
PB1897 MANAGEMENT OF AUTOIMMUNE CYTOPENIA COMPLICATING CHRONIC LYMPHOCYTIC LEUKEMIA
Author(s) -
Vyhovska O.,
Yevstakhevych Y.,
Maslyak Z.,
Yevstakhevych I.,
Pelenyo N.,
Semerak M.,
Vyhovska Y.
Publication year - 2019
Publication title -
hemasphere
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 11
ISSN - 2572-9241
DOI - 10.1097/01.hs9.0000566092.00935.f1
Subject(s) - medicine , cytopenia , rituximab , evans syndrome , autoimmune hemolytic anemia , gastroenterology , chronic lymphocytic leukemia , neutropenia , pure red cell aplasia , anemia , lymphoma , leukemia , chemotherapy , bone marrow
Background: Chronic lymphocytic leukemia (CLL) in 4.3‐9.7% of patients is complicated with immune cytopenia (IC). Timely diagnostics of IC is important, as the therapeutic tactics in patients with IC is significantly different from standards of treatment of patients with CLL without immune complications. Aims: Determination of the peculiarities of the course of IC in patients with CLL and assessment of different treatment regimens. Methods: Under our observation, there were 83 patients with CLL complicated with IC: 38 patients with autoimmune hemolytic anemia (AIHA), 26 patients with immune thrombocytopenia (ITP), 15 patients with Fischer‐Evans syndrome, 3 patients with partial red cell aplasia, one patient with immune neutropenia. The prognostic markers of the disease were determined: expression of CD38, ZAP70, β2 MG level, Coombs’ test, FISH analysis (del11q 22.3 and del17p 13.1). 58 patients received drug therapy (the first line ‐ steroid hormones, further: the schemes of COP, CHOP or RCOP, RCHOP or monotherapy with rituximab (rituximab at a dose of 375 mg / m2 / day, 4 injections with a weekly break), 25 patients underwent splenectomy. Results: The most severe course of the disease was observed in patients complicated with Fischer‐Evans syndrome (the median overall survival of 75 months). Insignificantly better results were observed in patients with AIHA (the median overall survival of 78 months). In patients with ITP, the median overall survival has not been achieved (observation time 12‐133 months). In case of steroid hormones use, as the first line of treatment, most patients experienced short‐term remissions.When using monotherapy with rituximab, the median progression‐free was 20 months. Conducted course therapy, based on RCOP or RCHOP regimens, was effective in 82% of patients. The median progression‐free survival has not been achieved in patients of this group (observation time 12‐57 months). The median of overall survival for CLL with ITP after splenectomy was 135.0 months, for CLL with AIHA it was 69.6 months, and for CLL complicated with Fischer‐Evans syndrome it was only 49.5 months. Summary/Conclusion: Treatment of patients with CLL complicated with immune cytopenias should be individualized: 1. For patients with CLL that runs without significant enlargement of lymph nodes and spleen, low lymphocytosis, in case of occurrence of AIHA or ITP the use of monotherapy with rituximabis is optimal. 2. In case of occurrence of AIHA, ITP or Fisher‐Evans syndrome in patients with CLL that runs with enlargement of lymph nodes, spleen, significant lymphocytosis, the use of R‐COP or R‐CHOP schemes, 4‐6 courses, is the most effective. 3. In case of resistance to drug therapy and the presence of splenomegaly in patients with CLL, complicated with IC, splenectomy is indicated.