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Severe and recurrent episodes of bronchiolitis obliterans organising pneumonia associated with indolent CD4+ CD8+ T-cell leukaemia

Depts of ¹ Immunology, ² Pneumology, and ⁵ Haematology, Hospital de la Santa Creu i Sant Pau, Barcelona, ³ Institucio Catalana de Recerca i Estudis Avancats, Irsicaixa, Hospital Universitario Germans Trías i Pujol, Badalona, and ⁴ Molecular Cytogenetics Group, Spanish National Cancer Centre, Madrid, Spain.

CORRESPONDENCE: O. de la Calle-Martin, Dept of Immunology Dept, Hospital de Sant Pau, C/ Sant Antoni M^a Claret 167, 08025 Barcelona, Spain. Fax: 34 932919066. E-mail: odlcalle{at}santpau.es

Keywords: Bronchiolitis obliterans and organising pneumonia, CC chemokine receptor 6, CD103, double-positive T-cells, indolent T-prolymphocytic leukaemia, interstitial lung disease

Received: May 22, 2007
Accepted December 12, 2007

The present study describes an adult male who has had recurrent episodes of pulmonary infiltrates with severe acute respiratory failure over a period of 10 yrs. Clinical and pathological characteristics revealed bronchiolitis obliterans with organising pneumonia (BOOP) that responded dramatically to prednisone. BOOP is characterised by inflammation of the bronchioles and surrounding tissue in the lungs. It can mimic infectious pneumonia but diagnosis is suspected when there is no response to multiple antibiotic treatment, and blood and sputum cultures are negative for microorganisms.

A high proportion of double-positive (DP)-T-cells was detected in peripheral blood and in bronchoalveolar lavage, expressing CD4 and CD8β heterodimer with memory phenotype. These DP-T-lymphocytes expressed specific homing molecules that could explain their tropism to lung tissue, giving rise to the clinical symptoms. The patient did not present organomegaly, lymphadenopathy, lymphocytosis or other features of malignancy. However, T-cell receptor Vβ chain analysis indicated clonal rearrangement, and cytogenetic studies displayed chromosomic alterations that were similar to clonal proliferation observed in ataxia-telangiectasia and T-prolymphocytic leukaemia.

The findings suggest a smouldering form of lymphoproliferation, the first sign of which was bronchiolitis obliterans organising pneumonia requiring constant corticoid treatment.

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