Chronic lymphocytic leukaemia and concomitant relapsing polychondritis: a report on one treatment for the combined manifestation of two diseases

T. Bochtler, M. Hensel, H.-M. Lorenz, A. D. Ho and U. Mahlknecht

University of Heidelberg Medical Center, Department of Haematology/Oncology, Heidelberg, Germany

Correspondence to: U. Mahlknecht, University of Heidelberg Medical Centre, Department of Haematology/Oncology, Im Neuenheimer Feld 410, D-69120 Heidelberg, Germany. E-mail: Ulrich.Mahlknecht{at}med.uni-heidelberg.de

SIR, We report the case of a 60-yr-old patient who suffered from chronic lymphocytic leukaemia (CLL) with concomitant relapsing polychondritis. Polychondritic exacerbations were regularly observed with CLL progression and included bilateral painful swellings of the external ear cartilage and of the trachea, disseminated inflammatory polyarthritis, ocular inflammation and a palatal ulceration (Fig. 1). All manifestations of polychondritis and CLL responded well to chlorambucil/prednisone initially, and to cyclophosphamide during later stages of the disease, while the response to methotrexate was poor and treatment with bendamustin was complicated by tumour lysis syndrome and subsequent acute renal failure. Treatment with cyclophosphamide repeatedly induced remissions for both diseases, but was regularly accompanied by serious infectious complications. Relapsing polychondritis is rare and is generally diagnosed clinically if the patient develops at least three of the following signs: bilateral chondritis of the external ears, inflammatory polyarthritis, ocular inflammation, nasal chondritis, vestibular/auditory malfunction and respiratory tract chondritis. Notably, its 5-yr mortality may be as high as 30%, due to collapse of laryngeal and tracheal cartilaginous supporting structures, or cardiovascular involvement. Mild cases may respond to NSAIDs. More severe cases are usually treated with prednisone and may require additional immunosuppressive agents, e.g. cyclophosphamide [1].



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FIG. 1. Pain and a purple swelling of the external ear are the most common first clinical signs of relapsing polychondritis. The swelling may extend into the ear canal and induce ear infections, hearing loss and balance disturbances with vertigo and vomiting. During later stages of polychondritis, the destruction of supporting cartilaginous tissue may become visible as floppy ears occurring together with auditory and vestibular abnormalities, a flattened nose bridge (saddle nose) or, less frequently, with visual disturbances due to recurring inflammation, which may lead to blindness. The two pictures at the top (A) show our patient prior to treatment, while the pictures at the bottom (B) show our patient after one cycle of chlorambucil. This figure may be viewed in colour as supplementary data at Rheumatology Online.

 
The authors have declared no conflicts of interest.

References

  1. Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004;16:56–61.[CrossRef][Medline]
Accepted 1 April 2005





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