Journal of Postgraduate Medicine
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Year : 2003  |  Volume : 49  |  Issue : 3  |  Page : 252-253  

Diffuse Large B Cell Lymphoma in a Patient with Hypocomplementemic Urticarial Vasculitis

JM Calvo-Romero 
 Department of Internal Medicine, Hospital de Zafra, Zafra (Badajoz), Spain

Correspondence Address:
J M Calvo-Romero
Sergio Luna 15, 2ºA. 06010 Badajoz


Hypocomplementemic urticarial vasculitis (HUV) is known to be associated with malignancies. Urticarial vasculitis has been linked to lymphomas, but to our knowledge, the association of HUV and non-Hodgkin lymphoma has not been described so far. A patient with HUV who developed 10 years later a diffuse large B cell lymphoma is reported here.

How to cite this article:
Calvo-Romero J M. Diffuse Large B Cell Lymphoma in a Patient with Hypocomplementemic Urticarial Vasculitis .J Postgrad Med 2003;49:252-253

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Calvo-Romero J M. Diffuse Large B Cell Lymphoma in a Patient with Hypocomplementemic Urticarial Vasculitis . J Postgrad Med [serial online] 2003 [cited 2022 Nov 27 ];49:252-253
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Urticarial vasculitis is known to be associated with a variety of disorders including viral infections, Henoch-Schönlein purpura, systemic lupus erythematosus, Sjögren's syndrome, IgM paraproteinaemia (Schnitzler's syndrome) and malignancies.[1],[2],[3] No underlying disease can be detected in most cases of urticarial vasculitis. Hypocomplementemic urticarial vasculitis (HUV) is an uncommon autoimmune disorder and little is known about its pathogenesis.[3],[4] A few case reports have described an association between HUV and malignancies.[3],[5],[6],[7] However, to our knowledge, the association of HUV and non-Hodgkin lymphoma has not been described so far. A patient with HUV who developed a diffuse large B cell lymphoma is reported here.


   Case History

A 60-year-old man was admitted to our hospital because of high-grade fever, lymphadenopathy and splenomegaly. He had been diagnosed to have HUV 10 years earlier. He had chronic urticaria and skin biopsy demonstrated fibrinoid necrosis of the vessel wall, extravasation of red blood cells, leukocytoclasia, and a mixed infiltrate of neutrophils and lymphocytes suggestive of leukocytoclastic vasculitis. He also had low serum C3, C4 and C1q levels. There was no history of angioedema, xerostomia, keratoconjuctivitis sicca, Raynaud's phenomenon, arthritis, pulmonary or renal disease. He had received prednisone intermittently for the treatment of HUV.

At presentation, he was receiving prednisone (15 mg per day). On examination he was febrile (39.10C) and he had cervical and supraclavicular lymphadenopathy, splenomegaly and residual hyperpigmented cutaneous lesions. Laboratory investigation revealed: haemoglobin concentration of 11.9 g/dl, ESR of 76 mm/hour, serum C-reactive protein of 124 mg/dl, serum lactate dehydrogenase of 673 U/l, serum C3 of 59.1 mg/dl (normal range 50-90 mg/dl), serum C4 of 2.1 mg/dl (normal range 10-40 mg/dl), serum C1q of 48 mg/l (124-190 mg/l) and serum C1-inhibitor of 23 mg/dl (normal range 15-35 mg/dl). The tests for cryoglobulins, rheumatoid factor, anti-nuclear antibodies and other antibodies such as anti-DNA native, anti-Sm, anti-RNP, anti-SS-A and anti-SS-B, and serological tests for hepatitis B, hepatitis C and HIV-1 were negative. The serum levels of IgG, IgA, IgM and IgE were within the normal range. Schirmer's test was negative. Computed tomography of the thorax and abdomen demonstrated mediastinal lymphadenopathy and splenomegaly. Bence-Jones protein was absent in the urine and serum electrophoresis and bone marrow examination were normal. Cervical lymph node biopsy revealed a diffuse large B cell lymphoma.



Complement deficiencies are known to precede the diagnosis of B cell lymphomas.[8] Patients with HUV may have undetectable or low-normal levels of C3 and C4 in the serum. The serum levels of C1q are usually profoundly decreased.[3],[4] Spontaneous or drug-induced remission of HUV may lead to normalisation of C3 and C4 levels, although the level of C1q may continue to remain modestly decreased.[3],[4] On the present admission, the patient was receiving prednisone and this may be the explanation for his normal serum C3 and modestly decreased serum C1q levels.

The patient did not have any of the systemic manifestations (ocular, pulmonary or renal involvement) commonly described with HUV.[3] Sjögren's syndrome is a close differential diagnosis of HUV, and lymphomas are known to be associated with Sjögren's syndrome. However, this patient did not have any diagnostic criterion for Sjögren's syndrome.

The mechanism for the association of lymphoma with HUV remains unclear. It is possible that anti-idiotypic antibodies produced by lymphoma cells against monoclonal immunoglobulins may play a role.[9] HUV might lead to prolonged B lymphocyte stimulation, and this would evolve into a lymphoma. This could be akin to the occurrence of B cell lymphoma years after the diagnosis of mixed cryoglobulinaemia, a disorder known to be associated with B lymphocyte expansion.[10] Recognition that HUV might antedate non-Hodgkin lymphomas would require a close follow-up in these patients.


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