Autoimmune Hemolytic Anemia

Anemia with Hemoglobin 4.2 mg/dL. What is the Cause?

Author: A. Rajaminackam, M.D., Department of Hospital Medicine at Cleveland Clinic
Reviewer: V. Dimov, M.D.
A 51-year-old female has had fatigue, weakness, and SOB with exertion during the past
4-5 days. She called her PCP who recommended she had hemoglobin checked. He called
her back with the results, and told her to go to the ER for further treatment of severe
anemia. The patient denied abdominal pain, chest pain, congestion, N/V/D/C, dysuria,
headache, chills, hemoptysis, neck pain, rash, or sore throat.
Symptoms were exacerbated by activity and relieved by rest and laying supine. She also
felt palpitations intermittently.
Past Medical History (PMH)
DM2.
Medications
Lantus (Insulin Glargine, rDNA origin) 25 mg SQ QHS, Humalog (insulin lispro) SSI SQ
with Accu-Chek Blood Glucose Monitoring TID.
Physical Examination
VS: mild tachycardia, no hypotension.
General appearance: pale, non-icteric.
Eyes: EOMI, PERRLA, sclerae non-icteric Definition: When a physician or nurse is
examining a patient's eyes, PERRLA is an acronym used to describe the look and
function of the eyes. It stands for:
Pupils
Equal
Round
Reactive to
Light and
Accommodation (which means the ability of the eyes to focus on objects that are
close up and faraway)
An eye exam is done to check how someone's nervous system is functioning, especially
after a head injury or during serious illness. An eye exam would be abnormal if the pupils
are:
not of equal size

not round (misshapen)

don't change when a light is shined on them (should get smaller)
don't change when looking at something close or faraway

EOMI
Extraocular Movements Intact

ENT: Oropharynx clear, no plaques or exudates

Chest: CTA (B)
CVS: Clear S1S2
Abd: Soft, NT, ND, +BS
Ext.: no c/c/e
Neurologic: AAA x 3
No lymphadenopathy
What is the most likely diagnosis?
Severe anemia that is symptomatic with fatigue and shortness of breath (SOB).
What are the likely causes of anemia in this patient?
Blood loss?
Hemolytic anemia?
Iron-deficiency?
What laboratory workup would you order?
CBC+Diff
CMP
Reticulocyte count
LDH
Haptoglobin
Peripheral smear
What happened?
Hemoglobin (Hgb) was 4.2 mg/dL, MCV 144 fl, reticulocyte count 41%. The patient
most likely has hemolytic anemia.
What other tests would you order?
Direct and indirect Coombs' test
ANA, RF

C3, C4
CXR
CT c/a/p (chest, abdomen and pelvis)

CBC and CMP

Immunology tests
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.
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.

Final diagnosis
Warm Antibody Autoimmune Hemolytic Anemia (AIHA)
References

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.