SEMINAR IN BIOLOGY
DIAGNOSIS OF HEMATOLOGIC MALIGNANCIES
896 patients (pts) with malignant non-Hodgkin's lymphomas were examined in this study. Т-cell immunophenotype has been established in 208 cases. Diagnosis was established according to the WHO criteria. We used the panel from 15 monoclonal antibodies for the detailed characteristic of Т-cell immunophenotype of non-Hodgkin's lymphomas. Lymphoblastic lymphomas was revealed in 115 pts and peripheral T-lymphomas in 93 pts. It was shown, that immunocytochemical research with quantitative calculation of antigen-positive cells has the certain value for differential diagnostics of peripheral Tcell lymphomas. We determined some quantitative diagnostics criteria in addition to WHO classification and proved their evidence in malignancy heterogeneity typical for T-cell lymphomas.
CLINICAL PICTURE OF HEMATOLOGIC MALIGNANCIES
During long term follow up (median 10 years) of 412 patients with primary Sjogren's syndrome (pSS) 46; (11,2%) women developed non-Hodgkin's lymphoma (NHL). Median pSS duration before development of NHL was 17 years. 43 (93,3%) patients had B-cell and 3 (6,7%) - T-cell NHL. All types of NHL were present except precursor cell lymphomas. Diffuse large B-cell lymphoma (LBCL) (39%) and MALT lymphomas (21%) prevailed. Nodal NHL with extranodal involvement (52,2%) were the most frequent. Nodal (21,8%) and extranodal (26%) lymphomas were less frequent. The most frequent target organs in lymphoproliferative disease (LPD) were lymph nodes (74%), salivary glands (45,5%), lungs (26%), bone marrow (19,5%), liver (17,5%), spleen (13%), lachrymal glands (6,5%). Waldeyer's throat ring (4%), oral cavity mucous membrane, ovary and brain (2%) were involved rarely. Immunoglobulin-secreting variant of lymphoma was revealed in 53,7% of cases. LPD developed predominantly in patients with systemic features and late stage of pSS. Significant increase of parotis, mixed monoclonal cryoglobulinemia, generalized lymphadenopathy, presence of more than 5 focuses of lymphoid infiltration in small salivary glands biopsies and thrombocytopenia were predictors of NHL development in pSS (p<0,001). Prolonged treatment with small doses of alkylating cytostatic agents (leukeran, cyclophosphan) decreased risk of LPD development in pSS (p><0,001). 5-year survival of patients from the moment LPD was diagnosed was 54% mainly due to high mortality in the group of patients with LCL. Survival of pSS patients with and without LPD s><0,001). Prolonged treatment with small doses of alkylating cytostatic agents (leukeran, cyclophosphan) decreased risk of LPD development in pSS (p<0,001). 5-year survival of patients from the moment LPD was diagnosed was 54% mainly due to high mortality in the group of patients with LCL. Survival of pSS patients with and without LPD significantly differed (p<0,001): after 20-year follow up survival was 32% in group with LPD and 76% in group without LPD. Probability of NHL development after 40-year course of pSS was 40% what allows to consider this disease simultaneously autoimmune and lymphoproliferative.
We followed 66 lymphoma -AIDS patients (pts): aggressive lymphomas were diagnosed in 48 pts (male — 35, female — 13, median age 32.5±1.2) and Hodgkin's lymphoma was established in 18 pts (male — 14, female — 4, median age 34.1±2.3). Median duration time from HIV exposure to the onset of lymphomas was 5 years (2—16 years). A part of pts was receiving HAART. 85 % of pts were drug users and association with HCV was shown in 60 % of them, with HCV and HBV- in 25 %. Sexual transmission was mentioned in 15 % of pts. «Immune status»: CD4 counts were from 50 to 500 (median 225) cells in mcL. Viral load was from 400 to 75000 (median 38000) copies in mcL. Histological diagnosis: diffuse large cell lymphomas — 60%, Burkitt lymphoma — 16%, follicular lymphoma — 12%, MALT-lymphomas-6%, T- cell lymphoma — 4%, primary CNS lymphoma — 2%. CHOP and CHOP-like courses had received 26 pts. Results: 3-year overall survival — 52%, 3-year disease free survival — 32%. Block therapy A-B-C of BFM — NHL— 90 and LB-M-04 with and without Mabthera had received 12 pts. Results: 3-year overall survival — 48%, 3-year disease free survival — 38%. After treatment CD4 count was 60 — 700 (median 316) cells in mcL, viral load 400 to 25000 (median 3000) copies in mcL. 75% of Hodgkin's lymphoma patients had III—IV stages of disease. Initial immune status: CD4 counts from 50 to 730 (median 363) cells in mcL, viral load — from 14000 to 150 000 (median 100 000) copies in mcL. Histological variants: mixed cellularity — 14 pts, lymphoid depletion — 3 pts, nodular sclerosis — 1 pt. Chemotherapy had received 11 pts: ABVD with or without radiotherapy — 8 pts, BEACOPP-escalated with or without radiotherapy — 3 pts. Complete 3-year remissions were achieved in 2 pts. Other pts are on therapy. After treatment CD4 counts were 184 — 900 (median 400) cells in mcL, viral load — 435000 (median 100 000) copies in mcL. Modern treatment approaches in lymphoma -AIDS pts can lead to complete remission, as well as in general non-HIV positive population.
THERAPY OF HEMATOLOGIC MALIGNANCIES
Russian Interregional Register of hematopoietic stem cell transplantation was established in 2000. 11 transplant centres from 7 Russian cities are including in Register now. In this analysis we applied transplant activity survey data from 01.01.1996 to 31.12.2006. A total 1118 transplants (846 – autologous and 272 – allogeneic) were carried during this period.
COMPLICATIONS OF HEMATOLOGIC MALIGNANCIES AND THEIR TREATMENT
RARE AND DIFFICULT HEMATOLOGIC SYNDROMES
We describe a rare case of myeloid/NK cell precursor acute leukemia (MNKL) in a 38-year-old woman. Upon diagnostic examination, a tumor invasion of bone marrow, lymphatic nodes, liver and spleen was found. The proportion of blasts in peripheral blood reached 54%. The blasts were polymorphic, with round or irregularly shaped nuclei. The tumor cells were negative for myeloperoxidase, non-specific esterase, and lipid staining. The blasts not only expressed CD13+ and CD33+ panmyeloid antigens, but also carried the markers of NK-cell differentiation (CD16+, CD56+, Perforin+). The leukemoid infiltration of liver, acute hepatitis and development of liver cell failure were the distinctive features of the described case. The issues pertaining to differential diagnostics and classification of MNKL are also discussed.
SEMINAR IN HEMATOLOGY
This article reviews the role of recombinant erythropoietin in the management of anemia in cancer patients based on literature data and our experience. We describe the problem of toxicity and report clinical recommendations and cautions for usage of drugs appertain to this pharmaceutical group.
CONGRESSES, CONFERENCES, SYMPOSIUMS
ISSN 2413-4023 (Online)