Assuntos
Leucemia Linfocítica Crônica de Células B , Mutação , Proteínas de Neoplasias/genética , Receptor Notch1/genética , Intervalo Livre de Doença , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/genética , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Taxa de SobrevidaRESUMO
AIMS: Hairy cell leukaemia (HCL) is an indolent B-cell neoplasm that primarily involves the peripheral blood, bone marrow, and spleen. Rarely, patients with HCL present with a lymphoma-like clinicopathological picture mimicking other types of low-grade B-cell lymphoma. Distinguishing HCL from other types of lymphoma is essential, given the different treatments and prognoses. We report two cases of the lymphomatous variant of HCL to draw attention to this unusual presentation. METHODS AND RESULTS: Two cases of HCL presented initially as salivary gland and soft tissue masses. Morphologically, the neoplastic cells showed a typical fried-egg or monocytoid appearance with reniform nuclei, and were positive for CD11c, CD20, CD25, CD103, annexin A1, and cyclin D1. Both cases were positive for the BRAF V600E mutation, as shown by pyrosequencing and mutation-specific immunohistochemistry. The diagnosis was challenging in both patients, and one patient experienced 5 years of disease relapse and progression after treatment with multiple lymphoma-type regimens before the final diagnosis of HCL was established and complete remission was achieved following cladribine therapy. CONCLUSIONS: HCL can very rarely present as an extranodal mass. Being aware of this unusual presentation of HCL is essential to avoid diagnostic confusion with other types of low-grade B-cell lymphoma and to facilitate appropriate therapy.
Assuntos
Diagnóstico Diferencial , Leucemia de Células Pilosas/patologia , Linfoma de Células B/patologia , Adulto , Erros de Diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias de Tecidos Moles/patologia , Neoplasias da Glândula Submandibular/patologiaRESUMO
AIMS: Cancer cell-derived immunoglobulin (Ig)G (cancer-IgG) has been found to be involved in the pathogenesis and progression of many cancers, including lung cancer. The aim of the present study was to investigate the relationship between cancer-IgG expression in lung adenocarcinoma (ADC) and clinicopathological characteristics and clinical outcome. METHODS AND RESULTS: Immunohistochemical analysis was performed using an RP215 monoclonal antibody to determine cancer-IgG expression in 140 lung ADC patients. Cell migration and invasion were analysed in A549 cell line after short interfering RNA (siRNA) knockdown of IgG and cell sorting by flow cytometry. Our results show that RP215 immunostaining score is correlated significantly with local invasion (P < 0.05) and tumour differentiation (P < 0.05) in ADC. Moreover, RP215 staining was significantly higher in metastatic tumours than in primary tumours (P < 0.0001). The knockdown of IgG resulted in a reduction of cell migration and invasion. In contrast, RP215-positive cells displayed greater migration and invasion ability than RP215-negative cells. Additionally, a higher RP215 immunostaining score was associated significantly with poor prognosis. CONCLUSIONS: RP215 staining is correlated strongly with differentiation, local invasion, metastasis and clinical outcome of patients with lung ADC. Our results suggest that RP215 can serve as a biomarker for prognosis of lung ADC.
