Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma

This study investigated the frequency of and predictive factors for autoimmune lymphoproliferative syndrome (ALPS) in children with lymphoma, chronic immune cytopenia, and nonmalignant organomegaly. Thirty-four children with suspected ALPS (n=13, lymphoma; n=12, immune cytopenia; n=9, nonmalignant organomegaly) were included. Double-negative T-cells, lymphocyte apoptosis, and genetic findings were analyzed. Patients were stratified into two groups as proven/probable ALPS and clinically suspected patients according to the ALPS diagnostic criteria. Of the 34 patients, 18 (53%) were diagnosed with proven/probable ALPS. One patient had a mutation (c.652-2A>C) in the FAS gene. The remaining 16 (47%) patients were defined as clinically suspected patients. Predictive factors for ALPS were anemia and thrombocytopenia in patients with lymphoma, splenomegaly and lymphadenopathy in patients with immune cytopenia, and young age in patients with nonmalignant organomegaly. ALPS may not be rare in certain risk groups. Our study indicates that screening for ALPS may be useful in children having lymphoma with cytopenia at diagnosis, in those having nonmalignant organomegaly with immune cytopenia, and in those having chronic immune thrombocytopenic purpura or autoimmune hemolytic anemia with organomegaly developing during follow-up.

Small bowel perforation due to metastatic lung squamous cell carcinoma.

Squamous cell cancer of the lung occurs with early dissemination but clinically significant metastases localized to the small bowel are rare. Small bowel metastasis is a fatal complication usually seen in advanced stages of the disease. In the present study, we report a case of a 65-year-old patient who was admitted to the emergency department with acute abdominal pain and during evaluation was found to have squamous cell carcinoma metastasis to small bowel leading to perforation. We also discuss the current management possibilities in the guidance of current literature.

Histopathological Effects of Parylene C (poly-chloro-p-xylylene) in the Inner Ear.

OBJECTIVE: To assess the histopathological effects of parylene C (PC) (poly-chloro-p-xylylene) in the inner ear. METHODS: Nine adult Dunkin Hartley guinea pigs (500-600 g) were included in the study. PC pieces were inserted into the cochlea in the right ear of the animals (study group). The round windows were punctured in the left ears comprised the control group. After three months, the animals were sacrificed, and the dissected temporal bones were examined under a light microscope. RESULTS: No significant difference was revealed between the study and control groups regarding histopathological findings such as perineural congestion, perineural inflammation, neural fibrosis, number of ganglion cells, edema, and degeneration of ganglion cells (p>0.05). CONCLUSION: PC did not cause any additional histopathologic damage in the cochlea. This finding may be promising regarding the use of PC in cochlear implant electrodes as an alternative to silicon materials in the future.

Mitomycin C effect on fibrous adhesions of rabbit temporomandibular joint.

OBJECTIVE: To evaluate influence of mitomycin C on fibrosis in temporomandibular joints of rabbits with mandibular traction. STUDY DESIGN: Twenty rabbits (8 control, 12 mitomycin C group) were included. Titanium screws were placed in the anterior edge of the right masseter tuberosity and the posterior part of the right zygoma. Elastic bands were placed between 2 screws to initiate posterio-superior displacement of the mandible to the right. Animals in the control group (CG) received intraarticular NaCl (0.9%) on the nondrawing (left) side, and animals in the study group (SG) received mitomycin C weekly (0.4 mg/mL) for 6 weeks. Rabbits were decapitated, temporomandibular joints were removed, and histopathological investigation was performed. RESULTS: Microscopically, 4 of 6 rabbits in the CG had fibrosis in the temporomandibular joint, whereas only 1 of 11 rabbits in the SG had fibrosis (P < 0.05). CONCLUSION: Application of mitomycin C to the temporomandibular joint of rabbits significantly reduces formation of intraarticular fibrous adhesions. SIGNIFICANCE: This is the first study in the literature investigating the use of mitomycin C in the joints. EBM RATING: B-2.

Immunomodulatory effectiveness of azithromycin in prevention of postlaminectomy epidural fibrosis.

OBJECTIVE: One of the important causes of failed back surgery is the extensive peridural fibrosis collecting in the surgical field after spinal surgeries. Today we know that inflammatory mechanisms mediated by the immune system of the body plays an important role in generation of fibrosis. Azithromycin, a macrolide antibiotic, has proven immunomodulatory effects in various diseases. This study aims to investigate the effects of azithromycin on peridural fibrosis. METHODS: Twenty-four Wistar rats received laminectomies before dividing them into three groups randomly. Animals of the control group received normal saline intraperitoneally while animals in the treatment groups received low (20 mg/kg) and high (80 mg/kg) doses of azithromycin intraperitoneally after surgical interventions. The amount of fibrosis, fibroblast density and inflammatory cell density were analyzed histologically. RESULTS: Analysis demonstrated significantly reduced fibrosis, fibroblast density and inflammatory cell density in treatment groups compared to the control group. There was no difference between the treatment groups. CONCLUSION: Immune system plays critical roles in tissue repair and fibrogenesis. Results of our study demonstrated that azithromycin application reduced formation of peridural fibrosis in experimental laminectomy model in rats. Further studies with different dose regimes and different application routes are required to carry these results to an advanced level.