A Unique Case of Erdheim-Chester Disease with Axial Skeleton, Lymph Node, and Bone Marrow Involvement / Journal of the Korean Cancer Association, 대한암학회지

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis with bone and organ involvement. A 76-year-old man presented with low back pain and a history of visits for exertional dyspnea. We diagnosed him with anemia of chronic disease, cytopenia related to chronic illness, chronic renal failure due to hypertension, and hypothyroidism. However, we could not determine a definite cause or explanation for the cytopenia. Multiple osteosclerotic axial skeleton lesions and axillary lymph node enlargement were detected by computed tomography. Bone marrow biopsy revealed histiocytic infiltration, which was CD68-positive and CD1a-negative. This report describes an unusual presentation of Erdheim-Chester disease involving the bone marrow, axial skeleton, and lymph nodes.

Systemic Treatment of Older Patients with Advanced Breast Cancer / 대한내과학회지

Due to its increasing incidence and longer life expectancy, more patients are being diagnosed with breast cancer at older ages. There are very limited data on the optimum management of older patients with advanced breast cancer, due to the under-representation of such individuals in clinical trials. Although older patients have more indolent disease with more Hormone Receptor positive disease and less HER2-positive disease, their disease-specific mortality remains lower than in younger patients, owing to the late diagnosis, under treatment due to age bias, reduced access to healthcare, and socioeconomic issues. Older patients with advanced breast cancer should be treated based on their biological tumor type, according to the patient's general health and preferences: endocrine treatment for HR-positive disease; Human Epidermal Growth Factor Receptor 2-targeted agent with chemotherapy, endocrine therapy or HER2-targeted agent alone for HER2 positive disease. Chemotherapy should be considered for patients who are HR-negative, HR-positive but refractory to endocrine treatment, or with a rapidly progressing visceral crisis. Generally, sequential chemotherapy with a single agent is recommended over combination chemotherapy, and agents with known toxicities in older patients are recommended, including weekly taxane, vinorelbine, capecitabine, and liposomal doxorubicin. Some form of geriatric assessment should be performed for older patients to assess the patients' biological age, functional status, and address age-specific problems, leading to early interventions. The goal of therapy should be individualized to maintain the quality of life, function, and independence of older patients with cancer.

Systemic Treatment of Older Patients with Advanced Breast Cancer / 대한내과학회지

Due to its increasing incidence and longer life expectancy, more patients are being diagnosed with breast cancer at older ages. There are very limited data on the optimum management of older patients with advanced breast cancer, due to the under-representation of such individuals in clinical trials. Although older patients have more indolent disease with more Hormone Receptor positive disease and less HER2-positive disease, their disease-specific mortality remains lower than in younger patients, owing to the late diagnosis, under treatment due to age bias, reduced access to healthcare, and socioeconomic issues. Older patients with advanced breast cancer should be treated based on their biological tumor type, according to the patient's general health and preferences: endocrine treatment for HR-positive disease; Human Epidermal Growth Factor Receptor 2-targeted agent with chemotherapy, endocrine therapy or HER2-targeted agent alone for HER2 positive disease. Chemotherapy should be considered for patients who are HR-negative, HR-positive but refractory to endocrine treatment, or with a rapidly progressing visceral crisis. Generally, sequential chemotherapy with a single agent is recommended over combination chemotherapy, and agents with known toxicities in older patients are recommended, including weekly taxane, vinorelbine, capecitabine, and liposomal doxorubicin. Some form of geriatric assessment should be performed for older patients to assess the patients' biological age, functional status, and address age-specific problems, leading to early interventions. The goal of therapy should be individualized to maintain the quality of life, function, and independence of older patients with cancer.

A Case of Autoimmune Hemolytic Anemia & Pericardial Effusion Developed in Hashimoto's Thyroiditis Patient / 대한내분비학회지

Hashimotos thyroiditis has been associated with a various autoimmune disorders. The immunologic mechanisms involved in the pathogenesis of these disorders have not always been thought to be the same. Although it was demonstrated that there were high prevalence of abnormal thyroid function and autoantibody in autoimmune hemolytic anemia(AIHA) and Fisher-Evans syndrome(FES), AIHA combined with Hashimotos thyroiditis is rare in Korean literature. It was suggested that a common immunologic mechanism may be involved in the pathogenesis of both disease and the possibility of multiple autoimmune syndrome might present in autoimmune hematologic disorders. We experienced a 74-year old woman with a 12-year history of a hypothyroidism due to Hashimotos thyroiditis was hospitalized with sudden development of warm AIHA with positive Direct & Indirect Coombs test and pericardial effusion. Her thyroid function test showed subclinical hypothyroidism with the maintenance dosage of levothyroxine(100pg/day). With glucocorticoid and plasmapheresis, AIHA and pericardial effusion were corrected successfully. It is suggested that the prudent immunologic study is needed for the anemia developed in patients with Hashimotos thyroiditis with or without hypothyroidism.

Hearing and Visual Loss with Renal Failure Due to Sodium Bromate Poisoning / 대한신장학회잡지

Hair neutralizer in home permanent cold wave kits contains a 2% to 10% potassium or sodium bromate, which is colorless, odorless and tasteless solution. Bromate salts are extremely toxic oxidants. The reported toxic effects of bromates poisoning are vomiting, diarrhea, depression of central nervous system, oliguric acute renal failure, sensorineural hearing loss, peripheral neuropathy and hemolytic anemia. Deafness seems to be almost permanent. In many cases, the recovery of renal function is not complete and some of them are transformed to chronic renal failure and maintained on hemodialysis. Since the serious side effects of deafness and acute renal filure usually occur within 4-16 h after ingestion, prompt removal of the bromate from blood by peritoneal dialysis or hemodialysis, which are proven method of removal, should be performed immediately. Recently, we encountered a 37 year old female hairdresser who was admitted to St. Mary Hospital due to oligura, hearing loss and visual loss after ingestion of sodium bromate. The patient required regular hemodialysis therapy and no recovery of hearing and visual loss was evident up to 8 month follow up.

A case of Budd-Chiari syndrome with high antiphospholipid antibody in a patient with systemic lupus erythematosus

Antiphospholipid syndrome is characterized by recurrent episodes of arterial and venous thrombosis, spontaneous fetal losses, thrombocytopenia and persistently elevated levels of antiphospholipid antibodies. We experienced a case of Budd-Chiari syndrome in a 32-year old female lupus patient who was presented with left leg edema, ascites and esophageal varix. The clinical and laboratory findings were compatible with the cirteria for systemic lupus erythematosus (SLE) and she was found to have anticardiolipin antibody, thrombocytopenia and prolonged partial thromboplastin time. Initially, she was treated with intravenous heparin and uroki nase and she was followed up with warfarin, baby aspirin and steroids.

A Case of Chorea Associated with Antipholipid Antibodies in Patients with Systemic Lupus Erythematosus / 대한류마티스학회지

The neuropsychiatric manifestations of systemic lupus erythematosus (SLE) are diverse but, among them, movement disorders such as chorea, ataxia are uncommon. We describe a 16 year old female SLE patient with positive anticardiolipin antibody who developed sudden onset of chorea. The clinical and laboratory findings that positive anticardiolipin antibody, thrombocytopenia, chorea were suggested antiphospholipid syndrome. Although the pathophysiology of chorea in SLE is unknown, an association with. antiphospholipid antibodies has been suggested. The underlying pathogenic mechanism of this case could not established but was most likely to have been an ischemia of the contralateral subthalamic nucleus. She was treated with corticosteroid, haloperidol and warfarin and now chorea is disappered.

Efficacy of Intravenous Immunoglobulin Therapy in Refractory Polymyositis and Dermatomyositis / 대한류마티스학회지

OBJECTIVES: Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many interventions including corticosteroids, immunosuppressive drug, and plasmapheresis to treat patients with PM/DM are not always effective, and may be associated with certain serious side effects. Intravenous immunoglobulin(IVIG) has been useful in a number of autoimmune diseases. An attempt was made to evaluate the efficacy of IVIG in refractory PM/DM. METHODS: Six patients with conventional treatment-refractory PM/DM [ 1 man and 5 women; mean age of 27(7-49)years ; PM(2), DM(2), juvenile DM(2)] received high doses of IVIG(2 mg/kg) dividing over consecutive 2 or 5 days. The IVIG infusion was conducted each month for 6 months. Clinical evaluations, including proximal muscle power, functional grading, and biochemical studies(creatine kinase(CK), aldolase) were performed before each or every IVIG infusion. Clinical evaluations were considered successful if more than 20% improvement of initial score was obtained. Biochemical results were considered "good" if muscle enzymes decreased more than 30% of initial values. RESULTS: Clinical improvement of proximal muscle power was noted in 5 patients after 6 courses of IVIG infusion. Timed stand test and functional grading score were also improved in all patients. All patients showed good response of biochemical results, except two cases with normal initial CK levels. Mean daily prednisolone dosage was significantly reduced(before IVIG: 39.2+/-9.2 mg/day vs after 6th IVIG: 7.3+/-2 mg/day, p<0.01). One patient experienced mild dyspnea and sweating during the course of IVIG infusion, which were disappeared with the reduction of daily IVIG dosage. CONCLUSIONS: These results suggest that IVIG is a safe and effective therapy in patients with PM/DM, who are resistant to traditional therapies or have limitations for their use.