2008
DOI: 10.1159/000115785
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Effective Treatment of a Refractory Hairy Cell Leukemia Variant with Splenic Pre-Irradiation and Alemtuzumab

Abstract: A 72-year-old Japanese man presented with 43.1 × 109/l hairy cells and apparent splenomegaly. The leukemia cells had unevenly distributed microvilli and round nuclei with dense chromatin and one or two clear nucleoli, lacked CD25 expression and were negative for tartrate-resistant acid phosphatase. The case was diagnosed as hairy cell leukemia variant (HCLv) and proved refractory to various chemotherapies, including cladribine, pentostatin, interferon-α, CHOP and rituximab. Because of the CD52 expre… Show more

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Cited by 17 publications
(11 citation statements)
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“…Rituximab alone or combined with other modalities was reported to induce complete or partial remission in several case reports [30,31,32]. Sasaki et al [33] reported an HCL-V patient who had partial remission after treatment with alemtuzumab following splenic irradiation. Complete remission after treatment with BL-22 (anti-CD22 immunotoxin) was reported in 3 patients refractory to 2-CDA by Kreitman et al [34].…”
Section: Discussionmentioning
confidence: 99%
“…Rituximab alone or combined with other modalities was reported to induce complete or partial remission in several case reports [30,31,32]. Sasaki et al [33] reported an HCL-V patient who had partial remission after treatment with alemtuzumab following splenic irradiation. Complete remission after treatment with BL-22 (anti-CD22 immunotoxin) was reported in 3 patients refractory to 2-CDA by Kreitman et al [34].…”
Section: Discussionmentioning
confidence: 99%
“…Alemtuzumab is a fully humanized monoclonal antibody against CD 52. Although attempts to treat HCL using anti-CD52 revealed limited success, there has been better response with treating refractory cases [16]. Its toxicity includes infusion reactions similar to rituximab and prolonged immunosuppression, leading to opportunistic infections.…”
Section: Anti-cd52 Antibody: Alemtuzumabmentioning
confidence: 99%
“…In the largest study on treatment in HCL-variant, splenectomy yielded clinical responses in 74% of patients (13/19) lasting for greater than 8 years in a proportion of patients [53]. An alternative approach in patients with surgical risk and poor performance status is splenic irradiation which may result in PR in around half of the patients [53,55] or may facilitate further treatment [56]. IFN-α has no role in the therapeutic scenario of HCL-variant as nearly all patients will be resistant to this drug unlike typical HCL [57].…”
Section: Treatmentmentioning
confidence: 99%
“…Alemtuzumab, the humanised anti-CD52 McAb is an attractive agent to be used in this disease on the basis that its activity is mainly seen in the spleen and bone marrow and it has been shown to be effective in CLL with TP53 abnormalities. So far, two cases have been reported successfully treated with either alemtuzumab as single agent or using alemtuzumab after splenic irradiation [56,63]. Further data on the use of alemtuzumab in HCLvariant is needed to confirm its activity.…”
Section: Treatmentmentioning
confidence: 99%