RESUMO
Based on the analysis of more than 1000 bone marrow trephine biopsy specimens from patients with myelodysplastic syndromes before starting any specific therapy, the authors propose histological criteria for their evaluation (an examination algorithm), an optimal panel of immunohistochemical examination, and histological criteria for predicting the course of the disease.
Assuntos
Biópsia , Medula Óssea/patologia , Síndromes Mielodisplásicas/diagnóstico , Medula Óssea/metabolismo , Humanos , Síndromes Mielodisplásicas/patologia , Manejo de EspécimesRESUMO
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. However, a repeat morphological study of a renal biopsy specimen showed that the crystalline inclusions were preserved in the podocytes. By comparing the immunological and renal responses, it is reasonable to suggest that monoclonal IgG rather than Bence-Jones protein is of value in the pathogenesis of crystal formation.
Assuntos
Histiocitose/patologia , Rim/patologia , Mieloma Múltiplo/patologia , Adulto , Antineoplásicos/uso terapêutico , Proteína de Bence Jones/metabolismo , Medula Óssea/metabolismo , Medula Óssea/patologia , Cristalização , Feminino , Humanos , Imunoglobulina G/imunologia , Transplante de Rim/métodos , Mieloma Múltiplo/tratamento farmacológicoRESUMO
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. There was a preponderance of patients with the generalized stages of the diseases: 2, 5, and 18 with Stages II, III, and IV, respectively. Mediastinal lesion was found in 20 (80%) of the 25 patients. Their treatment was performed according to the GMALL 2002 program and similar CHOP courses. Analysis was made in 2 groups that were not different in their clinical and morphological characteristics. Group 1 consisted of 17 of the 25 patients treated according to the GMALL programs; Group 2 comprised 8 patients who had similar CHOP and other chemotherapy regimens. RESULTS: In Group 1, 15 (88%) patients achieved a complete clinical and hematological remission and 2 (12%) patients died in the first stages of the treatment. No relapses were noted. The median survival had not been achieved; 5-year overall survival was 88 +/- 8%. In Group 2, three patients were alive; 2 completed their treatment; 5 (63%) patients died from treatment failures. The median survival was 23 +/- 18%; 5-year overall survival was 45%. CONCLUSION: The findings suggest that the GMALL 2002 programs are highly effective in treating patients with T-LBL at the first stage of treatment.
Assuntos
Linfoma de Células T/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica , Estudos de Viabilidade , Feminino , Humanos , Linfoma de Células T/mortalidade , Masculino , Pessoa de Meia-Idade , Moscou , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
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.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Intervalo Livre de Doença , Feminino , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/mortalidade , Masculino , Pessoa de Meia-Idade , Recidiva , Esplenectomia , Resultado do Tratamento , Adulto JovemRESUMO
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. RESULTS: The marker of hepatitis C was detected in 6 of the 7 patients with prolonged BMA; 3 of these 6 patients showed a simultaneous infection with hepatitis B. Six of the 7 patients were found to have concomitant BM lesion with various herpes group viruses. Two patients had a resistant form of AL. CONCLUSION: Hepatitis C virus infection in patients and the resistant form of the disease were the principal causes of the development of BMA inadequate to cytostatic drug exposure. Affliction of abundant bone marrow cells with herpes group viruses was not a direct cause, but might substantially aggravate BMA.
Assuntos
Anemia Aplástica/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Hepatite C/complicações , Leucemia Mieloide Aguda/tratamento farmacológico , Adulto , Idoso , Anemia Aplástica/virologia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medula Óssea/patologia , Medula Óssea/virologia , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Hepacivirus/isolamento & purificação , Hepatite C/virologia , Humanos , Leucemia Mieloide Aguda/patologia , Leucemia Mieloide Aguda/virologia , Leucopenia/etiologia , Leucopenia/virologia , Pessoa de Meia-Idade , Pancitopenia/etiologia , Pancitopenia/virologia , Fatores de Tempo , Adulto JovemRESUMO
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. B symptoms were found in 14 (93.4%) patients. Increased lactate dehydrogenase (LDH) concentrations were detectable in 14 (93.4%) patients; tumors of 10 cm or more (bulky disease) were seen in 11 (73.3%). Enlarged cervical, supraclavicular, and axillary lymph nodes were found in 9 (60%) patients; lung involvement via extension in 9 (60%), and invasion into the pericardium in 5 (33.3%) and soft tissues of the anterior thoracic wall in (13.3%). There were no signs of involvement of extranodal organs at the moment of diagnosis. All the 15 patients received PCT according to the modified NHL-BFM-90 program: 4 to 6 courses depending on the response to the therapy; 10 (66.6%) and 5 (33.3%) patients had 4 and 6 courses, respectively; for consolidating purpose, 11 (78.5%) patients were prescribed radiotherapy applied to the mediastinum in a cumulative dose of 36 Gy due to the fact that they had a residual mass. RESULTS: Thirteen (86.6%) patients achieved a complete remission (CR). Primary PCT resistance was confirmed in one case. Another patient was stated to have near-complete remission. No recurrences were notified during the follow-up. The mean CR duration was 24.5 (range 2-49) months. CONCLUSION: DLBCLS with primary LN involvement is an individual nosological entity to be differentiated from primary mediastinal large B-cell lymphosarcoma. In most cases, DLBCLS shows signs of a poor prognosis, which makes it necessary to perform aggressive PCT.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfonodos , Linfoma Difuso de Grandes Células B/diagnóstico , Neoplasias do Mediastino/diagnóstico , Mediastino , Adolescente , Adulto , Idoso , Antígenos CD/imunologia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Diagnóstico Diferencial , Intervalo Livre de Doença , Feminino , Humanos , Linfonodos/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/imunologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Neoplasias do Mediastino/tratamento farmacológico , Neoplasias do Mediastino/imunologia , Neoplasias do Mediastino/patologia , Mediastino/diagnóstico por imagem , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Cintilografia , Ultrassonografia , Adulto JovemRESUMO
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.
Assuntos
Coristoma/diagnóstico , Hematopoese Extramedular , Doenças do Mediastino/diagnóstico , Esferocitose Hereditária/diagnóstico , Esferocitose Hereditária/cirurgia , Adulto , Coristoma/patologia , Coristoma/cirurgia , Diagnóstico Diferencial , Humanos , Masculino , Doenças do Mediastino/patologia , Doenças do Mediastino/cirurgia , Esferocitose Hereditária/patologia , Esplenectomia , Toracotomia , Tórax/patologia , Resultado do TratamentoRESUMO
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. CsA was used in a daily oral dose of 5 mg/kg. Its efficacy was evaluated following 3, 6, and 12 months. Actuarial survival was determined by the Kaplan-Meier method. RESULTS: The efficacy of CsA used as first- and second-line therapy was 56 and 55%, respectively; complete remissions were achieved in 19 and 20% of cases. Baseline refractory anemia (RA) transformed to RA with excess blasts (RAEB) in 31% of cases; baseline RAEB did to acute myeloid leukemia in 34%. Overall survival was significantly associated with bone marrow (BM) blast cell percentage (< 5% or > 5%; p = 0.0009), BM cellularity (hypoplasia and focal hypoplasia of hematopoiesis or BM hyperplasia; p = 0.03), focal polyclonal lymphoid infiltration in the BM (p = 0.01) and karyotype anomalies (low, moderate, and high risks; p = 0.001). CONCLUSION: CsA is the drug of choice in treating patients with MDS, including RA, RA with ringed sideroblasts, refractory cytopenia with multilineage dysplasia, with hypoplasia of hematopoiesis, with nodular polyclonal lymphoid infiltration in the BM, a normal karyotype or changes corresponding to a low or moderate IPSS risk.
Assuntos
Ciclosporina/uso terapêutico , Imunossupressores/uso terapêutico , Síndromes Mielodisplásicas/tratamento farmacológico , Adolescente , Adulto , Idoso , Medula Óssea/patologia , Aberrações Cromossômicas , Ciclosporina/administração & dosagem , Ciclosporina/efeitos adversos , Relação Dose-Resposta a Droga , Feminino , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Estimativa de Kaplan-Meier , Cariotipagem , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/imunologia , Síndromes Mielodisplásicas/mortalidade , Síndromes Mielodisplásicas/patologia , Prognóstico , Adulto JovemRESUMO
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.
Assuntos
Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Síndrome Hemolítico-Urêmica/diagnóstico , Síndrome Hemolítico-Urêmica/terapia , Adolescente , Anticoagulantes/administração & dosagem , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/complicações , Doença Catastrófica , Diagnóstico Diferencial , Síndrome Hemolítico-Urêmica/complicações , Humanos , Masculino , Troca Plasmática , Plasmaferese , Diálise Renal , Tromboembolia/prevenção & controle , Resultado do TratamentoRESUMO
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 1-1.4% of all the patients treated at the Hematology Research Center, Russian Academy of Medical Sciences. There is a need for surgery in 0.5-0.7% of all the patients admitted. In this group of patients, annual postoperative mortality is 12-16%. CONCLUSION: The routine algorithm for a diagnostic search in hematological patients with acute abdominal syndrome can lead to both hyperdiagnosis and unwarranted surgery, and incorrect choice of expectant policy as well.
Assuntos
Abdome Agudo/diagnóstico , Doenças Hematológicas/complicações , Laparotomia/métodos , Abdome Agudo/etiologia , Abdome Agudo/cirurgia , Adulto , Diagnóstico Diferencial , Técnicas de Diagnóstico do Sistema Digestório , Evolução Fatal , Feminino , Seguimentos , Doenças Hematológicas/diagnóstico , Humanos , Masculino , Estudos Retrospectivos , Síndrome , Adulto JovemRESUMO
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. The grade of myelofibrosis was estimated from the degree of bone marrow fibrosis as 0, 1, 2, and 3 and the clusters of MGKC were in accordance with degrees: 0, 1, and 2. MVD was studied from the absolute number of CD34-positive vascular structures. RESULTS: In patients with TP, fibrosis was defined as grade 0 and 1 in 23 (82%) and 5 (18%) cases, respectively. The content of reticulin fiber was in the normal range in 19 (95%) of the 20 patients with ET. The clusters of MGKC of grades 1 and 2 showed an even distribution among patients with ET and those with TP. Fibrosis was absent in all the patients (n = 6) with prefibrotic-stage primary myelofibrosis (PMF). The patients with PMF had high MVD values [6.5 (range 2.8-22)] than those with TP [4.0 (range 1.76-10.2)] or ET [3.7 (range 2-8.5)] and the controls [3.2 (range 2-4.1)] (p < 0.001) confirming that angiogenesis is uninvolved at the onset of disease in patients with ET and those with TP. The patients with prefibrotic-stage PMF had higher values [6.0 (range 4.8-10.6)] than those with ET [3. 7 (range 2-8.5)] (p < 0.001). This suggests that angiogenesis is an early sign preceding the development of fibrosis. CONCLUSION: Bone marrow angiogenesis assessment (from MVD measurements) may be an additional criterion for the diagnosis of disease evolution and an additional criterion between ET and PMF in a prefibrotic stage.
Assuntos
Medula Óssea/irrigação sanguínea , Microvasos/patologia , Transtornos Mieloproliferativos/diagnóstico , Adolescente , Adulto , Idoso , Doença Crônica , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neovascularização Patológica/patologia , Reprodutibilidade dos Testes , Adulto JovemRESUMO
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. The number of SURF6-positive cells was directly correlated with that of Ki-67-positive cells. The maximum level (47-67%) of SUR6-positive lymphoid cells was found in splenic lymphosarcomas and mantle cell lymphoma. The findings suggest that SURG6 protein may be of additional diagnostic and prognostic value.
Assuntos
Nucléolo Celular/metabolismo , Transtornos Linfoproliferativos/metabolismo , Proteínas Nucleares/metabolismo , Biomarcadores/metabolismo , Nucléolo Celular/patologia , Feminino , Regulação da Expressão Gênica , Humanos , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/patologia , MasculinoRESUMO
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). The diagnosis was made using morphological, histological, immunohistochemical methods with application of monoclonal antibodies to CD30, ALK, CD3, CD4, CD8, CD7, CD34, CD15, CD68, CD20, CD45RO, CD45RA. The patients were treated according to the protocol NHL BFM-90. ASHC was made in two patients with the disease stage IV. RESULTS: We obtained a complete remission in 12 of 13 patients, one woman died of infectious complications in the beginning of the treatment, one man had early recurrence 45 days after the end of the treatment with lethal outcome and disease progression. Two patients at stage IV and poor prognosis had undergone ASHC transplantation. They are now in remission for 5 and 12 months. CONCLUSION: ALCL ALK+ is characterized by an aggressive clinical course (11 of 13 patients had stage III-IV), high rate of extranodal lesions. Twelve patients achieved a complete remission, 11 (91.6%) of 12 patients are alive in observation median 27 months. We effectively used ASHC transplantation in the first-line treatment of 2 patients with stage IV of the disease and poor prognosis.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Proteínas Tirosina Quinases/metabolismo , Adolescente , Adulto , Quinase do Linfoma Anaplásico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Feminino , Humanos , Linfoma Anaplásico de Células Grandes/diagnóstico , Linfoma Anaplásico de Células Grandes/enzimologia , Linfoma Anaplásico de Células Grandes/patologia , Masculino , Estadiamento de Neoplasias , Receptores Proteína Tirosina Quinases , Indução de Remissão , Linfócitos T/imunologia , Linfócitos T/patologia , Adulto JovemRESUMO
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). In patients with mycosis fungoides (MF) the transformation was local while in those with Sezary's disease (SD) transformation of the tumor clone was determined by appearance of peripheral blood tumor cells rejuvenation. Morphological alterations were accompanied with immunomorphological parameters of progression. Most significant of them were high expression of the proliferative activity marker Ki-67 (10-70%), enhancement of activation (CD30, CD25), loss of some linear T-cell markers. Treatment of lymphosarcoma arising on the background of lingering MF or SD may combine two types of antitumor treatment--intensive and supporting because of coexistence of different clones of one tumor. CONCLUSION: Verification of skin T-cell lymphoma diagnosis and its transformation into lymphosarcoma must be based on the evidence from a number of examinations: histological, immunophenotyping, moleculobiological and clinical. Among criteria of the transformation, markers of lymphoproliferative activation are of great importance.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transformação Celular Neoplásica , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Linfócitos T/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Proliferação de Células , Feminino , Humanos , Antígeno Ki-67/biossíntese , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/patologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/tratamento farmacológico , Micose Fungoide/patologia , Síndrome de Sézary/tratamento farmacológico , Síndrome de Sézary/patologia , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia , Linfócitos T/imunologia , Adulto JovemRESUMO
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. Further follow-up is needed.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Humanos , L-Lactato Desidrogenase/sangue , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Indução de Remissão , Neoplasias Cutâneas/patologiaRESUMO
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.
Assuntos
Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/cirurgia , Células Dendríticas Foliculares/patologia , Hialina , Hiperplasia do Linfonodo Gigante/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
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.
Assuntos
Antineoplásicos/administração & dosagem , Linfoma de Burkitt/tratamento farmacológico , Adulto , Biópsia , Linfoma de Burkitt/patologia , Relação Dose-Resposta a Droga , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Indução de Remissão/métodos , Resultado do TratamentoRESUMO
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.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Linfoma de Burkitt/tratamento farmacológico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias Gástricas/tratamento farmacológico , Adolescente , Adulto , Idoso , Asparaginase/administração & dosagem , Linfoma de Burkitt/mortalidade , Linfoma de Burkitt/patologia , Ciclofosfamida/administração & dosagem , Daunorrubicina/administração & dosagem , Relação Dose-Resposta a Droga , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Estudos Prospectivos , Estudos Retrospectivos , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Vincristina/administração & dosagemRESUMO
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. 14 cases of 22 were negative by kinase of anaplastic lymphocytes (ALK-) and 8 were positive (ALK+). Mean age of ALK-ALCL patients was 39.6 +/- 4.1 years, of ALK+ALCL patients - 23.4 +/- 2.6 years. 14 patients were treated by the protocol NHL-BFM-90, 8 were initially treated with other schemes (CHOP, MACOP-B, BEACOPP and others). All 14 patients treated according to NHL-BFM-90 had ALCL stages III-IV with B-symptoms. 12 patients who completed treatment by the above protocol achieved complete remission after the forth course, 2 patients failed the treatment. Of 8 ALCL patients treated initially according to other schemes, a complete remission was achieved in 4 patients (2 had stage II). One of 4 patients with remission had recurrence. Four patients who had failed to achieve complete remission died of the disease progression. CONCLUSION: ALCL occurs more frequently in young and middle-aged patients. The disease has an aggressive course with rapid generalization. For such processes it is more preferable to use a modified protocol NHL-BFM-90.