A 37-year-old woman with known systemic lupus erythematosus is evaluated for generalized weakness and dyspnea on exertion. She has no weight loss, night sweats, hemoptysis, or cough. Her current medications include hydroxychloroquine, naproxen, and low-dose prednisone. She stopped taking azathioprine 4 weeks ago due to side effects.
Her blood pressure is 142/90 mm Hg and pulse is 82/min. The oropharynx is moist with no visible lesions. Small, mobile lymph nodes are palpable in the submandibular area. Skin examination shows a scaly erythematous rash over the face, scalp, and upper trunk. Cardiopulmonary examination is within normal limits. There is no splenomegaly.
Laboratory results are as follows:

Peripheral blood smear shows polychromatic red blood cells, spherocytes, and normal platelets. No schistocytes are seen.
Which of the following is most likely responsible for this patient's symptoms?

Question

A 37-year-old woman with known systemic lupus erythematosus is evaluated for generalized weakness and dyspnea on exertion.  She has no weight loss, night sweats, hemoptysis, or cough.  Her current medications include hydroxychloroquine, naproxen, and low-dose prednisone.  She stopped taking azathioprine 4 weeks ago due to side effects.
Her blood pressure is 142/90 mm Hg and pulse is 82/min.  The oropharynx is moist with no visible lesions.  Small, mobile lymph nodes are palpable in the submandibular area.  Skin examination shows a scaly erythematous rash over the face, scalp, and upper trunk.  Cardiopulmonary examination is within normal limits.  There is no splenomegaly.
Laboratory results are as follows:

Peripheral blood smear shows polychromatic red blood cells, spherocytes, and normal platelets.  No schistocytes are seen.
Which of the following is most likely responsible for this patient's symptoms?

Quizplus · Accepted Answer

11ec193a_983a_a3a6_853d_778fc131ffec_MD0002_00 This patient's presentation suggests autoimmune hemolytic anemia (AIHA), a complication in about 10% of patients with systemic lupus erythematosus (SLE). AIHA is due to autoantibodies (warm agglutinins or IgG antibodies and cold agglutinins or IgM antibodies) destroying red blood cells. Nearly 80% of cases of AIHA are due to warm agglutinin disease. Patients typically present with signs and symptoms of anemia. Laboratory findings include decreased hemoglobin and serum haptoglobin, increased indirect bilirubin and lactate dehydrogenase, reticulocytosis, and spherocytosis on peripheral smear due to partial phagocytosis of IgG-coated red blood cells. Spherocytosis results in increased mean corpuscular hemoglobin concentration. Elevated mean corpuscular volume (MCV) is due to the reticulocytosis. Diagnosis is established by a positive direct antiglobulin (Coombs') test. Steroids are first-line treatment for SLE-associated AIHA, with rapid tapering after the hematocrit increases and reticulocytosis decreases. Splenectomy is indicated if there is no response to steroids or in patients who require high-dose steroids to maintain response. (Choice A) Anemia of chronic disease is usually normocytic and normochromic with low reticulocyte count, serum iron, and transferrin level. Serum ferritin levels are usually normal or elevated without evidence of hemolysis. MCV can be normal or low. Spherocytes are not seen. (Choice C) Folic acid deficiency alone does not cause hemolytic anemia, although its requirement is generally increased in pregnant patients and those with chronic hemolytic anemias. Elevated MCV in this patient is due to reticulocytosis. (Choice D) Microangiopathic hemolytic anemia typically has evidence of red cell fragmentation ( schistocytes) on peripheral blood smear. (Choice E) Pure red cell aplasia usually presents with normocytic and normochromic anemia with very low (or zero) reticulocytes. It is usually idiopathic but can be caused by drugs, thymoma, or parvovirus infection. Educational objective: Systemic lupus erythematosus patients may present with warm agglutinin autoimmune hemolytic anemia. Diagnosis is established with positive direct Coombs' test with IgG. Corticosteroids are the first-line therapy. Refractory disease requires splenectomy. __________ References: How I treat autoimmune hemolytic anemias in adults. (http://www.ncbi.nlm.nih.gov/pubmed/20548093) Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. (http://www.ncbi.nlm.nih.gov/pubmed/22330255)

A 37-Year-Old Woman with Known Systemic Lupus Erythematosus Is Evaluated