[Primary mediastinal large B-cell lymphoma in pregnant women].

AIM: To elaborate a management tactic for pregnant women with primary mediastinal large B-cell lymphoma (PMLBL) and to assess the toxicity of its treatment to the mother and fetus. SUBJECTS AND METHODS: In 2004 to 2014, the Hematology Research Center, Ministry of Health of Russia, treated 94 patients with mediastinal large B-cell lymphoma, 7 (7.4%) of them developed the disease during pregnancy. Induction therapy was performed according to the VACOP-B or R-EPOCH program. For consolidation, polychemotherapy (PCT) was made after 3-4 weeks postpartum in accordance with the R+Dexa-BEAM program, followed by radiotherapy (RT) applied to a residual mediastinal mass in a total focal dose of 36 Gy. To assess the nature of the residual mass, positron emission tomography was carried out 1 month following the induction and consolidation cycles of PCT. RESULTS: PCT was performed in 5 and 2 of the 7 patients diagnosed with PMLBCL in the second and third trimesters according to the VACOP-B and R-EPOCH programs, respectively; for consolidation, PCT was done using the R+Dexa-BEAM regimen in 7 patients: 10 men and 29 women whose ages were 18 to 60 years (median age 30 years); in 5 of the 7 patients, RT was applied to the residual mediastinal region in a total focal dose of 36 Gy. After induction treatment, 4 of 5 the patients in the VACOP-B group achieved partial remission; one of the 5 patients was stated to have disease progression. In the R-EPOCH group, 2 of the 2 patients achieved partial remission. After performing the treatment protocol, an early recurrence was recorded in 1 of the 5 cases in the VACOP-B/Dexa-BEAM/RT group. Effective autologous stem cell transplantation was carried out in patients with disease progression and early recurrence. Seven children (3 boys and 4 girls) were born. Their median body weight was 2182 g (1700 to 3600 g); the median height was 47 cm (40 to 53 cm). Two neonatal infants born to women who had received CT using the R-EPOCH regimen were diagnosed as having intrauterine pneumonia resulting from respiratory distress syndrome, which might be associated with fetal prematurity and the use of rituximab. One baby born to a patient who had been included in the VACOP-B treatment protocol was stated to have superior vena cava at birth. The median follow-up of the patients and born infants was 35 months (15 to 64 months). CONCLUSION: Due to the elaborated algorithm for the treatment and management of pregnant women, all the patients are alive without tumor signs and their babies are healthy without signs of development defects and retardation.

[Experience with high-dose chemotherapy in patients with testicular diffuse large B-cell lymphoma].

AIM: To evaluate the efficiency of high-dose therapy according to the DLBL-CNS-2007 protocol in patients with testicular diffuse large B-cell lymphoma (DLBL). SUBJECTS AND METHODS: Out of 408 male patients with non-Hodgkin lymphoma, 8 patients aged 50 to 69 years (median age 55.5 years) with primary testicular (n=3) or with generalized-stage testicular DLBL (n=5) were included in the study. These patients were followed up at the Hematology Research Center, Ministry of Health of the Russian Federation, in 2007 to 2013. Systemic chemotherapy was performed in accordance with the DLBL-CNS-2007 protocol. RESULTS: The DLBL-CNS-2007 protocol was implemented in first-line therapy in 7 patients. At the first diagnostic stage, one patient was found to have anaplastic seminoma; in this connection right orchifuniculectomy was carried out, followed by radiotherapy applied to the scrotal region in a total focal dose of 34 Gy. This patient with disease recurrence was included in the DLBL-CNS-2007 treatment protocol. The number of polychemotherapy (PCT) cycles (n=4 or 6) was determined by the time to achieve complete remission. After completion of DLBL-CNS-2007 PCT, 6 patients achieved complete remission; the primary resistant disease was noted in 2 cases. At this moment 6 patients are alive in first complete remission during the median follow-up of 50 months (10-54 months). CONCLUSION: The findings suggest that high-dose therapy according to the DLBL-CNS-2007 protocol in patients with testicular DLBL can achieve complete remission and increase overall and event-free survival rates. This fact should be borne out by a large number of observations.

[Myeloid sarcoma of the small bowel with inversion of chromosome 16: a description of 3 clinical cases].

Myeloid sarcoma (MS) is a rare malignant solid tumor presented with myeloid blast cells showing varying degrees of maturation. MS may have an extramedullary site, precede, or develop simultaneously with the clinical manifestations of acute myeloid leukemia (AML); it may also occur as an AML relapse. Besides AML, MS may be a manifestation of chronic myeloid leukemia or other chronic myeloproliferative diseases. Due to the fact that this disease is rare, the bulk of the literature on MS is presented with single descriptions of retrospective studies and clinical cases. The paper describes 3 cases of MS with inversion of chromosome 16 and small bowel lesion.

[Successful treatment of a patient with two hematologic tumors: double-hit lymphoma and acute myelomonoblastic leukemia].

Double-hit (DH) lymphoma, an extremely aggressive variant of B-cell lymphoma, is accompanied by chromosomal abnormalities leading to the activation of a few oncogenes, one of which is the c-MYC gene in conjunction with BCL2 or BCL6 gene rearrangements. There are most common cases of MYC/8q24 and BCL2/18q21 gene rearrangements (MYC/BCL-2 DH lymphoma). The tumor is characterized by an aggressive clinical course and a poor response to chemotherapy (CT). The median survival in patients with DH lymphomas varies from 4.5 to 18 months. Such patients are generally resistant to CHOP-21 and R-CHOP-21 therapy regimens. For the treatment of patients with DH lymphoma, the Hematology Research Center, Ministry of Health of the Russian Federation, chose an original BL-M-04 polychemotherapy (PCT) protocol in combination with rituximab, followed by autologous stem cell transplantation (allo-SCT). The paper describes the experience in successfully treating a patient with two hematologic tumors: 1) MYC/BCL-2 DH lymphoma with high-dose PCT cycles, followed by allo-SCT, and 2) a metachronously developed second tumor (acute myelomonoblastic leukemia (AMML)) with CT cycles, followed by auto-SCT. The incidence of tumors induced by the previous high-dose CT for aggressive lymphomas for 10 years is 0.7 to 10%. As a rule, the development of secondary AMML is preceded by a history of myelodysplastic syndrome (MDS); characteristic chromosomal abnormalities (deletions of the long arm of chromosomes 5 and 7) are detectable. In this case, the follow-up was 3 months before the development of AMML, during this period the patient was not found to have laboratory signs of MDS (anemia, thrombocytopenia) or chromosomal abnormalities associated with secondary MDS/AML. The presence of a leukemic stem cell is associated with the occurrence and development of hemoblastosis; that of the similar cell populations that may cause B-cell lymphomas remains uncertain. The described case may have defect in a hematopoietic stem cell that gives rise to both germs of hematopoiesis, as well as complete donor chimerism of bone marrow hematopoiesis, which gives hope to long-term remission in both DH lymphoma and AMML.

[Eight-year experience in treating aggressive mediastinal large B-cell lymphomas].

AIM: To make a differential diagnosis of diffuse large B-cell lymphoma (DLBCL) with primary involvement of the mediastinal lymph nodes (LN) and primary mediastinal large B-cell lymphoma (PMLBCL); to evaluate the efficiency of a modified NHL-BFM-90 (M-NHL-BFM-90) program in the treatment of the above nosological entities. SUBJECTS AND METHODS: The investigation enrolled 60 patients with large B-cell lymphoma (LBCL) with primary involvement of mediastinal LN who had been treated at the Hematology Research Center, Ministry of Health of Russia, in 2004 to 2012. The diagnosis of PMLBCL and DLBCL with primary involvement of mediastinal LN was based on histological findings, the phenotype of tumor cells, and molecular evidence. Treatment was performed according to the M-NHL-BFM-90 program. Three pregnant women received predelivery polychemotherapy (PCT) according to the VACOP-B protocol and continued to have a DexaBEAM chemotherapy regimen 3-4 weeks postpartum. In case of a residual mass, all the patients underwent consolidation radiotherapy to the mediastinal area in a total focal dose of 36 Gy. RESULTS: The diagnosis of PMLBCL was established in 39 patients: 10 men and 29 women whose ages were 18 to 60 years (median age 30 years); DLBCL with primary involvement of mediastinal LN was verified in 21 patients: 8 men and 13 women whose age was 21 to 70 years (median age 30 years). After m-NHL-BFM-90 treatment protocol, 5-year overall survival rates in the patients with DLBCL with primary involvement of mediastinal LN and in those with PMLBCL were 95+/-5 and 86+/-6% and 5-year event-free survival rates were 95+/-5 and 78+/-7%, respectively. All the pregnant women diagnosed with PMLBCL who had received the VACOP-B --> delivery--> Dexa-BEAM PCT regimen during pregnancy achieved remission. The follow-up periods were 30, 36, and 42 weeks. CONCLUSION: The patients with new-onset LBCL and primary involvement of mediastinal LN are a heterogeneous group that includes patients having two different diagnoses: PMLBCL and DLBCL. The efficiency of high-dose PCT is different in the patients with DLBCL with primary involvement of mediastinal LN and in those with PMLBCL (in spite of their similar clinical features, similar epidemiological characteristics, and the presence of the same unfavorable prognostic factors at onset).

[The specific features of renal lesion in Burkitt's lymphoma].

AIM: To analyze the causes of renal lesion in patients with Burkitt's lymphoma (BL) and to develop optimal treatment policy. MATERIALS AND METHODS: The data of examination and treatment were analyzed in 20 patients with BL (14 men and 6 women aged 15 to 57 years (median age 24 years)) who had been followed up for renal lesion at the Hematology Research Center (HRC) in 2003 to 2011. When admitted to hospital, all the patients were found to have ureteric compression, renal parenchymal tumor infiltration, massive tumor cytolysis syndrome (MTCS). Polychemotherapy (PCT) was performed in accordance with the original intensive BL-M-04 protocol. The extent of the process was estimated according to the classification developed by S.B.Murphy: L3 variant B of acute lymphoblastic leukemia in 10 cases; Stage IV in 2; Stage III in 8. Acute renal failure (ARF) was identified in 13 patients. A control group comprised 36 patients with BL without ARF who had been followed up at the HRC in 2003 to 2011 and included into the BL-M-04 protocol. The ratio of patients with bone marrow lesion was 7:13 and 9:36 in the BL + ARF and BL-ARF groups, respectively. RESULTS: Decreased urine specific gravity and proteinuria (0.4 to 1.3 g/l) were the first manifestations of renal lesion and were seen in approximately 50% of all cases both on admission to hospital and in the first stages of PCT (10 and 9, 8 and 7 of the 20 cases, respectively). Microhematuria more commonly developed after initiation of PCT (7 and 3 of the 20 cases, respectively). ARF was diagnosed in 13 patients (24% of the 55 BL patients followed up at HRC in 2003 to 2011). One female patient developed ARF after the start of PCT. Twelve patients developed this condition at the onset of disease; in 4 patients, ARF existing prior to PCT began progressing after drug administration. The etiology of ARF was generally mixed. At the onset of disease, MTCS (n = 6) and specific renal parenchymal infiltration (n=6) were more common causes of ARF. Postrenal anuria was present in 2 cases. ARF after PCT initiation resulted from the toxic effects of methotrexate and MTCS (3 and 4 cases, respectively). ARF regressed in the early periods: in the prophase (n = 4) and during or the first PCT block A (n = 9). The BL patients with ARF, as compared to those without the latter, showed a statistically significant earlier onset of myelotoxic agranulocytosis (MTA): on day 3 of an intercourse interval (95 CI from 0 to 3 days) versus its day 5 (95% CI from 2 to 5 days) and a statistically significant longer duration of MTA--12 days (95% CI from 7 to 16 days) versus 7 days (95% Cl from 3 to 10 days); they were observed to have more severe mucositis. Despite the longer intercourse interval, 10 patients with ARF achieved remission; 4 patients died from therapy-refractory sepsis and 1 patient from thrombocytopenia. In the patients with ARF, mortality rates were significantly higher than in those without ARF (33% versus 10%; p = 0.04). CONCLUSION: Although there is a high risk of worsening renal dysfunction, PCT is a necessary condition for ARF resolution in BL.

[Nine-year experience in the treatment of patients with diffuse large B-cell lymphosarcoma].

AIM: To ascertain indications to standard (CHOP-21/R-CHOP-21) and intensive (mNHL-BFM-90) treatment in patients with diffuse large B-cell lymphosarcoma (DLBCL) with involvement of lymphoid organs. MATERIAL AND METHODS: The trial, performed from January 2002 to December 2010, enrolled 139 DLBCL patients with affected lymph nodes (LN), tonsils, spleen, bone marrow (BM). The diagnosis was made according to WHO criteria. The patients were examined according to the protocol of lymphoproliferative diseases. Biopsy material from all 139 patients was studied immunohistochemically on paraffin blocks (LN, tonsils, spleen, BM) using a wide panel of antibodies. The same examinations of BM were made in all 18 cases of BM involvement. Cytogenetic examination was performed in 106 patients: 48 standard cytogenetic tests, 139 - FISH for t (14;18) as well as rearrangement of locus 3q27. Patients with a poor prognosis (n = 86, 61.8%) received intensive therapy according to mNHL-BFM-90 program. The signs of a poor prognosis were the following: massive tumor (tumor size more than 7.5 cm), invasion into the adjacent organs or tissues, stage III-IV disease by Enn-Erbor, high concentration of LDG. Patients without a poor prognosis (n = 53, 38.2%) received standard treatment CHOP-21 (n = 28) or R-CHOP-21 (n = 25). RESULTS: A complete remission without recurrences was achieved in all 53 patients without signs of unfavourable prognosis (100%). Overall 5-year survival was 96%, 2 patients died in remission of other causes. Of 86 patients with a poor prognosis a complete remission was achieved in 64 (74.4%) patients. Overall and recurrence-free 5-year survival was 65 and 86%, respectively. CONCLUSION: Standard treatment provided long-term complete remission in all the patients without poor prognosis. Intensive (mNHL-BFM-90) treatment produced the best results in generalized lesion without BM involvement. Overall 5-year survival was 84% in these patients and 12% in patients with BM involvement.

[Clinical and epidemiological features of Burkitt's lymphoma].

AIM: To characterize clinical and epidemiological features of adult Berkitt's lymphoma (BL). MATERIAL AND METHODS: The trial enrolled 72 patients (51 males and 21 females, age 14-69, mean age 27 years) treated in 1995-2008. RESULTS: Stage I BL (by S.B. Murphy) was diagnosed in 5 patients, stage II--in 9, stage III--in 25, IV--in 14 patients, B-cell acute lymphoblastic leukemia (ALL) (L3)--in 19 patients. Intoxication was seen in 56 (78%) patients, 38% patients had severe cachexia. Elevated concentration of lactatedehydrogenase (LDG) was detected in 57 (79%) patients. In all the cases clinical symptoms developed for 1-3 months, median 6 weeks. Bone marrow involvement was diagnosed in 22 (31%) patients, CNS was affected in 17 (24%) patients, of them 14 were males. Fifty two (72%) patients had abdominal, retroperitoneal and/or small pelvis tumors. Intestinal, hepatic, renal and gastric tumors occurred most frequently. Specific ascitis was detected in 25 (48%), tumor pleuritis--in 11 (15%) patients. BL of the facial skeleton, Waldeyer's ring, oro- and nasopharynx was in 12 (17%) patients. Seven patients had concomitant involvement of the CNS. Eight (38%) and 2 (10%) women had tumors of the ovaries and uterus, respectively. CONCLUSION: BL is characterized by the following clinical features: young age of the patients, most of them are males, B-symptoms, short history, generalized stages, extranodal lesions, firequent involvement of the bone marrow and CNS.

[First experience of using modified program NHL-BFM-90 for the treatment of primary diffuse large-B-cell lymphosarcoma of the bones and soft tissues with poor-prognosis].

AIM: To evaluate efficacy of intensive modified program NHL-BFM-90 (mNHL-BFM-90) in adult poor-prognosis patients with diffuse large B-cell lymphosarcoma (DLBCL) of the bones and soft tissues. MATERIAL AND METHODS: The mNHL-BFM-90 program was used in the treatment of 3 male and 2 female patients aged 17-69 years (median 42 years). Four patients had DLBCL of the bones and one patient--DLBCL of the soft tissues. All the patients had tumors more than 10 cm in size. B-symptoms, a high concentration of lactate dehydrogenase (LDG) were registered in 3 patients. One patient had stage IE by Ann-Arbor, two--stage IIE (involvement of regional lymph nodes), two--stage ME (multiple bone lesions). A total of 4-6 blocks of polychemotherapy according to mNHL-BFM-90 program were performed. RESULTS: Complete remissions were achieved in all the patients. They had no recurrences after 6 to 20 month (median 13 months) follow-up. CONCLUSION: Positive results of the program mNHL-BFM-90 in poor-prognosis patients with DLBCL of the bones and tissues necessitate further studies of this therapy.

[High-dose therapy of Burkitt's lymphoma in patients over 40 years of age].

AIM: To analyse efficacy and tolerance of high-dose polychemotherapy (PCT) of Berkitt's lymphoma (BL) in patients aged over 40 years. MATERIAL AND METHODS: High-dose PCT was given to 6 BL patients aged 41-56 years (median 48.1 years). RESULTS: Complete clinicohematological remissions were achieved in 4 patients. In two of them the treatment was discontinued after three blocks of PCT because of severe infectious complications. According to 4-12 month follow-up, remission continues. Remission was not achieved in two patients: one patient had primary resistance, the other died of sepsis after the second PCT course before remission. The time to remission did not correlate with age. Duration of myelotoxic agranulocytosis varied from 2 to 24 days. Duration of agranulocytosis did not correlate with age. Infections complicated 19 of 20 PCT blocks. Severity of complications caused withdrawal of three patients. CONCLUSION: BL is biologically heterogenous as it demonstrates different responses to BL-M-04 program. Causes of slow regression of tumor mass in some patients need further investigations. In spite of a great number of infectious complications high-dose therapy has no alternative.

[Efficacy of conservative treatment of gastric lymphosarcoma].

AIM: To compare efficacy and toxicity of conservative therapy (different programs of polychemotherapy) of gastric lymphosarcoma conducted for the last 10 years in Hematological Research Center of the Russian Academy of Medical Sciences. MATERIAL AND METHODS: The study included 63 patients (40 females and 23 males aged 14 to 78 years, mean age 49 years) with primary diagnosis of gastric lymphosarcoma (GL). Of them, 56 (89%) patients had diffuse large B-cell lymphosarcoma (DLBCL) and 7 (11%) had gastric Berkitt's lymphoma (BL). Only detection of t(8;14) with rearrangement of c-myc gene provided accurate diagnosis of gastric BL. By the treatment DLBCL patients were divided into two groups: 44 patients of group 1 received polychemotherapy (PCT) according to CHOP scheme or in combination with radiotherapy and surgical treatment; 12 patients of group 2 were treated according to modified program mNHL-BFM-90, without surgical or radiation treatment. Of 7 patients with gastric BL 5 patients received treatment according to a modified program mNHL-BFM-90 and 2 patients were given CHOP because of DLBCL misdiagnosis without cytogenetic detection of t(8;14). RESULTS: Overall survival in group 1 was 73% in mean follow-up 61 months. The survival depended only on initial factors of poor prognosis (PPF): tumor size over 10 cm, Ann-Arbor stage higher than IE, B-symptoms, elevated level of LDH. Overall survival of 18 gastric DLBCL patients without PPF reached 94%, of 26 patients with PPF - 60%. Lethality due to side effects was 4% (2 patients), primary resistance was 14% (6 patients), recurrence arose in 9% (4 patients). Overall survival in group 2 was 100% in mean remission duration 18 months, was unrelated to PPF (10 of 12 patients) but correlated with high toxicity. 5 BL patients treated with a modified mNHL-BFM-90 program achieved remission (a mean follow-up at present is 1 to 50 months, mean 24 months). 2 BL patients treated with CHOP died for a year. CONCLUSION: Gastric lymphosarcomas are sensitive to chemotherapy, thereby PCT only is effective in most patients. PPF in gastric DLBCL were responsible for poor outcome in 40% patients in CHOP treatment. The modified program mNHL-BFM-90 can produce up to 100% complete long-term remissions in therapy of gastric lymphosarcoma in adults both in BL and DLBCL patients. A cytogenetic examination of c-myc gene rearrangement is obligatory before initiation of PCT of gastric lymphosarcoma.

[Detection of translocations diagnostic for Berkitt's lymphoma by fluorescent in situ hybridization on histological sections of paraffin blocks].

AIM: To test feasibility of detection of translocations which are diagnostic for Berkitt's lymphoma with the method of fluorescence in situ hibridization (FISH) on histological sections of paraffin blocks. MATERIAL AND METHODS: FISH on histological sections for detection of t(8;14)(q24;q32) and variant t(2;8)(p12;q24) and t(8;22)(q24;q11) was performed on the material obtained from 53 patients with typical clinical, morphological and immunological picture. DNA probe LSI IgH/MYC, CEP 8 Tri-color, Dual Fusion Translocation Probe (Vysis, USA), for variant translocations DNA-probe LSI MYC, Dual color, Break Apart Rearrangement Probe (Vysis, USA) were used. RESULTS: Histological material from 31 patients contained translocations characteristic for LB: in 29 (93.5%)--t(8;14)(q24;q32), in 2--variant rearrangements of locus of gene c-myc. Translocation t(8;14)(q24;q32) and its variants were not detected in 22 patients, the diagnosis was changed for diffuse large B-cell lymphoma (DLBCL). CONCLUSION: Typical for BL clinical, morphological and immunological picture may present in extra-nodal diffuse large B-cell lymphoma with high proliferative activity. Differential diagnosis between BL and the latter lymphoma is possible only basing on detection of translocation t(8;14)(q24;q32) or its variants. If it is impossible to obtain native material, FISH on histological sections of parasffin blocks is the only possible method of differential diagnosis.

[The modified program NHL-BFM-90 in the treatment of patients with diffuse large B-cell lymphosarcoma].

AIM: To investigate efficacy of the modified protocol NHL-BFM-90 in patients with diffuse large B-cell lymphosarcoma (DLBCLS). MATERIAL AND METHODS: A total of 13 DLBCLS patients with stage II-IV of the disease with affection of lymph nodes at the disease onset (nodal lesion) and stage II with tumor size more than 10 cm (bulky disease) received first-line treatment according to the modified program NHL-BFM-90 from 2002 to 2005. The diagnosis was made by WHO criteria. RESULTS: A complete remission was achieved in 76.9% patients. Resistance to therapy was observed in the patients with bone marrow affection. The 2.5-year overall survival was 74%, 2-year event-free survival was 75% (the events were recurrence and resistance). Follow-up continued from 5 to 47 months. CONCLUSION: The efficacy of the modified protocol NHL-BFM-90 in DLBCLS patients with stage III-IV of the "nodal" disease and stage II of the "bulky" disease was high.

[First experience with the modified program NHL-BFM-90 application in adult patients with primary diffuse large B-cell gastric lymphosarcoma with unfavourable prognosis].

AIM: To assess efficacy of a modified program NHL-BFM-90 in adult patients with primary diffuse large B-cell gastric lymphosarcoms (PDLBGL) with unfavourable prognosis. MATERIAL AND METHODS: Modified courses of NHL-BFM-90 were conducted in 5 patients aged 27-67 years from January 2004 to September 2005. Four patients received chemotherapy of the first line, in one patient block therapy followed monotherapy with chlorambucil and a CHOEP course. All the patients were in a severe clinical condition and had several initial factors of unfavourable prognosis: size of the tumor more than 10 cm; stage IE and more advanced; B-symptoms; proliferative activity above 70%. The program NHL-BFM-90 was modified because of the patients' age. Chemotherapy was conducted according to the middle arm of the original program NHL-BFM-90, but methotrexate was introduced in a dose 1 g/m2 for 12 hours, while leukovorin was given 18 hours after the start of methotrexate injection. In two cases the blocks were enhanced with rituximab, 2 patients had doxorubicin in block A, in one case block C was enhanced with methotrexate. A total of 23 modified blocks NHL-BFM-90 were performed: one patient was given 6 blocks, two patients--5, one patient--4 blocks and one patient--3 blocks. RESULTS: Four patients after block 2 and one patient after block 3 of polychemotherapy NHL-BFM-90 achieved remission of the disease of 6 to 22 months duration which still continues. Infectious complications related to hematological toxicity arose more frequently at the latest courses of chemotherapy. CONCLUSION: Treatment according to the modified program NHL-BFM-90 in adult patients with PDLBGL and unfavourable prognosis is highly effective. For a mean follow-up of 10.2 months no recurrences occurred. The number of courses can be reduced to decrease accumulated hematological toxicity and in case of rapid achievement of remission.

[Treatment of adult Berkitt-like lymphoma].

AIM: To compare programs of chemotherapy used in adult Berkitt-like lymphoma (ABLL); to assess efficacy and toxicity of the protocol AblL-M-04. MATERIAL AND METHODS: 31 ABLL patients (23 males, 8 females, mean age 27 years) participated in the study performed in Hematological Research Center in 1995-2004. ABLL stage I, II, III and IV was diagnosed in 3, 5, 8 and 15 patients, respectively. 10 patients had diffuse large B-cell lymphoma. 9 patients received 2 to 6 courses of CHOP, 1 patient--6 courses of Pro-Mace-Cytabom, 11 patients with newly diagnosed ABLL and 5 pretreated with CHOP--NHL-BFM-90. The modified protocol ABLL-M-04 of intensive short-term therapy included 10 patients, 2 of them pretreated. RESULTS: Of 10 patients given CHOP or CHOP-like courses 9 were resistant to therapy, 2 died of rapid progression, 7 were converted to the program therapy. 5 patients on the protocol NHL-BFM-90 died after short-term improvement. None of them achieved remission. Of 10 patients with newly diagnosed ABLL treated according to NHL-BFM-90 protocol, remission was achieved in 4 patients, follow-up median--34 months (2-56). Six patients died: 4 of progression, 2 of chemotherapy complications. BLL-M-04 therapy was made in 9 patients: 7 patients persisted on the first remission, 2 patients died of chemotherapy complications. Overall duration of the treatment was 3-3.5 months. CONCLUSION: The protocol ABLL-M-04 seems to be more effective than a classic NHL-BFM-90, but this must be supported by more cases. CHOP therapy cannot be recommended for patients with ABLL because of poor efficacy (all the CHOP patients died).