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hemoglobin S/beta thalassemia 0

Pathology: 1) vasoocclusive crises 2) aseptic necrosis of bone Clinical manifestations: - episodic abdominal pain (case report) - gallstones [3] (case report) Laboratory: 1) complete blood count (CBC) - microcytic anemia - blood hemoglobin 7-9 g/dL [2] - MCV 60-80 fL 2) peripheral smear a) sickle cells b) target cells 3) hemoglobin electrophoresis: - Hgb S (90-99%), Hgb F (1-10%), Hgb A2 (>3.5%), Hgb A (0%) 4) serum unconjugated bilirubin elevated Radiology: - abdominal CT (images) [3]

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, American College of Physicians, Philadelphia 2015
  3. Gupta A, Jain P Calcified Spleen and Gallstones N Engl J Med 2018; 378:380. Jan 25, 2018 PMID: 29365300 Free full text http://www.nejm.org/doi/full/10.1056/NEJMicm1703915
  4. 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