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nephritis. Plasmapheresis has been used in the treatment of severe lupus nephritis, but
its use should be limited to centers experienced in the care of acutely ill children with
SLE.
Hematologic Complications. Anemia is common in SLE and may have many causes.
Most typically, patients have a nonspecific normocytic, normochromic anemia of
chronic disease. Microcytic anemia, in contrast, may be a sign of GI blood loss caused
by vasculitis or gastritis, with the urgency of further investigation dependent on the
severity of the bleeding and the patient’s overall well-being. Hemolytic anemia in SLE
may be related to the disease itself (anti-erythrocyte antibodies) or to medications. The
antibody responsible for autoimmune hemolytic anemia may not be demonstrated by
the standard Coombs test, so more sensitive assays may be necessary.
Management of anemia depends on the severity (see Chapter 93 Hematologic
Emergencies ). Corticosteroids are the most effective agents for the control of
autoimmune hemolytic anemia in SLE. Prednisone at 2 mg/kg/day is the initial
treatment of choice. For severe hemolytic anemia requiring transfusion, additional
immune modulatory therapies may be required. Consultation with a hematologist and
rheumatologist is recommended.
Leukopenia occurs in about 50% of patients with SLE. It may be caused by a
reduction in granulocytes, lymphocytes, or both. Granulocytopenia may be caused by
drugs used in the treatment of SLE or less commonly by disease-related destruction.
Febrile children with absolute granulocyte counts of <1,000/mm3 are at higher risk of
severe infections and should be admitted for empiric antibiotic coverage pending
results of further studies.
Thrombocytopenia occurs in approximately 25% of patients with SLE. More than
5% of children with an initial diagnosis of ITP eventually meet diagnostic criteria for
SLE. The usual causes of thrombocytopenia are circulating antibodies to platelets or
drug-induced bone marrow suppression. Infection should always be considered as a
possible cause, so the presence of purpura and ecchymoses requires immediate
investigation. Significant hemorrhage, a sudden drop in hemoglobin, and platelet
counts of <20,000/mm3 are the usual indications for admission to the hospital.
Diagnostic testing should include CBC, examination of the peripheral blood smear, and

appropriate cultures. At times, bone marrow examination and testing of serum for antiplatelet antibodies may be helpful in determining the cause of thrombocytopenia.
Patients with SLE are at risk of bleeding from any mucosal surface because of
vasculitic ulceration, impaired hemostasis, thrombocytopenia, or a combination of
these factors. Patients with life-threatening epistaxis may require local packing and
platelet replacement in addition to high-dose corticosteroids. Severe pulmonary
hemorrhage may necessitate transfusions, ventilatory assistance, and bronchial lavage.
Once infections have been excluded, the underlying condition is treated with high-dose