Between July 2005and October 2006, eight patients (pts) with hairy cell leukaemia were treated with Vero- cladribine at N.N. Blokhin Cancer Research Center. All pts achieved complete remission (CR). Duration of CR ranges from 1+ to 12+ months in 7 cases. There were no serious adverse events during the treatment and follow-up periods (1-12 months). Vero- cladribine was well tolerated. Anemia (grade IV) was seen in 1 patient and thrombocytopenia (grade I) was seen in another 1 patient. No one patient has febrile neutropenia or infectious complication.

The paper discusses the new 2008 WHO classification of myeloproliferative neoplasms (MPN) and compares it with its previous 2001 edition. The introduction of the last version of the WHO classification into clinical practice has been due to new molecular biological and histological evidence in patients with MPN. The classification contains substantial alterations made in a number of nosological entities and the new diagnostic marker for MPN - JAK2 gene mutation being proposed. Mastocytosis-specific c-KIT anomaly is identified. A new form of hematopoietic system tumors, such as myeloid and lymphoid neoplasias with eosinophilia and mutations of the PDGFR A and B and FGFR1 genes, is singled out and characterized. New differential diagnostic parameters of a histological study of bone marrow trepans in MPN are proposed.

Острый промиелоцитарный лейкоз - ОПЛ (МЗ по FAB-классификации) - довольно редкий вариант острого лейкоза, на его долю приходится не более 10% среди всех острых нелимфобластных лейкозов. Яркая клиническая картина и морфологические особенности заболевания позволили Hillestad еще в 1957 г., задолго до создания FAB-классификации, выделить его в качестве отдельной формы острого лейкоза. Современный этап терапии ОПЛ связан с расшифровкой молекулярно-генетических изменений гена рецептора ретиноевой кислоты в кроветворных клетках миелоидного ряда, возникающих при ОПЛ и лежащих в основе патогенеза заболевания.Успехи современной терапии ОПЛ - получение ремиссий, в том числе молекулярных, и долгосрочной выживаемости у 80-90% больных позволяют говорить о принципиальной излечимости этого варианта лейкоза. В настоящее время аллогенная трансплантация костного мозга или периферических стволовых клеток для этих больных считается показанной только во второй или последующих ремиссиях.

Examinations of 174 children and 188 adult patients with acute nonlymphoblastic leukemia (ANLL) demonstrated a similar structure of distribution of ANLL FAB-variants in children and adults, although the incidence of M0 and M4 blasts was somewhat higher in infants aged under 2. In patients under 15 and over 60 peroxidase activity in myeloblasts was reliably lower than in the rest patients. HLA-Dr, Thy-1, CD11a, T-CD19, Gly-A, and Eb antigens were equally incident in the cells of children and adults. The expression of CD11b, CD38, and CD10 antigens on the blasts was higher in children than in adults. An abnormal blast karyotype was detected in 81.8% children and 73.7% adults. Translocation (8;21) was observed in patients with the M2 variant, as a rule (82%), and reliably more frequently in children; t(9;22) and t(11q23) occurred in children somewhat more frequently than in adults. A group of children with primary ANLL (n = 3) was distinguished for the first time, in whose cell karyotype a deletion of chromosome 5 was found. The findings indicate that the biological characteristics of blast cells differ in children and adults. Evidently, the level of hemopoiesis involvement in ANLL is earlier in infants under 2 and subjects over 60 than in the rest patients.

Twenty-five patients with acute lymphoblastic leukemia [15 adults and 10 children] received standard treatment in which regular L-asparaginase was replaced for L-asparaginase of prolonged action [PEG-asparaginase]. The drug was administered once in two weeks in a dose 2500 IU/m2 for remission induction and consolidation or as a component of maintenance therapy. It was found that the response to primary PEG-asparaginase treatment or its use in the disease relapses produced the same response as regular L-asparaginase, being superior in convenience and feasibility of outpatient use. Side effects in the form of hypoproteinemia, hepatic toxicity and toxic pancreatitis [in children, 9 and 1 adults, respectively] were moderate and disappeared after 10-20-day discontinuation of the drug.

Expression of new Mab D11 on blood and bone marrow cells was investigated in 85 hemoblastosis patients. In normals, antigen D11 is expressed on some monocytes and all tissue macrophages. D11 was noted on lymphoblasts of 5 out of 10 cases with B-cell ALL and of 1 case in B-lymphoid blast crisis of chronic myeloid leukemia. In T-ALL, ANLL, non-Hodgkin's lymphomas in leukemization stage, hairy cell leukemia and chronic lymphoid leukemia the cells were nonresponsive to Mab D11. Unlike D11 which have round nuclei, lymphoblasts D11+ have folded nuclei and more pronounced cytoplasmic basophilia. There were both B and myeloid antigens on D11+ blasts. ALL D11+ patients had extramedullary foci, more suppressed granulocytic and thrombocytic components of hemopoiesis, shorter remissions than those with ALL D11-.

Bone marrow hairy cells were examined in 36 patients with hairy cell leukemia. Cytograms per 100 of hairy cells were assessed in all the patients. Leukemic cell size, cytoplasm profile, shape of the nucleus, and share of granular cells were under study. Cytochemical examination of hairy cells was carried out in 26 patients. Acid phosphatase and its tartrate inhibition, nonspecific alpha-naphthylacetate esterase, acid nonspecific esterase, and PAS reaction were investigated. In 15 patients hairy cells were immunologically examined using immunocytochemical APAAP method with IKO monoclonal antibodies 1, 91, 20, 124, 115, 92, 105, 30, 31, 86, 80, 87, GM-1. Morphologically two types of hairy cells were detectable: typical, with torn villose cytoplasm with numerous processes, and cells with clearly shaped scalloped cytoplasm. The two types did not differ by nuclear shape or size, presence of granules, but differed by their functions. Process hairy cells were characterized by features most typical of these cells: presence of tartrate-resistant acid phosphatase and CD22 (IKO91) B-cellular marker in the phenotype. In scalloped hairy cells cytochemical and immunological signs were less manifest.

Eighteen patients with acute granulocytic leukemia (MI-1, M2-10, M3-1 and M4-6) underwent AdOAP treatment. Complete remissions were achieved in 10 out of the 18 patients (55.6%), 3 patients proved resistant, 5 had died prior to recovery of normal hemopoiesis. Three lethal cases are attributed to the results of the therapy complications. Median duration of complete remissions reached 17.5 months. Two patients have been in remission over 3 years. Reduced duration or intensity of the induction therapy failed to diminish frequency of lethal complications due to progressive cytopenia.

This method (PAP method) may be used for determining the cellular immunologic phenotype in bone marrow and blood smears, of the blast elements in hemoblastosis patients among other things. The panels of monoclonal antibodies for the PAP method should be selected by their preliminary testing in the PAP method and in immunofluorescence test. PAP method with monoclonal antibodies may be used in both hematologic and cytologic laboratories for determining the histogenetic appurtenance of the cells in dubious diagnostic cases.

The possibility of TPA-induced differentiation of K-562 cell line was studied. Monoclonal antibodies against differentiating antigens of HAE erythroid lineage and against myelomonocytic ICO lineage raised in the USSR Cancer Research Centre were used. Changes in immunological and cytochemical indexes suggest that K-562 cell differentiation goes in the erythroid direction. The cells lost early differentiation and acquired late differentiation markers.

The results of programmed therapy of acute lymphoblastic leukemia (ALL) in 31 adult patients were analyzed. Ph-positive ALL were marked in 5 patients. Low hemosuppressive induction, heavy consolidation and multidrug maintenance therapy was used. Complete remissions were obtained in 18 patients, their median time was 18 months (from 2 to 46 months). The patients were divided into groups with a good, intermediate and poor prognosis on the basis of an immunological ALL variant, the presence or absence of basal hyperleucocytosis and Ph-chromosome. Data on adequate therapy for each group were presented.

The karyotype of leukemic cells was studied in 88 acute nonlymphocytic leukemia (ANLL) patients. Chromosome abnormalities were discovered in 78.4% of all patients and in 72.5% of the 69 patients studied before treatment. Characteristic abnormalities: translocations 8;21, 15;17, 9;22 or 6;9, rearrangements of 11q, gain of chromosomes 8 or 21, and loss or deletion of chromosomes 5 or 7 were detected in 56 of 69 patients with abnormal karyotypes. Translocation 8;21 was revealed in 27 patients; 20 of them had M2 FAB-form, four had M1, and three had M4. In patients with t(8;21) the incidence of complete remission was higher and the duration of first remission and survival longer than in patients with other abnormalities or with a normal karyotype.

The authors describe a female patient with acute lymphoblastic leukemia. After the first chemotherapy cycle according to the VAMP program the patient developed a complete remission. Further on, repeated extramedullary relapses (neuroleukemia, involvement of the mammary gland tissue and lymph nodes of the mesentery) were observed over 7 years, the data of blood and bone marrow analyses being within normal. At the end of the disease the patient developed a relapse with changes in the blood and bone marrow, however the blast cells were sudan and peroxidase-positive i.e. could be classified as myeloblasts.