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hemoglobin SC disease
Sickling disorders less severe than hemoglobin SS (sickle cell) disease.
Pathology:
1) splenomegaly rather than splenic atrophy generally occurs
2) rare vasoocclusive crises (painful crises)
3) rare aseptic necrosis of bone
4) overactivity of SLC12A4 may contribute to erythrocyte dehydration
Clinical manifestations:
- hematuria (painless)
Laboratory:
1) complete blood count (CBC)
- blood hemoglobin variable (10-15 g/dL) [1]
- MCV >= 75 fL
2) peripheral smear
- fat sickle cells
- target cells
3) reticulocyte count: reticulocytosis (case: 4.5%) [3]
4) hemoglobin electrophoresis:
-> Hgb S (50%), Hgb C (50%), Hgb A (0%)
5) hemoglobin genotyping
Interactions
disease interactions
General
hemoglobinopathy
hemolytic anemia
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 17.
American College of Physicians, Philadelphia, 1998, 2015
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 648
- NEJM Knowledge+
- Pecker LH, Schaefer BA, Luchtman-Jones L.
Knowledge insufficient: the management of haemoglobin SC disease.
Br J Haematol. 2017 Feb;176(4):515-526.
PMID: 27982424 PMCID: PMC5303157 Free PMC article. Review.