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Blood, Vol. 95 No. 9 (May 1), 2000:
pp. 2786-2792
Autoimmune hemolytic anemia in chronic lymphocytic leukemia:
clinical, therapeutic, and prognostic features
Francesca R. Mauro,
Robert Foa,
Raffaella Cerretti,
Diana Giannarelli,
Serelina Coluzzi,
Franco Mandelli, and
Gabriella Girelli
Dipartimento di Biotecnologie Cellulari ed Ematologia and
Dipartimento di Medicina Sperimentale, University "La Sapienza,"
Rome, Italy.
Fifty-two cases of autoimmune hemolytic anemia (AHA) were observed
within a series of 1203 patients (4.3%) with chronic lymphocytic leukemia (CLL) followed at a single institution. Nineteen were observed
at the time of CLL diagnosis and 33 during the clinical follow-up.
Ninety percent of the patients with CLL/AHA showed active CLL and 25%
had been treated previously. The antierythrocyte autoantibody (AeAb)
was an IgG in 87% of cases and an IgM in 13%. A lymphocyte count more
than 60 × 109/L (P < .00001), age above 65 years (P < .01), and male gender (P < .01)
emerged as independent parameters that correlated significantly with an
increased rate of AHA at CLL diagnosis. Patients previously treated
with chlorambucil (CB) plus prednisone (PDN) and with fludarabine plus
PDN showed a similar rate of AHA (1.8% and 2.5%, respectively). After
steroid therapy associated with CB in case of active CLL, 70% of
patients achieved the complete disappearance of the AeAb. The actuarial
AHA relapse-free survival probability was 54% at 5 years and the
median survival probability after AHA was 41 months. Infections
represented the main cause of morbidity and mortality. IgG AHA and the
occurrence of AHA at the same time of CLL diagnosis emerged as
independent factors significantly correlated with a better survival
probability of AHA/CLL patients. Taken together, this study indicates
that in CLL, AHA is a rare event with no independent effect on survival
for which steroids, associated with CB if required, and a careful
management of infections may successfully control the 2 conditions.
Cooperative studies are needed to better define the optimal steroid
schedule and the therapeutic role of other immunosuppressive agents and splenectomy.
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