Although sickle cell anaemia (Hb SS) and sickle cell beta zero thalassemia (Hb Sb thal) phenotypes typically represent those at highest risk for complications and the most likely to want a bone marrow transplant (BMT), other phenotypes, such as Hb SC
or Hb Sb+ thal, are not always spared the complications of SCD.
Among the 11 cases illustrated in Table 1, two were Hb SB-thal and the rest were equally divided among Hb SS and Hb SC
There have been case reports of luteal cyst rupture with massive hemoperitoneum during dialysis, thrombolytic therapy, patient with Hb SC
disease, liver disease and patient on anticoagulation therapy.
In addition, these assays create an increased risk for reporting misleading HbA1c results for patients harboring homozygous Hb S, Hb SC
, or Hb S-b-thalassemia, where red blood cell life span is decreased.
(7,8) In addition, the heterozygous conditions of Hb SC
and compound heterozygote Hb S-[beta]-thalassemia are also known to cause increased red blood cell turnover.
Subjects included individuals with Hb AA, Hb AS, Hb SC
, and Hb SS genotypes; the presence of Hb S in the blood samples did not affect the [Hb] measurements (data not shown).
The frequency of crisis, degree of anaemia, and the organ systems involved vary considerably from individual to individual.12 Morbidity in sickle cell disease arises Primarily from acute chest syndrome, cerebrovascular accidents, and splenic and renal dysfunction.13 Hb SS (sickle cell anaemia) or Hb S/b-0-thalassemia had higher frequency of acute chest syndrome or painful crises, than individuals and Hb S/b +- thalassemia or Hb SC
In patients with Hb SC
, [beta].sup.S] CAR haplotype was the most frequent, followed by haplotypes Benin and Cameroon.
Homozygous sickle cell anaemia (Hb SS) is the most common while sickle cell trait, doubly heterozygous conditions of Hb SC
and Hb Sssthal also cause sickling disease2.
Some people with this genotype develop Hb SC
disease, a variant of sickle cell disease.
Orak hucreli sendromlarin klinik ozellikleri Durum Klinik tablo Orak hucre tasiyiciligi; Yok, nadiren agrisiz hematuri Orak hucre Vazookluziv krizlerle beraber dalak, anemisi; beyin, kemik iligi, bobrek, akciger infarkti, aseptik kemik ve eklem nekrozlari, safra taslari, priyapizm, ayak bilegi ulserleri S/[[beta].sup.o] talassemi; Vazookluziv krizler, aseptik kemik ve eklem nekrozlari S/[[beta].sup.+]talassemi; Nadiren krizler ve aseptik kemik nekrozu Hb SC
; Nadiren krizler ve aseptik kemik nekrozu Durum Hemoglobin duzeyi g/dL Orak hucre tasiyiciligi; Normal Orak hucre 7,0-10.0 anemisi; S/[[beta].sup.o] talassemi; 7,0-10,0 S/[[beta].sup.+]talassemi; 10,0-14,0 Hb SC