[Crystalline histiocytosis].

The paper describes a case of diagnosis of the rare monoclonal secretion-associated disease crystalline histiocytosis with kidney and bone marrow involvement. The female patient with multiple myeloma (MM) was found to have intralysosomal crystals in the cells of the bone marrow (histiocytes, plasmocytes), kidneys proper (mesangiocytes, podocytes), and subsequently in those of a kidney graft. Lower secreted monoclonal IgG and ceased Bence-Jones protein secretion after MM chemotherapy were accompanied by improved and stabilized kidney graft function.

[Long-term results of treatment for T-cell lymphoblastic lymphomas].

Aim: To define the efficiency of the GMALL 2002 program for the treatment of patients with T-cell lymphoblastic lymphomas (T-LBL).

Subjects And Methods: Twenty-five patients with a verified diagnosis of T-LBL were examined. Male/female ratio was 19:6; median age was 33 (range 16-67) years.

[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 levels of key nucleolar proteins in the lymphoid cells of healthy individuals and patients with lymphoproliferative diseases].

Immunocytochemical staining with specific antibodies was used to study the expression of three nucleolar proteins (fibrillarin, B23/nucleofozmin, and SURF6), which were involved in pRNA maturation, in the lymphoid cells of healthy individuals and patients with lymphoproliferative diseases and to compare it with the expression of the known proliferation marker Ki-67 protein. The results indicated that fibrillarin was detectable at the comparable level in the lymphoid cells of the patients and in the peripheral blood lymphocytes of the healthy individuals. In one fourth of the patients, the proportion of cells containing B23/nucleofozmin was noticeably higher than that in the lymphocytes of donors; however, there was no great difference in patients with different types of the disease.

[Efficiency of cyclosporin A therapy in patients with myelodysplastic syndrome].

Aim: To evaluate the efficacy of cyclosporin A (CsA) in patients with myelodysplastic syndromes (MDS) and to identify determinants of a response to this therapy.

Subjects And Methods: The efficacy of CsA was evaluated in 52 patients (30 men and 22 women aged 16 to 74 years) with MDS. Thirty-two patients were given CsA as first-line therapy; 20 patients took the agent after prior therapy.

[Hereditary spherocytic hemolytic anemia in an adult with the formation of ectopic foci of extramedullary hemopoiesis in the chest].

The paper describes a rare case of formation of paravertebral extramedullary hemopoietic foci in microspherocytic anemia or Minkovsky-Shoffar disease in an adult. Therapeutic splenectomy has led to regression of extramedullary hemopoietic foci, which supports that there is a direct relationship of the above formations to the specific features of the etiology and pathogenesis of microspherocytic anemia.

[Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment].

Aim: To diagnose diffuse large B-cell lymphosarcoma (DLBCLS) with primary involvement of the mediastinal lymph nodes (LN) and to evaluate the efficiency of aggressive polychemotherapy (PCT).

Subjects And Methods: The study included 15 patients (6 men and 9 women aged 18 to 70 years; median 38 years) followed up at the Hematology Research Center, Russian Academy of Medical Sciences, in 2004 to 2009. Three and 12 patients had Stages II and IE DLBCLS, respectively.

[Prolonged bone marrow aplasia in patients with acute leukemia after chemotherapy].

Aim: To analyze the causes of prolonged hematopoietic tissue aplasias in patients with acute leukemias (AL) after chemotherapy courses.

Materials And Methods: Data on 7 patients with acute myeloid leukemia, followed up at the Hematology Departments, Hematology Research Center, Russian Academy of Medical Sciences, over the period 2003 to 2007, who had developed deep bone marrow aplasia (BMA) inadequate to cytostatic drug exposure during chemotherapy, were analyzed. The authors compared in all the patients the values of peripheral blood and bone marrow (BM) puncture specimens and the results of blood tests using the polymerase chain reaction at different AL development stages with the results of an immunohistochemical study using the markers of viruses of hepatitis C and B, a herpes group (EBV, CMV, HSV-1, HSV-2) and parvovirus B19.

[A case of hemolytic-uremic syndrome with development of catastrophic antiphospholipid syndrome: diagnosis and clinical tactics].

The paper describes a case of practically simultaneous development of the hemolytic-uremic syndrome (HUS) and the catastrophic antiphospholipid syndrome (CAPS) complicated by mesenteric vessel thrombosis and small bowel necrosis. Multimodality treatment comprising volume plasmapheresis, fresh frozen plasma transfusion, hemodialysis, anticoagulant and disaggregant therapy could relieve thrombogenic events, such as pulmonary artery thromboembolism and intestinal necrosis.

[Study of bone marrow microvessel density is one of the diagnostic approaches in patients with Ph-negative chronic myeloproliferative diseases].

Aim: To define an association of bone marrow microvessel density (MVD) with histological properties (the magnitude of fibrosis and quantification of megakaryocytes (MGKC)) in patients with Ph-negative chronic myeloproliferative diseases (CMPD).

Subjects And Methods: MVD was analyzed in 93 patients with different forms of CMPD, by estimating histological parameters. True polycythemia (TP) was present in 28 patients; 20 patients had essential thrombocythemia (ET), 36 had subleukemic myelosis, out them 6 were in a prefibrotic stage, and 9 with diagnosed post-TP (ET) myelofibrosis.

[Acute abdominal syndrome in blood system diseases].

Aim: To define an optimal diagnostic and therapeutic algorithm when the acute abdominal syndrome occurs in hematological patients.

Materials And Methods: The results of 145 emergency surgeries made in 2006-2008 for acute abdominal syndrome were studied in patients with blood system diseases.

Results: Clinical manifestations of acute abdominal syndrome emerge in 1-1.

[Tumor development from follicular dendritic cells in a hyaline-vascular variant of Castleman's disease].

Literature gives only few reports of hyaline-vascular variant of Castleman's disease associated with tumor from dendritic cells. A case of simultaneous detection of these diseases in the tumor located in the retroperitoneal space and successfully removed surgically is reported.

[First experience of using modified high-dose therapy NHL-BFM-90 in diffuse large B-cell lymphosarcoma with primary skin involvement. A case report].

Primary skin large B-cell lymphosarcomas (PLBCL) present with skin lesions, other organs and systems are not involved. As CHOP courses are not high effective in PLBCL, we were the first to treat a patient with modified block therapy NHL BFM-90. A complete remission was achieved after the first course of polychemotherapy and was consolidated by two courses of treatment.

[Diagnosis and treatment of skin T-cell lymphoma undergoing transformation in lymphosarcoma].

Aim: To define complex of parameters characterizing transformation of skin T-cell tumors in lymphosarcoma; to show specific treatment of patients with this transformation.

Material And Methods: Of 57 patients with primary T-cell lymphomas of the skin (mycosis fungoides, Sezary's disease), we studied 12 patients with transformation of the process into lymphosarcoma by clinical, histological, moleculobiological and immunophenotypical parameters.

Results: We found that transformation of T-cell lymphoma into lymphosarcoma occurred in different time from the disease onset (2-12 years).

[High-dose polychemotherapy of patients with poor-prognosis anaplastic T.0-large cell ALK+ lymphosarcoma].

Aim: To evaluate efficacy of the protocol NHL BFM-90 in the treatment of adult anaplastic large cell lymphosarcoma (ALCL) ALK+ and validity of addition of transplantation of autologous stem hemopoietic cells (ASHC) into first line treatment.

Material And Methods: We treated 13 patients with stage III-IV ALCL ALK+. The age of the patients ranged from 17 to 44 years (median 26 years).

[Hairy cell leukemia in young patients].

Aim: To characterize clinical symptoms, course, immediate and long-term treatment results in young patients with hair cell leukemia (HCL).

Material And Methods: The data on 41 HCL patients were analysed. The diagnosis was made by standard diagnostic protocol for HCL detection.

[Efficacy of therapy of different variants of anaplastic large T-cell lymphomas].

Aim: To compare efficacy of NHL-BFM-90 and CHOP-like courses in the treatment of anaplastic large cell lymphoma (ALCL).

Material And Methods: Twenty-two patients with ALCL participated in the study. The diagnosis was made basing on the findings of clinical, device, morphological, immunohistochemical and molecular-genetic examinations with application of a panel of monoclonal antibodies to CD30, ALK, CD3, CD4, CDS, CD7, CD34, CD15, CD68, CD20, CD45RO, CD45RA, Ki-67.

[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).

[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).

[Synchronous and metachronous tumors in patients with hematological neoplasms].

The study has indicated the relatively frequent occurrence of secondary tumors in hematological patients. These tumors may be both synchronous and metachronous. They generally occur in different periods after chemo- and/or radiotherapy.

[Parenchymatous-stromal factors in hemoblastoses].

The data available in the literature data on the role of neoangiogenesis and extracellular matrix factors in the pathogenesis of acute and chronic leukemias and lymphoproliferative diseases are presented. Hematopoiesis and endothelial cells have a common pluripotential progenitor. Angiogenic and fibroblast growth factors are involved chronic leukemia.