Hodgkin's lymphoma presenting with markedly elevated IgE: a case report
© Ellis and Waserman; licensee BioMed Central Ltd. 2009
Received: 27 October 2009
Accepted: 7 December 2009
Published: 7 December 2009
Markedly elevated IgE as a manifestation of a lymphoproliferative disorder has been only rarely reported.
We present the case of a 22 year old female referred to the adult Allergy & Clinical Immunology clinic for an extremely elevated IgE level, eventually diagnosed with Hodgkin's lymphoma. She had no history of atopy, recurrent infections, eczema or periodontal disease; stool was negative for ova & parasites. Chest X-ray revealed large bilateral anterior mediastinal masses that demonstrated prominent uptake on gallium scan. Mediastinal lymph node biopsy was consistent with Hodgkin's lymphoma, nodular sclerosing subtype, grade I/II.
Although uncommon, markedly elevated IgE may be a manifestation of a malignant process, most notably both Hodgkin's and Non-Hodgkin's lymphomas. This diagnosis should be considered in evaluating an otherwise unexplained elevation of IgE.
Elevated levels of total serum IgE are associated with many diseases, including allergic bronchopulmonary aspergillosis (ABPA), parasitosis, atopic dermatitis, adult HIV infection, hyper-IgE (Job's) syndrome, Sézary's syndrome, IgE myeloma, and Kimura's disease. Lymphoproliferative disorders are known associations of the hyper-IgE syndrome [2–4], however a marked elevation of IgE as an initial manifestation of a lymphoproliferative disease is rare, and mainly reported in IgE producing plasmacytomas; also rare (0.01% of plasmacytomas). Three cases are reported in the literature of non-Hodgkin's lymphoma associated with markedly elevated levels of IgE [6–8], one of which was asymptomatic and discovered serendipitously during an evaluation of perennial rhinitis. Here we present a patient referred for evaluation of a markedly elevated IgE, eventually diagnosed with Hodgkin's lymphoma.
Laboratory parameters upon referral to Allergy & Immunology Clinic.
She had no history of recurrent infections, eczema or periodontal disease, nor was there a history of foreign travel, diarrhea or other symptoms suggestive of parasitic infection. There was no history of allergic rhinitis (seasonal or perennial), asthma, sinusitis, otitis or other allergic disease. Her physical examination was entirely normal. Skin tests were positive to trees, grass and ragweed, and careful questioning confirmed an absence of clinical symptoms aside from intermittent cough. Stool examination was negative for ova & parasites. Spirometry and methacholine challenge revealed a mild isolated decrease in diffusion capacity, and no airway hyper-responsiveness.
Significant elevations of IgE are seen in various allergic conditions, parasitosis, and rarely, in lymphoproliferative malignancies. Specifically, extreme elevations of IgE have been documented in the setting of multiple myeloma, and B-cell lymphomas. In this case, the patient had no history of atopy, or parasitic infection and she had a normal protein electrophoresis and bone marrow evaluation.
Lymphomas are known to produce immunoglobulins, and rarely, cases have been reported of both B- and T-cell lymphomas associated with elevated IgE [6–8]. Sézary's syndrome (a peripheral T-cell neoplasm) has been associated with elevated IgE and/or eosinophilia when the malignant clone is of the CD4+ helper phenotype and produces an abnormal amount of the cytokine IL-4[9, 10]. Modestly elevated IgE has also been reported in B-cell chronic lymphocytic leukemia and in 2 patients with Hodgkin's disease (1 case of nodular sclerosing, one case of mixed cellularity, levels were 675 IU/mL and 310 IU/mL, respectively).
Markedly elevated IgE may rarely present as an initial manifestation of a lymphoproliferative disorder such as a lymphoma. These patients may be referred for evaluation of allergy or immunodeficiency, such as hyperIgE syndrome. This patient had unexplained fatigue and anemia, and only chest X-ray was suggestive of a malignant process. Underlying lymphoproliferative disease should always be considered when evaluating an otherwise unexplained significant elevation of IgE, particularly when features of allergy or parasitosis are distinctly lacking. Specific work-up of significantly elevated IgE levels should be tailored to the clinical features of the case, but in this circumstance a serum LDH and a CXR helped to reveal the underlying causative lymphoma.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
No external funding was received to support this publication.
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