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Background: Chronic lymphocytic leukemia (CLL) is a neoplasm composed of monomorphic small mature B cells that coexpress CD5 and CD23. The finding of ≥55% prolymphocytes defines B-cell prolymphocytic leukemia (B-PLL), and cases with 15-55% of the prolymphocytes called atypical CLL (aCLL, previously called CLL/PL). Aims: The aim of this study was evaluation of the clinical and prognostic significance of aCLL. Methods: We reviewed the medical records, peripheral blood, and bone marrow findings of 121 patients with untreated CLL (n = 101), aCLL (n = 9), and B-PLL (n = 11) between January 1995 and June 2018. CLL and aCLL patients were classified as Binet stage A (<3 areas of lymphadenopathy, hemoglobin >10 g/dL, platelets >100k/mL), B (≥3 areas of lymphadenopathy, hemoglobin >10 g/dL, platelets >100 k/mL), or C (hemoglobin <10 g/dL or platelets <100k/mL). All patients underwent immunohistochemistry and/or flow cytometric immunophenotyping, among them; 107 patients underwent karyotyping, and 15 patients underwent fluorescent in situ hybridization. Results:The median age at diagnosis was 63.5 (range 25-85) years, 68.0 (40-77) years, and 66.0 (29-78) years and the ratio of males to females was 2.0, 2.0, and 1.8 in CLL, aCLL, and B-PLL, respectively. Lymphadenopathy was more common in CLL (42%, 42/101) and aCLL (56%, 5/9) than in B-PLL (0%), whereas splenomegaly was more in B-PLL (100%) than CLL (25%, 25/101) and aCLL (33%, 3/9). (P = 0.683 and 0.010, respectively). aCLL showed more severe anemia, elevated lactate dehydrogenase, and b2-microglobulin than CLL and B-PLL (P = 0.001, 0.027, and 0.037, respectively). Binet stage A and B were more in CLL (51% and 26%) than in aCLL (30% and 0%), whereas Binet stage C was more in aCLL (70%) than CLL (23%). (P = 0.013). Patients with B-PLL had an atypical immunophenotype with high frequencies of CD5 or CD23 negativity, FMC7 positivity, and strong CD22 positivity (P = 0.672, 0.440, 0.004, and <0.001, respectively). Especially in this study, patients with aCLL showed higher frequencies of FMC7 positivity and strong CD22 positivity than CLL in Western study (P = 0.032 and <0.001, respectively). In B-PLL, normal karyotype was less common and complex karyotype was more common than CLL and aCLL (P = 0.028). In the CLL group, cytogenetic abnormalities were observed in 35% of patients (33/94). The descending order of frequency was trisomy 12 (11%, 10/94), 13q deletion (10%, 9/94), complex karyotype (7%, 7/94), 11q deletion (5%, 5/94), 14q deletion (2%, 2/94), and 17p deletion (1%, 1/94). In the aCLL group, cytogenetic abnormalities were present in 50% of patients (4/8), including 3 cases of trisomy 12, 2 cases of 14q deletion, 1 case of 13q deletion, 11q deletion, 17q deletion and complex karyotype. In the B-PLL group, cytogenetic abnormalities were observed in 80% of patients (8/10), of whom 4 had complex karyotypes. The overall survival rate at 10 years were 65.6%, 22.2%, and 46.3 % in patient with CLL, aCLL, and B-PLL, respectively (P = 0.155). However, only OS of CLL and aCLL showed ...

Objective. To present the immediate results of the splenectomy and preferable variants of transfusion therapy performance in patients with malignant non-Hodgkin’s lymphoma (MNHL). Materials and methods. 109 splenectomies were performed in patients with MNHL at the Department of General and Hematological Surgery of the institute from 1987 to 2020. The surgery was conducted by upper middle laparotomy under general anesthesia with intubation and, in particular cases, under spinal anesthesia. Results and discussion. The indications for splenectomy in patients with MNHL were as follows: massive splenomegaly, abdominal syndrome, associated hemocytopenia, inefficacy of cytostatic therapy, absence of diagnosis. All patients underwent vaccination against capsular bacteria for prevention of post-splenectomy infection in 10-14 days prior to the surgery. In case of anemia, which has been observed in 55 % of patients, the RBC concentrate was applied. All the patients, who received corticosteroid hormones prior to splenectomy, were administered prednisolone and hydrocortisone in the amount of 3 mg/kg of body mass at similar doses in an hour before the surgery for prevention of adrenal insufficiency during the surgery. The patients, who did not receive those medications, were also intramuscularly administered prednisolone at a dose of 0.5 mg/kg of body mass in an hour before the surgery for the same aim. The patients with PLT value <150.0×109/L were administered 1-2 doses of PLT concentrate immediately before the laparotomy. M-gradient was found in blood serum of 3 patients prior to the surgery. They underwent courses of therapeutic plasmapheresis due to the risk of intraoperative hemorrhage. 2 patients with hyperleukocytosis (WBC >80.0×109/L) underwent two courses of leukapheresis. The patients with concomitant regional portal hypertension and in case of manipulations close to the pancreatic tail were administered somatostatin drugs in the course of the splenectomy. The splenectomy proved to be effective in 100 (92 %) of patients with MNHL: the great tumor mass was removed, the abdominal syndrome and concomitant hemocytopenia were neutralized, the signs of hypersplenism ceased, the hemolysis ceased, the cytostatic therapy became less necessary or unnecessary, the final diagnosis was established. The most serious postsurgical complications were acute adrenal failure (n=3), postsurgical intra-abdominal hemorrhage (n=2), pancreonecrosis (n=6). The postsurgical lethality was 2.7 %. Conclusions. The splenectomy proved to be effective in 92 % of patients with MNHL. The infusion therapy is individual for each patient and may include transfusion surgeries if indicated. The main objective of the infusion therapy in patients with MNHL is prevention and elimination of intra- and postsurgical complications.

Summary. The aim of the study was to determine peculiarities of the distribution, diagnosis and development of immune cytopenias in patients with chronic lymphocytic leukemia (CLL) and to evaluate the efficacy of the different therapeutic approaches. Materials and Methods: Treatment response and survival of 83 patients with CLL complicated by immune cytopenia (IC) were analyzed. Treatment schedules in 58 medicated patients included corticosteroids; chemotherapy (COP, CHOP regimens), immunotherapy (rituximab alone), immunochemotherapy (rituximab-containing regimens — R-COP, R-CHOP). Twenty-five patients underwent splenectomy. Results: The use of corticosteroids, as the first line of treatment, resulted in short-term remission in most patients. Chemotherapy was effective in a half of CLL patients, but duration of the remission did not exceed 32 months in CLL associated with autoimmune hemolytic anemia and immune thrombocytopenia. After rituximab monotherapy (10 patients) the stable remission was reached in 60% of the patients with median relapse-free survival of 40 months. Rituximab containing chemotherapy (22 patients) caused the long-term remission in 72% of the patients with median relapse-free survival of 76 months. Splenectomy performed in 25 patients with CLL complicated by IC was effective in 70% of the patients. The outcome of splenectomy depends on IC entity. The best response was registered in associated immune thrombocytopenia (median overall survival 118 months), the worst — in Fisher — Evans syndrome (15 months). Conclusions: The treatment of patients with CLL complicated by ICs should be individualized. For CLL patients without significant enlargement of lymph nodes and spleen, low lymphocytosis, associated with autoimmune hemolytic anemia or immune thrombocytopenia, the monotherapy with rituximab is optimal. In case of occurrence of autoimmune hemolytic anemia, immune thrombocytopenia or Fisher — Evans syndrome in CLL patients with enlargement of lymph nodes, spleen, significant lymphocytosis, the use of R-COP or R-CHOP schemes, 4–6 courses, is the most effective. Splenectomy is indicated in patients with massive splenomegaly, the resistance to medication, recurrent relapses after adequate therapy.

Державна установа «Інститут патології крові та трансфузійної медицини НАМН України», Львів, Україна Реферат Актуальність. Лімфома з клітин мантії (MCL) -В-клітинна неоплазія, яка характеризується хромосомною транслокацією t(11;14)(q13;q32), з агресивним клінічним перебігом, поганою відповіддю на терапію. При масивній спленомегалії та резистентних до лікування імунних цитопеніях виникає питання про доцільність проведення спленектомії при цій лімфомі. Метою роботи було оцінити ефективність спленектомії у хворих на MCL щодо симптомів, пов'язаних із спленомегалією, та цитопеній. Матеріали і методи. Спленектомію виконано у 4 хворих на MCL. Результати. У всіх хворих виявлено лімфаденопатію і масивну спленомегалію, регіональну портальну гіпертензію, залучення кісткового мозку, підвищену (≥10,0×10 9 /л) кількість лімфоїдних клітин у периферичній крові з імунофенотипом CD19 + CD5 + CD22 + CD23 -. Цитогенетичне дослідження лімфоїдних клітин одного хворого показало наявність типової для MCL t(11;14)(q13;q32). У всіх хворих діагностували ІV стадію лімфоми. MCL у трьох пацієнтів асоціювалась з імунними цитопеніями. Операція супроводжувалась значною кровоточивістю тканин та крововтратою (≈500 мл). В результаті спленектомії усунено абдомінальний дискомфорт, видалено велику масу пухлини, припинився гемоліз, ліквідовано анемію, нормалізувалось число тромбоцитів, гістологічно уточнено варіант лімфоми. Курси цитостатичної терапії після спленектомії виявилась значно ефективнішими. У жодного пацієнта не спостерігали рецидиву гемолізу та/або тромбоцитопенії. Пацієнти після операції прожили 49, 70, 140 та 158 міс. Висновки. Спленектомія виявилася методом, придатним у лікуванні MCL з масивною спленомегалією для корекції абдомінальних симптомів, імунних цитопеній та підвищення ефективності цитостатичної терапії. Ці результати свідчать про біологічну роль селезінки у патогенезі форми MCL з лейкемізацією і спленомегалією. Ключові слова: лімфома з клітин мантії; спленомегалія; імунна тромбоцитопенія; синдром Івенса-Фішера; спленектомія; виживання. Abstract Background. Mantle cell lymphoma (MCL) is a B-cell neoplasia characterized by chromosomal translocation t (11; 14) (q13; q32), with an aggressive clinical course, poor response to therapy. In the case of massive splenomegaly and resistant immune cytopenias, the question arises if splenectomy can be used as a treatment for the lymphoma. Aim of this study was to define the efficacy of splenectomy in patients with MCL with regard to symptoms of splenomegaly and cytopenias.Materials and methods. Four patients with MCL underwent splenectomy. Results. All patients revealed lymphadenopathy and massive splenomegaly, regional portal hypertension, bone marrow involvement, increased (≥10. 0×10 9 /l) count of lymphoid cells in peripheral blood with immunophenotype CD19 + CD5 + CD22 + CD23 -. Cytogenetic examination of lymphoid cells of one patient revealed the presence of typical for MCL t(11;14)(q13;q32). All patients were diagnosed with lymphoma, stage IV. Three patients had MCL associated with imm...