The evaluation of malignancies in Turkish primary immunodeficiency patients; a multicenter study.

BACKGROUND: There are no data regarding the prevalence of malignancies in patients with primary immunodeficiency (PID) in Turkey. Along with the prevalence of malignancy, we aimed to present the types of malignancy and define the underlying immune deficiency of the patients. METHOD: Between the years 1992 and 2018, from five tertiary immunology clinics, fifty-nine patients with PID who developed malignancy were included. All patients were evaluated for demographics, clinical features, and prognosis. RESULTS: The prevalence of malignancy in our cohort was detected as 0.9% (59/6392). The male-to-female ratio was 1.8 (38/21), and the median age of patients was 14 years (range: 1.5-51). The median age at diagnosis of malignancy was 10 years (range: 1.5-51). Ataxia-telangiectasia was the most frequent PID in patients with malignancy (n = 19, 32.2%), and non-Hodgkin lymphoma was the most common malignancy (n = 32, 51.6%). The rate of malignancy in DOCK8 deficiency (n = 7/43, 16.3%) was higher than AT (n = 19/193, 9.8%), Wiskott-Aldrich syndrome (n = 2/22, 9.1%), and common variable immunodeficiency (n = 11/205, 5.4%). EBV quantitative PCR was positive in 16 out of 53 patients (30.2%). Three patients had secondary malignancies. Remission was achieved in 26 patients (44.1%). However, 31 patients (52.5%) died. Two patients (3.4%) are still on chemotherapy. CONCLUSION: This study is the largest cohort investigating the association of malignancy in patients with PID in Turkey. While lymphoid malignancies were the most common malignancy and observed more frequently in AT patients, the risk for malignancy was higher in patients with DOCK8 deficiency compared to AT.

Vedolizumab treatment in a patient with X-linked agammaglobulinemia, is it safe and efficient?

Çekiç S, Özgür T, Karali Y, Özkan T, Kiliç SS. Vedolizumab treatment in a patient with X-linked agammaglobulinemia, is it safe and efficient? Turk J Pediatr 2019; 61: 937-940. The loss of inflammatory regulation resulting from the absence of B-lymphocytes leads to a risk for autoimmune and autoinflammatory complications. There is no data on the use of Vedolizumab in patients with X-linked agammaglobulinemia (XLA) as well as children with another primary immunodeficiency (PID) diseases. A 4-year-old boy was admitted to our clinic with a history of recurrent respiratory tract infections. He was diagnosed with XLA based on extremely low immunoglobulins, very low level of B cells, genetic mutation of BTK gene, and family history. At the age of 8, he suffered from intermittent fever attacks, abdominal pain, weakness, oral aft, and weight loss. His clinical and laboratory features were consistent with inflammatory bowel disease. Histopathological examination of the biopsy material obtained from terminal ileum, colon and cecum showed Crohn`s disease. Initially, he was treated with prednisolone and infliximab. Because of the lack of response, infliximab treatment was switched to adalimumab. Terminal ileum was resected to relieve obstruction complication. Although he had been treated with adalimumab, a significant improvement was not observed. Vedolizumab (Entyvio™), a humanized monoclonal antibody α4ß7 integrin-receptor antagonist, was commenced. After treatment with vedolizumab, his fever and abdominal pain attacks reduced, his total daily calorie intake increased and weight gain improved. He began to walk again and continued his school education properly. No side effects were observed in 18 months. This is the first immunocompromised child treated with vedolizumab. The symptoms of the patient receded and no side effect were seen during the treatment.

Use of complementary and alternative medicine in children with cancer: effect on survival.

The objective of the present study was to determine the type, frequency, the reason why complementary and alternative medicine (CAM) treatments are used, the factors related with their use, and the effects of CAM usage on long-term survival. Families of a total of 120 children with cancer between 0-18 years of age, including 50 (41.7%) girls and 70 (58.3%) boys, participated in our study. The authors found that 88 patients (73.3%) used at least one CAM method, the most common (95.5%) of which was biologically based therapies. Most frequently used biologically based therapies were dietary supplements and herbal products. The most commonly used dietary supplement or herbal product was honey (43.2%) or stinging nettle (43.2%), respectively. We found that patients used such CAM methods as complementary to, but not instead of, conventional therapy. Sixty-nine out of 88 patient families (78.4%) shared the CAM method they used with their physicians. No statistically significant relation was found between socioeconomic, sociodemographic, or other factors or items and CAM use. The mean follow-up period of the CAM users and nonusers groups was 79.4 ± 36.7 (21.3-217.9) and 90.9 ± 50.3 (27.4-193.7) months, respectively. Five-year survival rates for CAM users and nonusers were found as 81.5% and 86.5%, respectively (P > .05). In conclusion, families of children with cancer use complementary and alternative treatment frequently. They do not attempt to replace conventional treatment with CAM. Higher rates of CAM use was found in families with higher educational level. CAM usage did not affect the long-term survival.