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Original Investigation |

High Incidence of Gastrointestinal Tract Disorders and Autoimmunity in Primary Cutaneous Marginal Zone B-Cell Lymphomas

Joan Guitart, MD1; Janyana Deonizio, MD1; Tara Bloom, MS1; Maria Estela Martinez-Escala, MD1; Timothy M. Kuzel, MD2; Pedram Gerami, MD1; Mary Kwasny, ScD3; Steve T. Rosen, MD2
[+] Author Affiliations
1Division of Hematology/Oncology, Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois
2Division of Hematology/Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
3Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
JAMA Dermatol. 2014;150(4):412-418. doi:10.1001/jamadermatol.2013.9223.
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Importance  To our knowledge, this is the first comprehensive study addressing comorbidities associated with primary cutaneous marginal zone B-cell lymphomas (PCMZLs).

Objective  To determine if patients with PCMZL were at risk for other medical conditions.

Design, Setting, and Participants  Case-control study at a cutaneous lymphoma clinic and a dermatopathologic consultation service at a single academic institution using an extensive questionnaire of illnesses, symptoms, and environmental exposures for 80 sequential patients with PCMZL and 80 matched controls.

Main Outcomes and Measures  The frequency of several morbidities was obtained in both groups from data gathered from the questionnaire and corroborated by reviewing medical records when available.

Results  We found a high incidence of past or present gastrointestinal tract problems in 49 patients (61.2%) with PCMZL compared with 30 participants (37.5%) in the control group (CG) (P = .003). Gastroesophageal reflux was reported in 50.0% (40 vs 27 in the CG, P = .04) and gastric ulcers in 10.0% (8 vs 3 in the CG, P = .13); 20.0% of the cohort had positive Helicobacter pylori serology (16 vs 2 in the CG, P = .003). Colon disorders, including irritable bowel syndrome and inflammatory bowel disease, were more common in the PCMZL cohort (20 vs 7 in the CG, P = .01). Autoimmunity was reported in 20.0% of participants (16 vs 6 in the CG, P = .03). Eight patients had a history of Hashimoto thyroiditis. Three patients had a positive antinuclear antibody. Two had a diagnosis of lupus erythematosus and 1 had Sjögren syndrome. Sicca syndrome was noted in 12.5% (10 vs 3 in the CG, P = .052). A history of noncutaneous malignant neoplasms was observed in 21.3% of the patients (17 vs 8 in the CG, P = .050). Other notable morbidities did not reach statistical significance.

Conclusions and Relevance  Our results indicate a high incidence of systemic conditions in patients with PCMZL, especially involving the gastrointestinal tract, but also autoimmunity and cancer.

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Figure 1.
Clinical Presentation of Marginal Zone Lymphoma in 2 Elderly Men

A, Erythematous papule within a background of erythematous patch with follicular-based papules. B, Tumor lesions on the lower leg developed within an erythematous plaque with prominent follicular-based papules.

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Figure 2.
Histologic Features of Marginal Zone Lymphoma

A, Low-power view (hematoxylin-eosin, original magnification ×40) of the skin biopsy specimen showing a deep nodular infiltrate with nodular aggregates alternating with pale zones reflecting the proliferative monocytoid cells with an abundant pale cytoplasm. B, High-power view (hematoxylin-eosin, original magnification ×400) with a colonized germinal center and peripheral accumulation of plasma cells.

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