Ocular adnexal mucosa-associated lymphoid tissue lymphoma with polyclonal hypergammaglobulinemia.
OBJECTIVE
To determine the characteristics of patients with primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma associated with polyclonal hypergammaglobulinemia.


METHODS
Case series study.


METHODS
Among 81 Japanese patients with primary ocular adnexal MALT lymphoma, seven patients (9%) were diagnosed with polyclonal hypergammaglobulinemia. Patient clinical data included a history of autoimmune disease and dissemination. Peripheral blood collected from all patients was analyzed for serum levels of rheumatoid factor, soluble interleukin-2 receptor (sIL-2R), and immunoglobulins at the time of diagnosis and after each treatment.


RESULTS
Seven patients with polyclonal hypergammaglobulinemia had elevated serum levels of rheumatoid factor, sIL-2R, immunoglobulin G (IgG), and immunoglobulin E (IgE) at the time of diagnosis. One patient had Sjogren syndrome. Six patients (86%) had a dissemination of the MALT lymphoma or lymphadenopathy at the time of diagnosis. Histopathologic examination of the patients with lymphadenopathy revealed not only MALT lymphoma but also secondary follicles. None of the seven patients showed improvement in serum levels of IgG, rheumatoid factor, or sIL-2R in spite of complete regression of the ocular lesions after radiotherapy. After administration of cyclophosphamide/doxorubicin/vincristine/prednisone and/or rituximab to three patients, all three showed improved serum levels of IgG, rheumatoid factor, and sIL-2R.


CONCLUSIONS
Patients with ocular adnexal MALT lymphoma and polyclonal hypergammaglobulinemia have elevated serum levels of rheumatoid factor, sIL-2R, and IgE, and high dissemination or lymphadenopathy. These unique characteristics may correlate with the systemic immunologic imbalances.
Kubota
Toshinobu
T
Department of Ophthalmology, National Hospital Organization, Nagoya Medical Center, 4-4-1 Sanno-maru, Naka-ku, Nagoya, Japan. ganiky@nnh.hosp.go.jp
Moritani
Suzuko
S
Yoshino
Tadashi
T
Nagai
Hirokazu
H
Terasaki
Hiroko
H
D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003230:Conjunctival Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000150:complications* / Q000628:therapy; D007074:Immunoglobulin G / Q000097:blood; D007766:Lacrimal Apparatus Diseases / Q000150:complications* / Q000473:pathology / Q000628:therapy; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D011878:Radiotherapy; D015375:Receptors, Interleukin-2 / Q000097:blood; D012217:Rheumatoid Factor / Q000097:blood
D007074:Immunoglobulin G; D015375:Receptors, Interleukin-2; D012217:Rheumatoid Factor