Author: A. Rajaminackam, M.D., Department of Hospital Medicine, Cleveland Clinic
Reviewer: V. Dimov, M.D.
A 51-year-old female has had fatigue, weakness, and shortness of breath (SOB) with exertion during the past 4-5 days. She called her primary care physician (PCP) who recommended that she had her hemoglobin checked. He called her back with the results, and told her to go to the emergency room (ER) for further treatment of severe anemia. On admission, the patient denied abdominal pain, chest pain, congestion, nausea/vomiting/diarrhea/constipation (N/V/D/C), dysuria, headache, chills, hemoptysis, neck pain, rash, or sore throat.
Her symptoms were exacerbated by activity and relieved by rest and laying supine. She also felt palpitations intermittently.
Past Medical History (PMH)
Diabetes type 2 (DM2).
Lantus (Insulin glargine, rDNA origin) 25 mg SQ QHS, Humalog (insulin lispro) SSI SQ with Accu-Chek Blood Glucose Monitoring TID.
VS: mild tachycardia, no hypotension.
General appearance: pale, non-icteric.
Eyes: EOMI, PERRLA, sclerae non-icteric.
ENT: Oropharynx clear, no plaques or exudates.
Chest: CTA (B).
CVS: Clear S1S2.
Abd: Soft, NT, ND, +BS.
Ext.: no cyanosis/clubbing/edema (c/c/e).
Neurologic: AAA x 3.
What is the most likely diagnosis?
Severe anemia that is symptomatic with fatigue and shortness of breath (SOB).
What are the most likely causes of anemia in this patient?
What laboratory workup would you order?
The hemoglobin (Hgb) was 4.2 mg/dL, MCV 144 fl, and reticulocyte count 41%. The patient most likely has hemolytic anemia.
What other tests would you order?
Direct and indirect Coombs' test
Chest X-ray (CXR)
CT of the chest, abdomen and pelvis (CT c/a/p)
CBC and CMP (click to enlarge the images).
Immunology tests (click to enlarge the images)
What happened next?
The patient was admitted to a regular medical floor and a hematology consult was called. The direct Coombs' test was reported as positive.
The CXR and CT scans were negative for neoplastic disease.
The patients has autoimmune hemolytic anemia (AIHA) mediated by warm antibodies because the hemolysis is observed at normal body temperature. By contrast, in the cold antibody AIHA, the autoantibodies attack the red blood cells only at temperatures significantly below normal body temperature, e.g. when working outside in the winter.
Would you transfuse this patient?
The hemoglobin was 4.2 mg/dL and if the patient was symptomatic. A blood transfusion was indicated.
In general, it may be difficult to find compatible blood in AIHA because of the presence of autoantibodies. RBC transfusions are generally avoided unless absolutely necessary.
How would you treat this patient?
Solu-Medrol (methylprednisolone) 100 mg IV q 6 hr.
Consider immune globulin infusion.
Follow-up on the Hem/Onc recommendations.
Hemoglobin response to steroid treatment in AIHA. Taper glucocorticoids very gradually to avoid a relapse of hemolysis (click to enlarge the images).
Warm Antibody Autoimmune Hemolytic Anemia (AIHA).
Coombs' test (click to enlarge the diagram). Source: A. Rad. GNU Free Documentation License. Wikipedia.
Hemolytic Anemia. eMedicine.
Autoimmune Hemolytic Anemia. Merck Manual.
Seasonal Hemolysis Due to Cold-Agglutinin Syndrome. Lyckholm L. J., Edmond M. B.
N Engl J Med 1996; 334:437, Feb 15, 1996. Images in Clinical Medicine.
Robin Coombs, 85, Inventor of a Diagnostic Blood Test, Dies. The New York Times, March 27, 2006