Aplastic anemia associated to systemic lupus erythematosus in an AIDS\npatient: a case report
This report describes the clinical course of a 39-year-old woman who was referred to our\ninstitute for evaluation due to persistent pancytopenia with intermittent transfusion\ntreatment (packed red blood cells and platelets). Her medical history indicated HIV\ninfection for 15 years, regular HAART (tenofovir, lamivudine, ritonavir, and atazanavir)\nwith good and prolonged virological control (viral load < 50 copies/mL and CD4\nT-lymphocyte count of 628 cells/ µL), intermittent polyarthritis of the hands,\ntransvaginal bleeding and bacterial pneumococcal meningitis four months earlier. There\nwas no report of fever, rash, photosensitivity, oral or nasal ulcers, or neurological or\nrenal disorders. Her physical examination was normal, except for pallor and petechiae in\nthe lower limbs without adenopathy or hepatosplenomegaly.\nRepeated tests performed within the previous 16 weeks showed anemia (hemoglobin level:\n6.8-9.7 g/dL; mean cell volume, 90.5-110.0 fL), absolute reticulocyte count (8.7-29.1 ×\n109/L), leukopenia (1.71-3.49 × 109/L) with neutropenia\n(0.58-1.53 × 109/L), variable lymphocyte count (0.44-2.79 × 109/L)\nand severe thrombocytopenia (2-10 × 109/L). An extensive laboratory\nevaluation was conducted for vitamin B12, folate, bilirubin, lactate dehydrogenase,\nhaptoglobin, rheumatoid factor, complement components (C3 and C4), prothrombin time,\nactivated partial thromboplastin time, thyroid function, and renal and hepatic tests\nwithout alterations. Serologic markers for hepatitis B and C, cytomegalovirus\n(immunoglobulin M), and parvovirus (immunoglobulin M) were nonreactive. A direct\nantiglobulin test was negative. Urinary protein was 610 mg/24h. Effusions were not\ndetected in imaging studies. A myelogram showed severe hypoplasia of the erythrocytic,\ngranulocytic, and megakaryocytic series with the presence of 40% of typical lymphocytes\nand no evidence of infiltration by parasites or tumor cells. A bone marrow biopsy\nconfirmed hypocellularity with reduced hematopoiesis (5% cellularity) and absence of\nreticular fibrosis or necrosis ( and\n). Analysis of antinuclear antibodies (>\n1:640, coarse speckled nuclear pattern), anti-ribonucleoprotein (1:1600) and anti-Sm\n(1:800) antibodies were reactive at high titers (antiScl70 and anti-native DNA did not\nreact). In accordance with the criteria of the American College of Rheumatology for SLE\nand the International Agranulocytosis and Aplastic Anemia Study Group, the diagnosis of\nSLE-associated AA was proposed.\nThe potential toxic effects of antiretroviral drugs as a cause of AA were tested before\nthe start of immunosuppressive therapy through a temporary eight-week treatment\ninterruption, but no change in the hematological picture or transfusion dependence was\nobserved. Thus, according to the hypothesis of SLE-associated AA, immunosuppression with\nprednisone (1 mg/kg/day) and hydroxychloroquine (400 mg/day) was initiated with\nprogressive and sustained improvement of the cytopenias during the following six weeks\nand total transfusion independence. The patient remained in follow-up with regular use\nof hydroxychloroquine and HAART; the CD4 count > 500 cells/µL and no cytopenias were\nreported after 20 months.