RESUMO
Stage IV disseminated non-Hodgkin lymphomas show kidney involvement with frequency; on the contrary, primary kidney lymphoma, as the sole presenting feature, is a very rare disease. Non-Hodgkin histological pattern, atypical symptoms, marked discrepancy between parenchymal involvement and impairment of renal function are the main features of this tumor. A case-report of a primary kidney non-Hodgkin lymphoma will be described; a short review of literature on this subject will be discussed.
Assuntos
Neoplasias Renais/patologia , Leucemia Linfocítica Crônica de Células B/patologia , Linfoma não Hodgkin/patologia , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada , Feminino , Humanos , Neoplasias Renais/diagnóstico , Neoplasias Renais/terapia , Leucemia Linfocítica Crônica de Células B/terapia , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/terapia , NefrectomiaRESUMO
Malignant histiocytosis (MH) is a rare and severe disease caused by malignant histiocyte degeneration in the sinuses of the reticuloendothelial system. The clinical picture presents fever, wasting, enlargement of the liver and spleen and lymphoadenopathy. Diagnosis is based on histological criteria and it may prove very difficult to differentiate between MH and malignant lymphomas, acute monocytic leukemia and the histiocytoses considered benign. The main features of the disease are described with comments on two personally experienced clinical cases. Emphasis is placed on the leukaemic aspect, the involvement of the CNS, the association, in one case, with sarcoidosis and the poor prognosis.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Sarcoma Histiocítico/patologia , Adulto , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Feminino , Histiócitos/patologia , Sarcoma Histiocítico/complicações , Sarcoma Histiocítico/tratamento farmacológico , Humanos , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Vincristina/uso terapêuticoRESUMO
Splenic lymphoma with circulating villous lymphocytes is a rare B-lymphoproliferative disorder of the elderly which has been only recently defined. Clinical features are spleen enlargement of various degree without lymphadenopathy and an indolent course, with a long survival, in most cases. Absolute lymphocytosis is present; atypical circulating lymphocytes show a medium or large size, a small prominent nucleolus and a few short and thin cytoplasmic protrusions and projections (villi), which are distributed at one or both poles of cell surface. Reaction for tartrate-resistant acid phosphatase is almost always negative. Immunological markers are as follows: CD 19+, CD 20+, CD 22+, CD 11c+/-, CD 5-, CD 23-, CD 25-, HLA DR+, SmIg+. Differential diagnosis with other chronic lymphoproliferative disorders, particularly chronic lymphocytic leukemia, hairy cell leukemia, prolymphocytic leukemia, follicular and mantle-cell lymphoma in leukemic phase, is based on clinical and immunocytomorphologic criteria. Bone marrow biopsy shows involvement of different degree and pattern; splenic involvement mostly occurs in the white pulp; hepatic nodules in portal areas may be present. Cytogenetic alterations are often present but not specific, such as increased serum LDH and monoclonal gammopathy. No therapy should be made in asymptomatic patients. In case of systemic symptoms, symptomatic splenomegaly or cytopenias, treatment may consist on splenectomy, splenic irradiation or alkylating agents. A case of splenic lymphoma with circulating villous lymphocytes is reported; differential diagnosis, particularly with other B lymphoproliferative disorders, is discussed.
Assuntos
Linfócitos/patologia , Linfoma de Células B/patologia , Neoplasias Esplênicas/patologia , Idoso , Idoso de 80 Anos ou mais , Humanos , Linfoma de Células B/sangue , Masculino , Neoplasias Esplênicas/sangueRESUMO
Extraskeletal involvement of the testis during multiple myeloma is a rare disease; primary extraosseus myeloma of the testis is even more unusual, and shows progression to typical multiple myeloma after orchiectomy in most cases. A case-report of a multiple myeloma with testicular involvement is discussed; radiotherapy was performed successfully, instead of surgery, but diffuse skeletal lesions showed progression in spite of chemotherapy. A short review of the literature on this subject is made.
Assuntos
Mieloma Múltiplo , Neoplasias Testiculares , Humanos , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Neoplasias Testiculares/diagnósticoRESUMO
Primary cardiac lymphoma is classically defined as an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. However, over the last few years, this definition has been extended to include other localizations on condition that these are clearly less important then a cardiac site, that must remain the first, during the illness course, and the most important for its entity. PCL is extremely rare in immunocompetent patients, accounting for 1.3% of all cardiac tumours and 0.5% of all extranodal lymphomas, but it has been encountered with increasing frequency in patients with AIDS or other severe immunodepressive syndromes. PCL is difficult to diagnose, especially during the early stage of the disease, because of its non-specific clinical manifestations, the limited possibility of using non-invasive diagnostic techniques, and difficulties or delays in applying invasive methods. The malignancy of its histotypes and its delicate location are responsible for its rapid and frequently unfavourable evolution. Successful treatment, which is mainly based on anthracycline-containing polychemotherapies, is heavily dependent on an early diagnosis. After a general review of the literature, the authors describe the clinical case of a patient with a PCL that had a secondary central nervous system location, treated with polychemotherapy and autologous peripheral blood stem cell transplantation. Emphasis is placed on the fact that it is more difficult to eradicate the disease from the central nervous system than from the heart.