Leukemia & Lymphoma, June 2011; 52(S2): 50–52
Infectious complications in hairy cell leukemia
ERIC KRAUT Department of Internal Medicine, The Arthur G. James and Richard Solove Cancer Hospital and Research Institute, The Ohio State University, Columbus, Ohio, USA
Abstract The natural history of hairy cell leukemia (HCL) includes frequent and potentially life-threatening infections. Prior to the development of effective therapy, the incidence in patients followed for several years was as high as60%,with infection as a prime cause of death in patients. Studies of the immune system of patients with HCL identified several potential reasons, including profound neutropenia and monocytopenia. In addition, treatment including chemotherapy and splenectomy further compromised the immune system. The success of new therapies has changed the frequency and severity of infections in patients withHCL.Duringthe initial phase of treatment, however, infection risk remains high, with incidence ranging from30to
50%.Attempts to ameliorate the risk with growth factors in conjunction with treatment have not been successful, but lower doses of drugs and/or combination therapy have been tried with reported success. In the majority of patients, successful therapy results in normalization of the neutrophil count and marked reduction in the severity and frequency of infections. Interestingly, after
purine nucleoside treatment, there is profound depression of CD4þ cells without development of the opportunistic infections seen with patients withhumanimmunodeficiency virus (HIV). Studies to reduce morbidity and mortality should focus on initial induction regimens, as well as confirming the long-term benefit of treatment on risk of infection.
Keywords: Hairy cell leukemia, infections, complications
One of the most recognized and important clinical problems in patients with hairy cell leukemia (HCL) is the development of severe life-threatening and unusual infections [1–3]. Prior to the introduction of interferon and purine nucleosides, these infectious complications were a common part of the natural history of the disease. These often involved both gram-positive and gram-negative organisms as well as Aspergillus and other fungi [1–3]. Clinical reviews of patients with HCL documented both the frequency and severity of this complication. Golde et al. [3] evaluated 20 patients with HCL and noted 13 episodes of severe infection in eight patients apparently related to marked neutropenia. Golomb and Hadad [1] studied 127 patients, and found 47 (37%) with documented infections and an additional 40 (31%) with presumed infections. Twenty-nine of the 47 patients died as a result of these infections. An additional series of 22 patients identified 18 life- threatening infections [2].
In order to explain the frequency and severity of these
infections there have been several investigations of the immune system in patients with HCL. In addition to neutropenia, patients with HCL have reductions in monocytes, dendritic cells, and natural killer cells and an alteration in host defense associated with splenect- omy [4]. Although it is unclear which of these defects is most important, the introduction of effective therapy has made a major impact on both the presence of immune defects and also the frequency of infection. There is now evidence of a reduced incidence of
infections over the course of a patient’s disease that correlates with the response to therapy and has led to improvement in survival. However, infectious risk is still high during the early period of treatment, no matter what therapy is being used (Table I). In five series involving 649 patients treated with pentostatin as initial treatment [5–9] for HCL, with most patients with neutropenia, 25% of the patients had a documented infection and/or neutropenic fever. Not all patients were treated with antibiotics.
Correspondence: Prof. Eric Kraut, MD, Department of Internal Medicine, The Arthur G. James and Richard Solove Cancer Hospital and Research Institute, The Ohio State University, Columbus, Ohio, USA. E-mail:
Eric.Kraut@
osumc.edu
ISSN 1042-8194 print/ISSN 1029-2403 online 2011 Informa UK, Ltd. DOI: 10.3109/10428194.2011.570819
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