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Vol. 15. Issue 1.
Pages 121-127 (January - February 2009)
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Vol. 15. Issue 1.
Pages 121-127 (January - February 2009)
Caso Clínico/Case Report
Open Access
Imunocitoma IgA. A propósito de um caso clínico
Immunocytoma IgA. Case report
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Bebiana Conde1,
Corresponding author
bebianaconde@gmail.com

Centro Hospitalar de Trás-os-Montes e Alto Douro Av. da Noruega 5000 Vila Real
, Ana Fernandes2, Manuel Cunha3, Abel Afonso2
1 Interna do Internato Complementar de Pneumologia
2 Chefe de Serviço de Pneumologia
3 Assistente Graduado de Hematologia Clínica
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Bebiana Conde, Ana Fernandes, Manuel Cunha, Abel Afonso
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Resumo

O imunocitoma é um linfoma não Hodgkin (LNH) de células B, com evolução habitualmente indolente. Representa aproximadamente 1-3% dos LNH e atinge habitualmente adultos com mais de 50 anos, podendo manifestar-se por adenomegalias, hepatomegalia, esplenomegalia e linfocitose em 15 a 30% dos casos. Raramente tem envolvimento pulmonar. Com frequência ocorrendo picos monoclonais de imunoglobulinas, séricos, frequentemente IgM e raramente IgA.

Como exemplo desta patologia apresentamos o caso clínico de um doente do sexo masculino, 52 anos, com clínica de infecções respiratórias bacterianas de repetição, com necessidade de internamentos sucessivos, cuja investigação identificou um imunocitoma IgA, estádio IV.

Assumindo-se o diagnóstico de um linfoma indolente, decidiu-se iniciar terapêutica profiláctica com imunoglobulinas humanas poliespecíficas, tendo havido diminuição das infecções respiratórias. Posteriormente, a evidência de progressão do linfoma condicionou o início de poliquimioterapia, com o esquema ciclofosfamida, vincristina, prednisolona (CVP) e rituximab®, tendo-se alcançado uma resposta parcial, que se manteve durante dois anos.

Rev Port Pneumol 2009; XV (1): 121-127

Palavras-chave:
Imunocitoma
linfoma não Hodgkin
envolvimento pulmonar
Abstract

Immunocytoma is a non-Hodgkin’s indolent evolution B cell lymphoma. It accounts for approximately 1-3% of non-Hodgkin's limphomas and usually onsets in adults aged over 50 years old. It manifests as lymphadenopathy, splenomegaly, hepatomegaly and lymphcytosis in 15-30% of cases and is rarely seen with pulmonary involvement. Monocloncal peaks of serum immunoglobulin often occur. These are IgM and rarely IgA.

We present as an example a male patient aged 52 years old, with recurrent respiratory infections. Clinical work-up identified an immunocytoma IgA stage IV. Diagnosing an indolent lymphoma, we prophylactic polyspecific human immunoglobulin to treat the respiratory infection. Evidence of lymphoma progression leads us to prescribe combined cyclo-phosphamide (C), vincristine (V), prednisone (P) e rituximab (R) (CVP-R), which has obtained a partial response over two years.

Rev Port Pneumol 2009; XV (1): 121-127

Key-words:
Immunocytoma
non-Hodgkin’s lymphoma
pulmonary involvement
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