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hemoglobin S/beta thalassemia +

Pathology: 1) rare vasoocclusive crises 2) rare aseptic necrosis of bone Laboratory: 1) complete blood count (CBC) - microcytic anemia (mild when it occurs) - blood hemoglobin 9-12 g/dL [2] - MCV 70-75 fL [2] 2) peripheral smear a) rare sickle cells b) target cells 2) hemoglobin electrophoresis: - Hgb S (>60%), Hgb A (10-30%), Hgb A2 (>3.5%) [2]

General

beta thalassemia

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 648
  2. Medical Knowledge Self Assessment Program (MKSAP) 17, 18. American College of Physicians, Philadelphia 2015, 2018
  3. Benites BD, Bastos SO, Baldanzi G et al Sickle cell/beta-thalassemia: Comparison of Sbeta0 and Sbeta+ Brazilian patients followed at a single institution. Hematology. 2016 Dec;21(10):623-629. Epub 2016 May 28. PMID: 27237196