Bronchial associated lymphoid tissue (BALT) lymphoma presenting as chronic lung sepsis
- Anne Ingrid Hoeritzauer1,
- Laskshmi Venkatraman2,
- Kieran Mc Manus3,
- Paul Kettle4,
- Shatrugan Sah2,
- Stuart Elborn1
- 1Centre for Infection and Immunity, Queens University Belfast, City Hospital, Belfast, BT97AB, UK
- 2Royal Victoria Hospital, Pathology Department, 274 Grosvenor Road, Belfast, Co. Antrim BT12 6BA, UK
- 3Royal Victoria Hospital, Cardiothoracics, 274 Grosvenor Road, Belfast, Co. Antrim BT12 6BA, UK
- 4Belfast City Hospital, Haematology, Lisburn Road, Belfast, Co. Antrim BT9 7AB, UK
- Stuart Elborn,
- Published 7 September 2009
A 58-year-old woman was referred from her general practitioner to the respiratory clinic with a 2 year history of recurrent pulmonary infections, mucus hypersecretion and right lobe consolidation following a severe pneumonic illness in 2006. She had no significant risk factors for respiratory disease. Chest computed tomography showed an air bronchogram and right lower lobe consolidation. On initial routine investigation IgA and IgG were normal; however, a discrete IgM paraprotein band in the mid gamma region was seen on serum electrophoresis. She was referred for haematological investigations. Bone marrow biopsy was positive for monoclonal lymphoplasmocytoid B cells and the patient was diagnosed with Waldenström’s macroglobulinaemia. Due to recurrent infections and an unclear diagnosis of the lung process, a right lower lobectomy and wedge resection of the middle lobe was performed. This showed bronchial associated lymphoid tissue lymphoma arising in the marginal zone. She has been well since surgery with no further respiratory infections.
This case highlights the importance of investigating the aetiology of patients with symptoms of bronchiectasis, particularly when computed tomography (CT) scan appearances are unusual.
Bronchial associated lymphoid tissue (BALT) lymphoma is often described as an indolent condition presenting with cough, dyspnoea or as an incidental radiological finding. In this case report, however, it presented with extensive damage to a lobe of the lung and recurrent lung sepsis.
Surgical management of this woman’s condition led to vastly improved quality of life and long term resolution of her recurrent chest sepsis.
Other case reports of BALT lymphoma associated with chronic lung sepsis have demonstrated Mycobacterium tuberculosis and Mycobacterium avium infection. Our patient grew only Haemophilus influenzae and Staphylococcus aureus.
At present no clear and substantiated guidelines exist for treatment of BALT lymphoma.
A 58-year-old woman presented to the respiratory clinic with recurrent pulmonary infections and continuous right lower lobe consolidation on x ray following a severe pneumonic infection 2 years previously.
Her symptoms were increased shortness of breath, wheeze, and production of sputum of approximately one third of a cup per day. Physical examination demonstrated a respiratory rate of 12, decreased air entry at the right base, and some focal crepitations. No obvious peripheral lymphadenopathy was present. She had no history of M tuberculosis, smoking, exposure to asbestos, birds or dust.
Chest x ray showed right middle and lower lobe consolidation with a small volume pleural effusion (fig 1). Spirometry was normal. Bronchoscopy demonstrated narrowed and chronically fibrosed right lower lobe airways. The bronchial washing was positive for H influenza and S aureus, and negative for acid fast bacilli and malignant cells. Dense consolidate change and an air bronchogram in the right lower lobe and a number of opacities in the middle lobe were seen on high resolution chest CT scan (fig 2). These findings were not in keeping with the preliminary diagnosis of bronchiectasis.
At initial presentation immunoglobulins were checked to rule out inherited immunodeficiency disorders causing recurrent lower respiratory infections culminating in bronchiectasis. IgG and IgA were normal; however, IgM was elevated at 9.03 g/l and a discrete band, size 7 g/L, in the mid gamma region was seen on serum electrophoresis. Four months later both IgM and the size of the para protein band were found to have increased; there was no immune paresis. Urinary Bence Jones protein was negative.
A CT scan of the abdomen and pelvis and a repeat CT scan of the chest did not demonstrate any other abnormalities.
She was referred to haematology due to the increasing immunoglobulins. Small aggregates of mature lymphoplasmocytoid lymphocytes were found in the bone marrow biopsy. Immunophenotypically, these were clonal B lymphocytes expressing IgM, CD21, CD20 and bcl2 and negative for CD5 and CD10. The patient was diagnosed with Waldenstrom’s macroglobulinaemia.
She remained under the care of the respiratory team for her recurrent chest sepsis. A core lung biopsy was non-diagnostic.
Due to recurrent infections in the right lower lobe and the lack of a clear diagnosis linking the respiratory complaints with the imaging and other pathological findings, a right lower lobectomy and wedge resection of the middle lobe were performed. Intraoperatively the lower lobe was found to be solid. Loco-regional lymph nodes were enlarged and frozen section indicated a lymphomatous process.
The pathology from the lymph nodes and the right middle and lower lobes were in keeping with bronchial associated lymphoid tissue in marginal zone B cell non-Hodgkin lymphoma (fig 3).
OUTCOME AND FOLLOW-UP
The patient recovered well postoperatively and has had no further respiratory infections since the operation 10 months ago.
BALT lymphoma is a marginal zone lymphoma which originates from acquired lymphoid tissue in the lung and accounts for <1% of all lymphomas.1
Although there is no clear aetiology for BALT lymphoma the relationship between smoking and BALT lymphoma and a possible link with autoimmune conditions supports the current hypothesis that BALT lymphoma develops in a similar way to MALT lymphoma.2–4 Our patient had no risk factors for chronic lung inflammation such as a history of M tuberculosis, immunodeficiency, childhood illness with whooping cough, measles or pneumonia or inflammatory diseases—for example, rheumatoid arthritis or Crohn’s disease. The patient’s recurrent chest infections were secondary to damage caused by BALT lymphoma which is interesting as the usual presentation of BALT lymphoma is non-specific respiratory complaints such as cough, chest pain or dyspnoea2 or is an incidental radiological finding.5
This is the only case report of bronchiectasis symptoms without corresponding CT findings secondary to an infected sump of BALT lymphoma. Two cases of BALT lymphoma and associated bronchiectasis have been published. Both of these were associated with infection—M tuberculosis and M avium, respectively.6,7
In our patient there was no evidence of lung damage secondary to a destructive infective process and no evidence of bronchiectasis on CT despite her characteristic symptoms of mucous hypersecretion and recurrent infections. Instead the patient’s chest CT showed dense consolidation and an air bronchogram. This is in keeping with the CT findings in BALT lymphoma.3 Also commonly seen is the “tree-in-bud” sign which is due to small centrilobular or branching nodules and airspace consolidation.
Surprisingly a recent review of CT findings in patients with BALT lymphoma found that three out of the 21 patients’ CTs reviewed had evidence of bronchiolitis/bronchiectasis.3 Unfortunately no details of whether these patients had corresponding symptoms of bronchiectasis are available. Bearing in mind that different treatments are required for these two conditions and the possibility of greatly increased quality of life associated with successful treatment of BALT in patients with symptoms of bronchiectasis, this case report highlights the importance of investigating the aetiology of patients with symptoms of bronchiectasis, particularly when CT scan appearances are abnormal.
The initial diagnosis of Waldenstrom’s macroglobulinaemia, a low grade lymphoplasmacytic lymphoma, was based upon the presence of increased IgM paraprotein, the indolent course of the disease, and the presence of lymphoplasmocytoid lymphocytes on bone marrow biopsy. This diagnosis was made before the lobectomy and wedge resection following which histology confirmed BALT lymphoma. Differentiation between Waldenstrom’s macroglobulinaemia and BALT lymphoma can be difficult as there are several similarities. In up to one quarter of patients with non-gastrointestinal primary sites of MALT lymphomas, disseminated disease is present in the form of infiltration into non-mucosal sites such as bone marrow or multiple mucosal sites.8 A third of the patients with MALT/marginal zone B cell lymphoma demonstrate plasma cell differentiation and a serum M protein band. In the absence of clinical–pathological correlation a correct diagnosis may be difficult, particularly if all the lesional tissues are not sampled. In this case histology from the lymph nodes and lung confirmed a pulmonary marginal zone lymphoma and provided a unifying diagnosis.
Before the histology report the diagnosis of Waldenstrom’s macroglobulinaemia was undermined by the chronic lung lesion as only 3–5% of patients with Waldenstrom’s macroglobulinaemia have pulmonary involvement, usually in the form of nodules, pleural effusion or diffuse infiltrates.9
The main treatment for single lobe BALT lymphoma is lobectomy as occurred with our patient. There is lack of evidence that adjuvant treatment with chemo- or radiotherapy improves mortality in these low grade lymphomas. Chemotherapy is usually used in patients who have BALT lymphomas affecting both lungs or multiple sights unilaterally. Radiotherapy is uncommonly used, although case reports do demonstrate that it may be useful as a primary treatment for multilobe BALT lymphoma.2 Outcomes are excellent, irrespective of treatment type, with approximately 90% 5 year survival.1,4,5,10
The aetiology of patients with symptoms of bronchiectasis should be investigated thoroughly, particularly when CT scan appearances are unusual.
In BALT lymphoma affecting a single lobe, surgery provides optimal treatment and can greatly improve quality of life.
Further research is required to provide definitive guidelines for treatment of BALT lymphoma.
In chronic chest sepsis without a definitive infective cause, bronchoalveolar carcinoma and lymphoma should be ruled out.
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication