possible ameliorating effect of SCT on spontaneous bleeding rates in severe haemophilia A. If our hypothesis is correct, severe haemophiliacs with SCT will have lower frequencies of spontaneous bleeding than their counterparts with normal Hb phenotype. Our results revealed that severe haemophiliacs with normal Hb phenotype had significantly higher mean annual bleeding episodes per patient in comparison with their counterparts with SCT (45±7 vs 31±5, p=0.033), suggesting that severe haemophiliacs with SCT had lower frequencies of spontaneous bleeding episodes. The result of this study indicates that coinheritance of SCT in patients with severe haemophilia may be associated with reduced frequency of spontaneous bleeding, which may imply better overall prognosis. However, the study has important limitations, which include its retrospective nature and the very low number of subjects. The findings should therefore be validated by a larger and prospective study.


The Lancet ◽  
1953 ◽  
Vol 262 (6792) ◽  
pp. 909-911 ◽  
Author(s):  
C Choremis ◽  
ElizabethW Ikin ◽  
H Lehmann ◽  
A.E Mourant ◽  
Leda Zannos

1966 ◽  
Vol 15 (4) ◽  
pp. 404-408 ◽  
Author(s):  
Narendra Kumar

SummaryBlood samples from five hundred and ninety five individuals belonging to Indore district of west Madhya Pradesh were tested for ABO blood groups, and two hundred and ninety three of them were tested for the sickle-cell trait.The investigated groups include: the Kadve Kulmi, the Khati, the Rami Mali, the Balai, the Bhil and a mixed sample of various castes. The aforesaid groups, within the district, are found to be homogeneous, as far as the ABO blood groups distributions are concerned. Frequencies for the O, A and B genes have been found to be 0.5251, 0.1587 and 0.3162, respectively, in the pooled data.Sickle-cell trait has been detected in the Balai, the Charmakar or Chamar and the Bhil.


The Lancet ◽  
1952 ◽  
Vol 260 (6743) ◽  
pp. 1010-1012 ◽  
Author(s):  
F. Dreyfuss ◽  
ElizabethW. Ikin ◽  
H. Lehmann ◽  
A.E. Mourant

1977 ◽  
Vol 137 (3) ◽  
pp. 281a-281
Author(s):  
P. E. Mickelson

2020 ◽  
pp. 1-2
Author(s):  
Michael Alperovich ◽  
Eric Park ◽  
Michael Alperovich ◽  
Omar Allam ◽  
Paul Abraham

Although sickle cell disease has long been viewed as a contraindication to free flap transfer, little data exist evaluating complications of microsurgical procedures in the sickle cell trait patient. Reported is the case of a 55-year-old woman with sickle cell trait who underwent a deep inferior epigastric perforator (DIEP) microvascular free flap following mastectomy. The flap developed signs of venous congestion on postoperative day two but was found to have patent arterial and venous anastomoses upon exploration in the operating room. On near-infrared indocyanine green angiography, poor vascular flow was noted despite patent anastomoses and strong cutaneous arterial Doppler signals. Intrinsic microvascular compromise or sickling remains a risk in the sickle cell trait population as it does for the sickle cell disease population. Just like in sickle cell disease patients, special care should be taken to optimize anticoagulation and minimize ischemia-induced sickling for patients with sickle cell trait undergoing microsurgery.


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