B cells expressing CD5 are increased in Sj?gren's syndrome

In this investigation of B cells expressing the CD5 (Leu-1) cell surface marker, we found increased numbers of these cells in 13 of 19 patients with primary Sj?gren's syndrome (SS) (68%), as well as in the rheumatoid arthritis patients. The percentage of B cells that demonstrated increased expression of CD5 was 46% in SS patients, 47% in rheumatoid arthritis patients, 24% in systemic lupus erythematosus patients, and 26% in normal subjects. Over a 2-year period, CD5 expression on B cells was a stable finding in several patients, except for 2 who required either steroid therapy or combined chemotherapy and irradiation for malignant lymphoma. Both of these patients had clinical remissions and their levels of CD5+ B cells returned to normal. The first patient had a clinical picture of SS/systemic lupus erythematosus overlap, associated with polyclonal B cell activation and decreased production of interleukin-2 in response to stimulation with phytohemagglutinin. These cellular immune abnormalities returned to normal after the institution of corticosteroids. Our observations suggest a relationship between the CD5+ B cell abnormality and disease activity. The results are discussed in relation to immunoregulatory properties of CD5+ B cells in autoimmune mice and the characteristic predisposition to malignant lymphoma among SS patients.     

Targeted inactivation of the IL-4 receptor alpha chain I4R motif promotes allergic airway inflammation

The insulin/interleukin-4 (IL-4) receptor (I4R) motif mediates the association of insulin receptor substrate (IRS)-2 with the interleukin-4 (IL-4)Ralpha chain and transduces mitogenic signals in response to IL-4. Its physiological functions were analyzed in mice with a germline point mutation that changed the motif's effector tyrosine residue into phenylalanine (Y500F). The Y500F mutation abrogated IRS-2 phosphorylation and impaired IL-4-induced CD4+ T lymphocyte proliferation but left unperturbed Stat6 activation, up-regulation of IL-4-responsive gene products, and Th cell differentiation under Th2 polarizing conditions. However, in vivo the Y500F mutation was associated with increased allergen-induced IgE production, airway responsiveness, tissue eosinophilia, and mucus production. These results define an important role for the I4R motif in regulating allergic inflammation.     

Immune complexes in hepatitis B antigen-associated periarteritis nodosa. Detection by antibody-dependent cell-mediated cytotoxicity and the Raji cell assay.

A subpopulation of peripheral blood lymphocytes with the ability to lyse target cells coated with specific antibody (antibody-dependent cell-mediated cytotoxicity, ADCC) was serially studied in a patient with hepatitis B antigen-associated periarteritis nodosa. The effector lymphocytes possess FC and complement receptors but do not require complement for functional activity. We found that the patient's ADCC was decreased during periods of disease activity and was almost normal during remission. The patient's serum could block ADCC in normal lymphocytes, and the blocking ability correlated with the concentration of immune complexes as determined by the Raji cell assay (a radioimmunoassay using complement receptors on human cultured lymphoblastoid cells). The concentration of immune complexes and the ADCC blocking ability of ther serum both correlated with disease activity. Serum from five other patients with active vasculitis was found to contain significant amounts of immune complexes and was able to block normal ADCC. It appears that the ADCC assay can be used to detect the presence of circulating immune complexes and to monitor disease activity in periarteritis nodosa.     

Comparative studies of antilymphocyte, antipolynucleotide, and antiviral antibodies among families of patients with systemic lupus erythematosus

Antilymphocyte, antipolynucleotide, and antiviral antibodies in 15 Soviet systemic lupus erythematosus (SLE) probands and their 41 relatives were compared with those in 57 members of 15 control Russian families. Seventy-eight and three-tenths percent of SLE relatives were positive for antilymphocyte antibody versus 26.3% positive reactions in controls. Antipolynucleotide antibody closely paralleled antilymphocyte antibody in both groups. Antiviral antibody was significantly increased in SLE relatives as compared to control family members. No correlation was noted between quantitative IgG, IgA, or IgM levels and various antibodies studied.     

Clinicopathological pattern of malignant parotid gland tumors in Saudi Arabia

OBJECTIVE: To report our experience of varied presentations and diverse histopathological spectrum of parotid gland malignancies. METHODS: This retrospective analysis incorporated patients with histological evidence of malignant parotid tumors at King Khalid University Hospital, Riyadh, Saudi Arabia over a 20-year period from 1984 through 2004. The medical records of these patients were analyzed for their demographic characteristics, clinical features, operations performed, and pathological diversity. RESULTS: Thirty-two patients comprised this study group. There is a male preponderance over females with a ratio of 2.2:1 (22 men and 10 women) and mean age of 51.8 (range 28-81 years). A painless lump was the most frequent clinical manifestation observed in 23 (71.8%) patients followed by facial nerve dysfunction in 14 (43.7%) patients. Parotidectomy was performed in 22 (68.7%) patients: 16 superficial and 6 total. A partial facial nerve sacrifice was undertaken in 14 (43.7%), and total nerve sacrifice in 9 (28.1%) patients. Four (12.5%) patients presented with cervical lymph node metastases necessitating radical neck dissection. Nine (28.1%) patients had mucoepidermoid carcinoma, 8 (25%) adenoid cystic carcinoma, 6 (18.7%) adenocarcinoma, not otherwise specified, and 2 (6.2%) were reported to have carcinoma in pleomorphic adenoma. Twenty (62.5%) specimens revealed high-grade aggressive lesions, and out of these, 19 (59.3%) patients presented with stage III/IV disease. CONCLUSION: Malignant parotid tumors are exceedingly rare, occurring at a relatively earlier age group with male preponderance, and invariably declare at a late clinical stage in our community. Histopathological features hallmark a locally advanced disease with an aggressive behavior.     

A new method for lateral pterygoid electromyographic electrode placement

STATEMENT OF PROBLEM: Making electromyographic recordings of the lateral pterygoid muscle (LP) is difficult because of potential electrode damage to, for example, the maxillary artery and long buccal nerve, and because of pain and reduced jaw mobility characteristic of many orofacial pain patients. PURPOSE: The purpose of this study was to develop a reliable intraoral placement technique for the inferior head of the lateral pterygoid (IHLP) that minimizes jaw displacement. MATERIAL AND METHODS: In 2 dried skulls and 7 human cadavers, it was estimated that, with the mandible in an ipsilateral closed position, a straight needle could be used to position fine-wire electrodes into the midportion of IHLP by inserting the needle through the mucosa adjacent to the distal root of the maxillary second molar, towards the external auditory meatus and parallel to the buccal alveolar bone of the maxilla. The needle avoided the maxillary artery and long buccal nerve. Using this approach in 5 adults, 2 fine-wire electrodes were placed into the IHLP. Placement was verified by computer tomography (CT) and electromyography. RESULTS: In all subjects, the ideal insertion depth to place the electrodes in the middle of IHLP was 29 mm. CONCLUSIONS: This technique is a reliable method for IHLP electrode placement for patients with impaired jaw function, minimizing risk of damage to major structures.     

Light Microscopic, Immunophenotypic, and Molecular Genetic Study of Autoimmune Lymphoproliferative Syndrome Caused by fas Mutation

This case provides a complete light microscopic, immunophenotypic, and molecular genetic analysis of autoimmune lymphoproliferative syndrome (ALPS), a rare benign cause of dramatic lymphadenopathy with atypical histology and phenotype that may be mistaken for malignancy. The patient is 3-year-old child who was first clinically evaluated at the age of 16 months because of marked generalized lymphadenopathy and hepatosplenomegaly. Histologic sections of a cervical lymph node demonstrated a marked paracortical proliferation of occasional small and intermediate-sized lymphocytes and numerous large immunoblasts, the majority of which displayed a CD3⁺, CD43⁺, CD45RO⁻ (OPD4, UCHL1) CD4⁻, CD8⁻ phenotype on paraffin sections, and which had a CD2⁺, CD3⁺, CD5⁺, CD56⁻, Tδ1⁻, [CD4⁻, CD8⁻] double negative profile on flow cytometric analysis. Southern blot analysis did not identify a clonal T or B cell population, and sequencing of the fas gene identified a mutation that caused a single amino acid substitution in the intracytoplasmic death domain of this protein. An enriched population of CD45RO-negative naive T cells in the paracortex may explain the atypical histologic and immunophenotypic features of this case. Greater awareness of this heritable lymphoproliferative disorder will facilitate its recognition and minimize the possibility of misdiagnosis. Received January 22, 1999; accepted April 7, 1999.