hemoglobin SC disease

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hemoglobin SC disease

Hematology A sickling disorder resulting from inheritance of a HbS gene from one parent and a HbC gene from the other; RBCs contain ± equal amounts of each Hb; HbA is absent; HbF may be ↑ Clinical Similar to, but less severe than, sickle cell anemia; by preadolescence, episodic skeletal or abdominal pain, moderate splenomegaly; unique to SC disease is an ↑ risk of retinal disease–proliferative retinopathy, retinal vascular disease, aseptic necrosis of femoral head, acute chest syndrome after fat embolism due to bone infarction. See Sickle cell anemia.

hemoglobin SC disease

A disease of those who have inherited two abnormal forms of hemoglobin, S and C. The affected may have vaso-occlusive crises similar to those seen in sickle cell anemia, with bony and visceral infarcts.
References in periodicals archive ?
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 genotypes.
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.14
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; 10,0-14,0