A case of mantle cell lymphoma presenting as IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz's disease.

BACKGROUND: Mantle cell lymphoma (MCL) is a relatively uncommon type of non-Hodgkin lymphoma. It develops in the outer edge of a lymph node called the mantle zone. In contrast, IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) is characterized by elevated serum IgG4 and persistent bilateral enlargement of lacrimal glands (LGs) and salivary glands (SGs), with infiltration of IgG4-positive plasma cells. Recent studies indicated the importance of differentiation between IgG4-DS and malignant lymphoma. CASE PRESENTATION: An 82-year-old man was suspected of IgG4-DS because of a high serum IgG level (2174 mg/dL) and bilateral swelling of LGs and SGs. Lip biopsy and fine needle biopsy of submandibular gland were performed, and subsequently, MCL was diagnosed through the histopathological findings. CONCLUSIONS: MCL most commonly occurs in the Waldeyer ring, but rarely in the stomach, spleen, skin, LG, and SG. We report an unusual case of MCL involving LGs and SGs mimicking IgG4-DS, which suggests that IgG4 testing may be useful in the differentiation of IgG4-DS in the presence of bilateral swelling of LGs or SGs.

A case of marginal zone B cell lymphoma mimicking IgG4-related dacryoadenitis and sialoadenitis.

BACKGROUND: IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease). CASE PRESENTATION: We report on the case of a 70-year-old man who was strongly suspected IgG4-DS because of high serum IgG4 concentration (215 mg/dl) and bilateral swelling of parotid and submandibular glands. Biopsies of cervical lymph node and a portion of submandibular gland were performed. These histopathological findings subsequently confirmed a diagnosis of marginal zone B cell lymphoma. CONCLUSION: Differential diagnosis of IgG4-DS is necessary from other disorders, including Sjögren's syndrome, sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, and cancer. We suggest that biopsy of swollen lesions is important for a definitive diagnosis of IgG4-DS and discuss the mechanism of development in this case.

Mikulicz's disease: a new perspective and literature review.

PURPOSE: To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature. METHODS: Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of Medicine at UCLA. RESULTS: Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders. CONCLUSIONS: Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of Sjögren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS.

[Mikulicz disease as the beginning of AIDS]. / Enfermedad de Mikulicz como debut en un caso de SIDA.

Major salivary lymphoepithelial lesions accounted for less than 3 percent of all benign parotid tumors, prior to AIDS acquaintance. Defined as the presence of enlargement of one or more salivary glands and, in some cases, with diminished salivary function. The paper present the case of an acquired immunodeficiency syndrome starting as a Mikulicz's disease. Reviewed the medical literature and treatment options.

[Malignant change in the lymphoepithelial lesion of the parotid gland--report of 3 cases].

3 rare cases of malignant change of lymphoepithelial lesion (Mikulicz's disease) of parotid gland are presented. 1 was male and 2 female. Their ages ranged from 40 to 71 with an average of 57 years. All were pathologically proved. The common clinical manifestations were local painless masses. 2 of the 3 cases were treated only by local excision. Extended excision with simultaneous neck dissection of the metastatic lymph nodes was done in the remained one. All patients received postoperative radiotherapy at doses of 3,000-5,000 rad over 19 to 36 days. One of them died 2.5 years later. The other two are still living without evidence of recurrence more than 3 and 8 years respectively. The prognosis of malignant Mikulicz's disease is probably better than that of parotid carcinoma. The authors suggest that the treatment of this disease, especially in those with regional metastasis, be managed according to the same principle of parotid cancer. Postoperative radiotherapy should be given as a routine for still better results. The clinico-pathologic features and pathogenesis of this disease are discussed briefly.

Benign lymphoepithelial lesions of the salivary glands.

The benign lymphoepithelial lesion of the salivary glands has been labeled with multiple, confusing terms. We recommend the abandonment of the vague term of Mikulicz disease. The histopathologic findings of lymphoid infiltration, intraductal proliferation, epimyoepithelial islands, and acinar atrophy are presented. The relationship between this lesion and autoimmune diseases, including Sjögren syndrome, is noted. Difficulty of histopathologic differentiation between this lesion and malignant lymphoma can occur. The association of the benign lymphoepithelial lesion with the simultaneous presence or future development of lymphoma is discussed.