Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai-Dorfman Disease.

Rosai-Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment.

Evaluation of Iron Overload in the Heart and Liver Tissue by Magnetic Resonance Imaging and its Relation to Serum Ferritin and Hepcidin Concentrations in Patients with Thalassemia Syndromes.

Iron overload is one of the major prognostic factor in thallassemia patients. We aimed to evaluate iron accumulation in the heart and liver by MRI in thalassemia major, thalassemia intermedia, and S-ß thalassemia patients and to examine its association with ferritin and hepcidin levels. Serum ferritin and hepcidin levels were recorded. Iron overload (IOL) in the heart and liver parenchyma was determined based on the standardized T2* and R2 values measured by MRI. The results were evaluated considering the tissue iron overload, serum ferritin and hepcidin levels. Comparing the 109 patients with the 30 healthy controls revealed the mean age: 24.4 ± 11 versus 31.2 ± 5 years, median levels of serum ferritin: 1693 versus 40 ng/mL, and hepcidin: 1.94 versus 0.355 ng/mL; p < 0.001, respectively. Comparison of age, serum ferritin and hepcidin levels and MRI findings of the patients with or without IOL revealed that, ferritin and T2* were significantly different in the patients with IOL in cardiac tissue (p = 0.004 and p < 0.001), and, age, ferritin and R2 were significantly different in the patients with IOL in liver tissue (p = 0.036, p < 0.001 and p < 0.001). The MRI-based T2* and R2 values were moderately and inversely correlated with serum ferritin (r = -0.37; p < 0.001 and r = -0.46; p < 0.001). No correlations were found between the MRI-based T2*, R2 values and serum hepcidin. A moderate and positive correlation existed between serum ferritin and hepcidin (r = 0.45; p < 0.001). We considered that, enhanced intestinal iron absorption characterized by decreased serum hepcidin levels in the intervals between successive transfusions were resulted in iron accumulation in our patients.

A rare case of systemic lupus erythematosus with chylous ascites and chylothorax.

During the course of the disease a patient with systemic lupus erythematosus (SLE) may develop inflammation of one or more serous membranes, resulting in pleural, peritoneal, or pericardial effusion. Chylous ascites and chylothorax have rarely been described in patients with SLE. Therefore, in parallel with the analysis of blood samples, detailed analysis of the effusions should be carried out. Supportive measures are often needed to relieve the symptoms of chylothorax or chylous ascites together with the treatment of the primary disease. The available literature had reported just 4 cases of chylous ascites and/or chylothorax in association with SLE, and this patient presented here is one of the rare cases apart from the reported ones.

Evaluation of transient hyperglycemia in non-diabetic patients with febrile neutropenia.

BACKGROUND: We aimed to examine the effect of transient hyperglycemia in non-diabetic patients with febrile neutropenia. METHODS: A total of 86 patients with febrile neutropenia were evaluated between June 2006 and December 2009. After measuring random blood glucose level at admission, cases with stress hyperglycemia were included in the study. Stress hyperglycemia was defined as documented random blood glucose level of 140 mg/dl and above without known diabetes mellitus, impaired glucose tolerance and impaired fasting glucose. A Multinational Association for Supportive Care in Cancer (MASCC) scoring system was used for the prediction of low and high risk patients according to medical complications at the onset of the febrile episode. RESULTS: There were more patients with stress hyperglycemia than the patients with normoglycemia in the high risk group (p = 0.001). The growth of gram negative bacteria and fungi was higher in patients with stress hyperglycemia than with normoglycemia (p = 0.001). The patients receiving antifungal therapy had a higher rate of stress hyperglycemia than the patients without receiving antifungal therapy (p = 0.009). The patients with stress hyperglycemia had higher mortality rates than the patients with normoglycemia (p = 0.007). According to the MASCC risk-index, stress hyperglycemia increased 3.35 fold in the high risk patients compared to the low risk patients (p = 0.046) and 4.14 fold in the patients treated with antibacterial and antifungal agents compared to the patients treated with only antibacterial agents (p = 0.038). CONCLUSION: Patients with stress hyperglycemia had more adverse clinical outcomes than patients with normoglycemia. We think further studies are needed to evaluate the relationship between stress hyperglycemia and febrile neutropenia.

Monoballism associated with newly onset ketotic hyperglycemia.

Movement disorders as the initial symptoms of diabetes mellitus are rare. Here, we describe one of these rare manifestations of primary diabetes: a case of newly diagnosed diabetes mellitus in an old-age female patient with transient monoballismus during an episode of ketotic hyperglycemia.