M Felizardo¹

A C Mendes²

A Fernandes³

P Campos⁴

V Magalhães²

I Correia²

A Pignatelli⁵

C Ferreira⁶

A Bugalho de Almeida⁸

Resumo

O linfoma intravascular é uma forma muito rara de linfoma não Hodgkin de células grandes B. Caracteriza-se pela proliferação celular tumoral de linfócitos limitada aos pequenos vasos, particularmente nos capilares. Apresentamos o caso de uma doente de 54 anos, não fumadora, que foi admitida no nosso hospital para investigação de um quadro com quatro meses de evolução de febre, sudorese nocturna, emagrecimento não quantificado e dispneia progressiva. Ao exame objectivo apresentava-se febril, taquicárdica e polipneica. Analiticamente, destacava-se anemia, leucocitose e LDH elevada. Gasometria arterial - FiO2 1 l/m: PaO2-63,6 mm Hg. A telerradiografia de tórax revelava infiltado intersticial difuso. Foram excluídas todas as causas de febre de origem indeterminada. O diagnóstico foi realizado por biópsia pulmonar cirúrgica e foi prescrita terapêutica citostática combinada e rituximab com boa resposta clínica. Relatamos o caso pela dificuldade diagnóstica e pela boa resposta à terapêutica.

Palavras-chave: Linfoma, intravascular, febre, pulmonar.

Intravascular pulmonary lymphoma with good response to treatment. A case report

Intravascular lymphoma is a very rare form of large B cell non-Hodgkin’s lymphoma, characterised by the presence of lymphoma cells in the lumina of small vessels only, particulary in the capillaries. We report a 54 year-old female non-smoker, admitted to hospital for further examination of a four month long clinical condition involving high fever, night sweats, unqualified weight loss and progressive dyspnea. Patient’s temperature was 38.5 ºC, pulse 100/min and respiratory 22 cycles/min. Patient’s haemoglobin was 9.4g/dL, she had leukocytosis, elevated LDH and arterial blood gas analysis with moderate hypoxaemia (FiO2 1l/m: PaO2-63.6 mm Hg). Chest X-ray revealed diffuse interstitial changes. All the possible causes of unknown origin fever were excluded. Diagnosis was made through lung biopsy and treatment with combined chemotherapy and rituximab was prescribed leading to a 48 hours clinical remission. We present this case to show how difficult this diagnosis can be and how a good response to therapy is possible.

Key-words: Lymphoma, intravascular, fever, pulmonary.

Texto completo disponível apenas em PDF.

Full text only available in PDF format.

Bilbiografia / Bibliography

1. Takamura K, Nasuhara Y, Mishina T, Matsuda T, Nishimura M, Kawakami Y, Fujita M, Mikuni C, Yamashiro K. Intravascular lymphomatosis diagnosed by transbronchial lung biopsy. Eur Respir J 1997; 10: 955 -7.        [ Links ]

2. Sastre JM, Folgado R, Burges O, Zaragoza MD, Oliver V. Linfomatosis intravascular de presentación pulmonar. An Med Interna 2001; 18 (6): 319 -22.

3. Fiegl M, Greil R, Pechlaner C, Krugmann J, Dirnhofer S. Intravascular large B -cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. Annals Oncol 2002; 13: 1503 -6.

4. Yamagata T, Okamoto Y, Ota K, Katayama N, Tsuda T, Yukawa S. A case of pulmonary intravascular lymphomatosis diagnosed by thoracoscopic lung biopsy. Respiration 2003; 70: 414 -8.

5. Ferreri A. JM, Campo E, Seymour JF, Willemze R, Ilariucci F, Ambrosetti A, Zucca E, Rossi G, Lopez -Guillermo A, Pavlovsky MA, Geerts M, Candoni A, Lestani M, Asioli S, Milani M, Pins MA, Pileri S, Facchetti F, Cavalli F, Ponzoni M. Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special enphasis on the ‘cutaneous variant’. British J Haematol 2004; 127: 173 -83.

6. Williams G, Foyle A, White D, Greer W, Burrell S, Couban S. Intravascular T -cell lymphoma with bowel involvement: case report and literature review. Am J Haematol 2005; 78: 207 -11.

7. Martusewicz -Boros M, Wiatr E, Radzikowska E, Roszkowski -Sliz K. Pulmonary intravascular large b –cell lymphoma as a cause of severe hipoxemia. J Clin Oncol 2007;25: 2137 -9.

8. Ko YH, Han JH, Go JH, et al. Intravascular lymphomatosis: a clinicopathologic study of two cases presenting as an intersticial lung disease. Histopathology 1997; 31: 555 -62.

² Assistente Hospitalar Graduada de Pneumologia / Consultant, Pulmonology specialist

³ Director do Serviço de Anatomia Patológica. Professor Graduado da Faculdade de Medicina de Lisboa / Director, Anatomo-Pathology Unit. Specialist professor, Lisbon University School of Medicine

⁴ Assistente Hospitalar Graduada de Imagiologia / Consultant, Radiology specialist

⁵ Interna do Internato Complementar de Anatomia Patológica / Resident, Specialist training in Anatomo-Pathology

⁶ Assistente Hospitalar de Anatomia Patológica / Consultant, Anatomo-Pathology

⁷ Chefe de Serviço de Pneumologia. Assistente convidado da Faculdade de Medicina de Lisboa / Head, Pulmonology Unit. Guest assistant professor Lisbon University School of Medicine

⁸ Director do Serviço de Pneumologia. Professor da Faculdade de Medicina de Lisboa / Director, Pulmonology Unit, Specialist professor, Lisbon University School of Medicine