Onkológia 5/2018
Variant hairy cell leukemia in complete remision after treatment of rituximab and fludarabine – case report
Introduction: Hairy cell leukemia (HCL) is a rare chronic indolent lymphoproliferative disease. Diagnostically relevant is presence of circulating B-lymphocytes drained from the bone marrow and prominent hair-like projections of the cytoplasm on their surface visible by light and electron microscopy. Accurate treatment of the classic type (cHCL) has a good prognosis with long-term survival, responds excellently to standard purine analogue treatment (one cycle of 2-CdA as first-line treatment: CR 95 %, PR 5 %, 2-CdA as second-line treatment CR 75 %). The variant form (vHCL) represents 10-20% of all HCL cases. It’s prognosis is unfavorable, tends to be associated with a more aggressive disease course and resistance to standard treatment 2-CdA (cladribine), 2-dCF (pentostatin, unregistered in SK), and interferon-α (IFN-α). Purpose: Our role, while exactly determining the variant subtype HCL by immunohistochemistry, flow cytometry and cytogenetics, was to provide the best response with second-line treatment for vHCL, known for it’s resistance to standard chemotherapy 2-CdA (BRAF wild type without mutation, conversion of IgHV4-34 (14q32.33) and an adverse prognosis with a short time to progression. Case: Our patient with variant HCL achieved a complete remission with the disappearance of bone marrow infiltration (BM) by the combination of rituximab and fludarabine. Complete remission currently takes 36 months after ending the treatment, with no laboratory or clinical signs of reactivation of leukemia. Conclusion: In cases of variant HCL diagnosis, we confirmed the combination of the monoclonal antibody rituximab and purine analog fludarabine to be an effective treatment. Standard treatment with the cladribine (2-CdA) as the first line was proven to be unnecessary with a partial response to blood counts but with a persistent bone marrow infiltration in the unchanged range. Therefore, in the cases of variant HCL, the use of the combination of rituximab and fludarabine in the first line may replace cladribine (2-CdA), which is only effective for the classic HCL.
Keywords: variant hairy cell leukemia, rituximab, fludarabine
