Malignant melanoma (MM) has become the fifth most frequent cancer in the UK. It is the most common carcinoma to metastasize to the gastrointestinal (GI) tract. MM particularly has an affinity to spread to the small bowel, which is followed by the involvement of the stomach and large intestine. Excellent endoscopic options including video capsule endoscopy and enteroscopy are available for a precise diagnosis of GI involvement by a metastatic MM. The complete surgical resection of GI metastatic MM in carefully selected patients not only provides symptom control, but has also been associated with an increase in overall survival. The approval of BRAF-targeted therapies and immune checkpoint inhibitors has transformed therapeutic approaches for patients with metastatic MM over the past decade. Currently, the overall survival of patients with advanced metastatic MM who have been treated with a combination of immunotherapeutic agents reaches 52% at five years. The role of surgery for patients with the metastatic involvement of the GI tract with MM is evolving in the era of effective systemic treatments.
Involvement of the peripheral nerves in metachromatic leucodystrophy has received increasing interest in recent years (Brain and Greenfield, 1950;Norman, Urich, and Tingey, 1960). Though definite pathological findings have been made there have been few clinical observations of peripheral neuropathy in this condition. CASE REPORTThis patient was born to unrelated parents without any family history of neurological disease. The birth history was without incident. She had normal milestones until the age of 1j years, when she was noticed to be unsteady on her legs, and within the course of a few weeks was unable to support herself unaided. There was a history of recurrent attacks of infection of the upper respiratory tract. On examination at the age of 18 months she was mentally alert, there was a left sixth-nerve palsy, the arms were normal but the legs were hypnotic, with weakness of both proximal and distal muscles, and the reflexes were depressed. A lumbar puncture examination at the time showed cerebrospinal fluid (C.S.F.) protein 180 mg./100 ml. without cells, and a negative Lange curve. In view of these clinical and biochemical findings a diagnosis of postinfective peripheral neuritis was made.Electrophysiological studies were done at this stage (Dr. D. Taverner). The conduction in the right lateral popliteal nerve was 15 metres per second. Electromyographic studies were done on the right anterior tibial and left extensor digitorum brevis muscles. There was discrete motor activity with spontaneous fibrillation potentials in both those muscles, confirming denervation in the muscles of the legs.Prednisone 5 mg. b.d. was given, and in the following three weeks she made a definite but slight improvement, and at the time of her discharge from hospital could walk with support. While at home prednisone was increased to 30 mg. daily, but her neurological condition progressed. She could walk only with the aid of the furniture, and after six months from the onset of the illness she had become helpless and unable to stand. There was no change in the sixth nerve palsy. At this stage her hands were unsteady and she had a coarse tremor while feeding herself. She was mentally alert and would play with her brother.On readmission, when the duration of her illness was 10 months, she was found to be mentally alert but very irritable. Sucking and grasp reflexes were absent. There was some intention tremor, and arm movements were clumsy. She could not support herself, and her legs were found for the first time to be spastic; the tendon jerks were normal in the arms, but the knee jerks were pathologically brisk. The abdominal reflexes were present, and the right plantar response was equivocal. Lack of co-operation precluded a sensory examination. During this admission she had a major epileptic attack. DISCUSSIONThe neurological manifestations of metachromatic leucodystrophy are mainly due to involvement of the central nervous system, and clinical involvement of the peripheral nerves has been largely overlooked. This is apparent ...
Background: Multiple myeloma (MM) is influenced by genetic and micro-environmental changes. Malignant plasma cells produce an abnormal monoclonal immunoglobulin, as well as cytokines, such as IL-10 and IL-6 which stimulate cells of the bone marrow microenvironment (BMM) and cause dysfunction and failure of many organs. B cell activating factor (BAFF), IL6 and IL10 are known to influence the growth and survival of malignant clones. Aim: The objectives of the present study were to investigate the circulating levels of BAFF , IL-10 and IL-6, correlate them with well-known parameters of disease activity in patients with MM, and to detect their impact on patients' survival. Materials and Methods: This study was conducted on 89 newly diagnosed MM patients and seventy apparently healthy volunteers as a normal control group. BAFF, IL6, IL10 were measured by ELISA for both groups and survival analysis was performed for all patients. Results: Studied markers were higher in the MM patients compared to the normal control subjects. Patients survival was improved by high serum BAFF levels. Conclusions: High levels of BAFF were found to improve patients' survival. BAFF and IL-6 can be considered probable diagnostic markers for MM.
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