RESUMO
A 48-year-old woman was hospitalized because of severe thrombocytopenia, leg edema, and fever. Intravenous immunoglobulin therapy was administered, but no efficacy was obtained. Her bone marrow was dry-tap, and fibrosis was found in the biopsy specimens. A positron emission tomographic study showed FDG-avid lymphadenopathy and hepatomegaly. Biopsy specimens of axillary lymph nodes showed Castleman's disease-like findings. Since she then developed severe proteinuria and massive pleural effusion, steroid therapy was started, providing temporary relief of symptoms other than the thrombocytopenia. However, rapid worsening of her general condition prompted us to attempt rituximab as salvage therapy. The pleural effusion, edema, and proteinuria disappeared soon after starting rituximab administration. Platelet counts also normalized and fibrosis of the bone marrow showed amelioration. Recently, a variant of multicentric Castleman's disease, termed the TAFRO syndrome, has been proposed, and our patient's features fit the diagnosis of this syndrome. Rituximab might be considered as a therapeutic option in such cases.
Assuntos
Anticorpos Monoclonais Murinos/administração & dosagem , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Medula Óssea/patologia , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/patologia , Esquema de Medicação , Feminino , Humanos , Linfonodos/patologia , Pessoa de Meia-Idade , Contagem de Plaquetas , Rituximab , Terapia de Salvação , Síndrome , Resultado do TratamentoRESUMO
AIMS: To evaluate the role of tumour-associated macrophages (TAMs) of the M1 and M2 types in the behaviour of diffuse large B-cell lymphoma (DLBCL). METHODS AND RESULTS: Double immunohistochemical staining of HLA-DR/CD68 (M1) or CD163/CD68 (M2) was performed in 101 cases of DLBCL. CD68+ cells represent the total number of TAMs. The average number of double-positive cells was counted, and the cut-off value was set at the mean number of counts, i.e. 30.7 and 27.0 for M1 TAMs and M2 TAMs, respectively. That for total TAMs was set at the 90th percentile number of total counts, i.e. 132.3. Cases were categorized into three pairs: high (34 cases) and low (67 cases) M1 TAM groups, high (39 cases) and low (62 cases) M2 TAM groups, and high (10 cases) and low (91 cases) total TAM groups. The difference in overall survival rates was statistically significant between the high and low M2 TAM groups (P < 0.01) and between the high and low total TAM groups (P < 0.05). Multivariate analysis revealed that the presence of a bulky mass and a higher number of M2 TAMs were significant factors for poor prognosis (P < 0.05). CONCLUSIONS: Estimation of specific type of macrophages, of the M1 and M2 types, is superior to the estimation of TAMs as a whole (CD68+ cells) for prediction of the prognosis of DLBCL patients.
Assuntos
Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/patologia , Macrófagos/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Feminino , Humanos , Japão , Linfoma Difuso de Grandes Células B/classificação , Macrófagos/imunologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Adulto JovemRESUMO
A 48-year old man was admitted with idiopathic fever, and subsequently diagnosed as having hemophagocytic lymphohistiocytosis (HLH). Though an extensive series of laboratory examinations failed to demonstrate an apparent etiology, empirical use of steroids achieved remission. About two years later, the patient developed Pneumocystis Jiroveci pneumonia and was diagnosed as HIV-positive. Based on this case, HIV-screening tests would be performed whenever we encounter HLH in Japan, where the number of HIV-positive patients is increasing.
Assuntos
Infecções por HIV/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Infecções por HIV/diagnóstico , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pneumonia por Pneumocystis/diagnóstico , Pneumonia por Pneumocystis/etiologia , Prednisolona/administração & dosagemRESUMO
We describe a 69-year-old man with refractory relapsing thrombotic thrombocytopenic purpura (TTP) successfully treated with rituximab. The patient had once been successfully treated with plasmapheresis and vincristine, but he had relapsed after a short period. Although plasmapheresis, vincristine, and splenectomy could not achieve a consistent elevation of the platelet count, rituximab administration provided sustained remission for more than 7 months. Rituximab should be considered as a therapeutic alternative for refractory TTP.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Proteínas ADAM , Proteína ADAMTS13 , Idoso , Anticorpos Monoclonais Murinos , Autoanticorpos/sangue , Intervalo Livre de Doença , Humanos , Masculino , Metaloendopeptidases/imunologia , Púrpura Trombocitopênica Trombótica/imunologia , Indução de Remissão , Rituximab , Terapia de SalvaçãoRESUMO
Cytoplasmic vacuolation was seen in patients with a variety of plasma cell dyscrasia. We report here a case of leukemic non-secretory multiple myeloma with many azurophilic granules. By electron microscopy, the myeloma cells were found to have a well-developed rough endoplasmic reticulum and a clear Golgi apparatus, and azurophilic granules were identified as phagocytic vacuoles. In addition to myeloma markers, the cells were positive for B cell-associated, myeloid and stem cell markers. The diagnosis is difficult because of its misleading morphology and unusual surface markers. We consider that electron microscopy is useful for the identification of cell lineage in this disorder.
Assuntos
Complexo de Golgi/ultraestrutura , Mieloma Múltiplo/patologia , Mieloma Múltiplo/ultraestrutura , Idoso , Idoso de 80 Anos ou mais , Células da Medula Óssea/patologia , Células da Medula Óssea/ultraestrutura , Feminino , Citometria de Fluxo , Seguimentos , Humanos , Imuno-Histoquímica , Microscopia Eletrônica/métodos , Medição de Risco , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Vacúolos/ultraestruturaRESUMO
To evaluate roles of tumor-associated macrophages (TAMs) for prognosis of classical Hodgkin lymphoma (CHL). Expression of markers for TAMs, CD68, HLA-DR, CD163, HLA-DR/CD68 (M1), and CD163/CD68 (M2) was immunohistochemically examined in 82 cases with CHL. Positively stained cells were counted and correlation of number of TAMs and patients' survival time was analyzed. Number of CD163+ cells and M2 cells was significantly correlated with shorter overall survival (P < 0.05), while it was marginally significant for CD68+ cells (P = 0.0827). HLA-DR + cells and M1 cells showed no significant correlation with overall survival. When confined to mixed cellularity subtype, number of M1 cells was correlated with favorable prognosis (P < 0.05), while M2 did not (P = 0.7). Older age and male sex were unfavorable factors for prognosis. At multivariate analysis, number of CD163+ cells, M2+ cells, and age were independent factors for poor overall survival (P = 0.03, 0.02, and 0.01, respectively). CD163+ cells and M2 cells might work to be tumor promotive in CHL. M1 cells might be tumor suppressive in mixed cellularity type.
Assuntos
Doença de Hodgkin/imunologia , Doença de Hodgkin/patologia , Macrófagos/imunologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Imuno-Histoquímica , Estimativa de Kaplan-Meier , Macrófagos/metabolismo , Macrófagos/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Adulto JovemRESUMO
We report a 39-year-old man with intravascular large B-cell lymphoma (IVLBCL) who had been treated as a case with pulmonary arterial hypertension (PAH) for one year. After he became worse, diffuse pulmonary (18)F-fluorodeoxyglucose (FDG) uptake in positron emission tomography (PET) suggested the existence of IVLBCL in the lung showing normal CT images. The diagnosis was confirmed with random transbronchial lung biopsy, and he was then successfully treated. Since IVLBCL presenting PAH has been rare and is difficult to diagnose, early application of FDG-PET may provide early recognition of the disorder, leading to a better outcome.