RESUMO
Ocular adnexal marginal zone B-cell lymphomas (OAMZLs) arise in the connective tissues of the orbit or in the mucosa-associated lymphoid tissue of the conjunctiva. Here, we present the immunological and genetic analyses of 20 primary Chlamydia psittaci (Cp)-negative OAMZLs. Analysis of the immunoglobulin variable heavy chain (IgV(H)) gene usage demonstrated a significant preference for V(H)4-34. A combined analysis across all previously published OAMZLs confirmed that this is a general feature of OAMZL, in particular of the Cp-negative group. Our series of OAMZLs did not express the characteristic rheumatoid factor V(H)DJ(H) rearrangements that were previously found in salivary gland- and gastric-marginal zone B-cell lymphomas (MZBCLs). We did not detect the MZBCL-specific chromosomal translocations, t(11;18) API2-MALT1 (mucosa-associated lymphoid tissue1) and t(14;18) IgH/MALT1. Two cases contained a premature stop codon in the A20 gene (TNFAIP3) and one case harbored the activating MYD88 hotspot mutation L265P. Variable nuclear expression of BCL10, NFκB1 (p50) and NFκB2 (p52) suggests that other additional genetic abnormalities affecting the NFκB pathway exist within this group of lymphomas. OAMZL showed variable expression of the chemokine receptor CXCR3 and integrin α4ß7 by the tumor B cells, and low interferon-γ and interlukin-4 mRNA levels in the tissue, indicative of an inflammatory environment with features in between those previously found in cutaneous and other extranodal MZBCL. The strongly biased usage of V(H)4-34 in Cp-negative OAMZLs suggests involvement of a particular stimulatory (auto-) antigen in their development.
Assuntos
Rearranjo Gênico de Cadeia Pesada de Linfócito B , Cadeias Pesadas de Imunoglobulinas/genética , Região Variável de Imunoglobulina/genética , Inflamação/metabolismo , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma de Zona Marginal Tipo Células B/imunologia , Western Blotting , Núcleo Celular/metabolismo , Chlamydophila psittaci/genética , Chlamydophila psittaci/isolamento & purificação , DNA Bacteriano/genética , Humanos , Técnicas Imunoenzimáticas , Inflamação/genética , Inflamação/imunologia , Interferon gama/genética , Interferon gama/metabolismo , Interleucina-4/genética , Interleucina-4/metabolismo , Linfoma de Zona Marginal Tipo Células B/microbiologia , Mutação/genética , NF-kappa B/genética , NF-kappa B/metabolismo , Prognóstico , Psitacose/genética , Psitacose/imunologia , Psitacose/microbiologia , RNA Mensageiro/genética , Reação em Cadeia da Polimerase em Tempo Real , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Translocação GenéticaRESUMO
A male patient developed acute pulmonary histoplasmosis 2 weeks after bathing in the water falls of Wli, Ghana. Exposure to Histoplasma capsulatum was probably mediated through inhalation of an aerosol of water and guano from the large colony of fruit bats of the falls. More cases of acute pulmonary histoplasmosis can be expected.
Assuntos
Quirópteros/microbiologia , Histoplasmose/diagnóstico , Doença Aguda , Adulto , Animais , Anticorpos Antifúngicos/sangue , Diagnóstico Diferencial , Febre de Causa Desconhecida , Gana , Histoplasma/imunologia , Histoplasma/isolamento & purificação , Histoplasmose/diagnóstico por imagem , Humanos , Pulmão/microbiologia , Pulmão/patologia , Masculino , Radiografia Torácica , ViagemRESUMO
OBJECTIVE: The majority of ocular adnexal lymphomas are marginal zone lymphomas, which occur rarely in children. This case report describes a 6 years old child with a precursor B lymphoblastic lymphoma presenting in the ocular adnexa. The combination of multi-agent chemotherapy with adjuvant radiotherapy seems to be necessary in order to achieve a complete remission of this subtype of lymphoma's in ocular adnexa. DESIGN: Retrospective case study. METHOD: A review of the clinical, pathological, radiological findings and follow-up in a patient from the files available at our center, which were reviewed between the years 1974 and 2004.