MEDizzy - A 28-year-old African-American woman has had worsening

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Combined Hematology II

A 28-year-old African-American woman has had worsening fatigue for the past 2 months. On physical examination she has an erythematous macular rash on her upper chest, forearms, and face. Laboratory studies show Hgb 9.2 g/dL, Hct 27.9%, MCV 101 fL, platelet count 179,000/microliter, and WBC count 5850/microliter. The red blood cell distribution width is markedly increased. Her peripheral blood smear shows polychromasia. Her reticulocyte count is 4.2%. The serum haptoglobin is 3 mg/dL. Serum chemistries show total protein 7.9 g/dL, albumin 3.8 g/dL, alkaline phosphatase 49 U/L, AST 81 U/L, ALT 27 U/L, total bilirubin 3.3 mg/dL, and direct bilirubin 0.8 mg/dL. Hemoglobinuria is detected on urinalysis. Which of the following underlying conditions is she most likely to have?

ExplanationB. Clinically this patient is having hemolytic anemia with intravascular hemolysis and features of SLE (presence of rash). In some AIHA, the hemolysis can be intravascular. The major hematological manifestations of SLE are anemia, leucopenia, thrombocytopenia, and antiphospholipid syndrome (APS). Autoimmune hemolytic anemia (AIHA) may manifest in SLE patients at the time of diagnosis or within the first year of the disease. Anemia is found in approximately 50% of patients, with anemia of chronic disease (ACD) being the most common form. Iron deficiency anemia (IDA), autoimmune hemolytic anemia (AHA), anemia of chronic renal insufficiency and cyclophosphamide-induced myelotoxicity are the most frequent causes. Patients with autoimmune hemolytic anemia usually belong to a distinct category, which is associated with anticardiolipin antibodies, thrombosis, thrombocytopenia and renal involvement, often in the context of secondary antiphospholipid syndrome. Overt autoimmune hemolytic anemia (AIHA) is characterized by an elevated reticulocyte count, low haptoglobin levels, increased indirect bilirubin concentration, and a positive direct Coombs' test, has been noted in up to 10 percent of patients with SLE. The AIHA in SLE patients is seldom severe and rarely fatal as prednisolone is usually sufficient in controlling hemolysis.