No abstract available.
Cerebral Infarction ; Global Health ; Humans ; Mass Screening ; Retrospective Studies ; World Health Organization

BACKGROUND/AIMS: Renal complications related to BCR-ABL1-negative myeloproliferative neoplasms (MPNs) have not been examined fully in Asian populations. METHODS: We analyzed estimated glomerular filtration rate (eGFR) and its changes with time retrospectively in patients with BCR-ABL1-negative MPN from 2005 to 2015. RESULTS: The prevalence of chronic kidney disease (CKD) was 11% (6.6% having stage 3 and 4.4% having stage 4). In a linear regression analysis of eGFR versus time (years), overall, patients showed increased eGFR (mL/min/1.73 m2) by 0.51 (95% confidence interval [CI], –0.30 to 1.33; p = 0.22). Patients with polycythemia vera (PV), and those treated with hydroxyurea, showed statistically significant increases in eGFR (1.59; 95% CI, 0.28 to 2.90; p = 0.22 in PV; and 1.55; 95% CI, 0.56 to 2.54; p = 0.22 in treatment with hydroxyurea). In total, 17 patients (20.5%) showed rapid loss of eGFR (<–3 mL/min/1.73 m2per year). This rapid loss in eGFR was associated with a higher incidence of kidney disease (23.5% vs. 6.1%, p= 0.05) and a higher percentage of patients with high neutrophil (>7.0 × 109 /L) and high monocyte (> 0.7 × 109 /L) counts (76.5% vs. 50%, p=0.05; 52.9% vs. 28.8%, p= 0.06, respectively). More patients had high serum lactate dehydrogenase (> 500 U/L) levels (52.9% vs. 25.8%, p = 0.03) at diagnosis. CONCLUSIONS: CKD is prevalent in patients with BCR-ABL1-negative MPN. Active cytoreductive therapy has the potential to improve kidney function in BCR-ABL1-negative MPN.
Asian Continental Ancestry Group ; Diagnosis ; Glomerular Filtration Rate ; Humans ; Hydroxyurea ; Incidence ; Kidney ; Kidney Diseases ; L-Lactate Dehydrogenase ; Linear Models ; Monocytes ; Neutrophils ; Polycythemia Vera ; Prevalence ; Renal Insufficiency, Chronic* ; Retrospective Studies*

No abstract available.
Hemoglobinuria, Paroxysmal* ; Humans ; Prednisolone*

Plasmacytoma in patients with multiple myeloma usually develops in the advanced stage of the disease. We report herein an atypical case of extramedullary relapse of multiple myeloma that presented as mechanical obstruction of the small bowel in a patient who had achieved complete remission after chemotherapy. A 75-year-old man was diagnosed with multiple myeloma 25 months previously and treated with a bortezomib-containing chemotherapy regimen. He presented for evaluation of abdominal pain. A circumferential mass resulting in mechanical ileus was observed by abdominal computed tomography. Biopsy after surgical resection confirmed the diagnosis of plasmacytoma. The patient was subsequently treated with thalidomide-containing chemotherapy, but he died of disease progression after 6 months. We suggest careful observation of unusual relapses of multiple myeloma in patients who have achieved complete remission after antimyeloma therapy.
Abdominal Pain ; Aged ; Biopsy ; Diagnosis ; Disease Progression ; Drug Therapy ; Humans ; Ileus ; Intestinal Obstruction ; Multiple Myeloma* ; Plasmacytoma ; Recurrence*

PURPOSE: The aim of this study is to determine the diagnostic and prognostic role of baseline spinal magnetic resonance imaging (MRI) in patients with multiple myeloma. MATERIALS AND METHODS: We enrolled patients newly diagnosed with multiple myeloma from 2004-2011 at a single center. Abnormal MRI findings that were not detected in radiographs have been analyzed and categorized as malignant compression fractures or extramedullary plasmacytoma. The bone marrow (BM) infiltration patterns on MRI have been classified into five categories. RESULTS: A total of 113 patients with a median age of 65 years (range, 40 to 89 years) were enrolled in the study. Malignant compression fractures not detected in the bone survey were found in 26 patients (23.0%), including three patients (2.6%) with no related symptoms or signs. Extramedullary plasmacytoma was detected in 22 patients (19.5%), including 15 (13.3%) with epidural extension of the tumor. Of these 22 patients, 11 (50.0%) had no relevant symptoms or signs. The presence of malignant compression fractures did not influence overall survival; whereas non-epidural extramedullary plasmacytoma was associated with poor overall survival in the multivariate analysis (hazard ratio, 3.205; 95% confidence interval [CI], 1.430 to 9.845; p=0.042). During the follow-up for a median of 21 months (range, 1 to 91 months), overall survival with the mixed BM infiltrative pattern (median, 24.0 months; 95% CI, 22.9 to 25.1 months) was shorter than those with other patterns (median 56 months; 95% CI, 48.9 to 63.1 months; p=0.030). CONCLUSION: These results indicate that spine MRI at the time of diagnosis is useful for detecting skeletal lesions and predicting the prognosis in patients with multiple myeloma.
Bone Marrow ; Diagnosis* ; Follow-Up Studies ; Fractures, Compression ; Humans ; Magnetic Resonance Imaging* ; Multiple Myeloma* ; Multivariate Analysis ; Plasmacytoma ; Prognosis ; Spine*

No abstract available.
Plasmacytoma

We report a 62-year-old woman with multiple myeloma associated with cryoglobulinemia accompanied by gangrene of the digits. She presented with generalized purplish net-like discoloration (livedo reticularis), which was more prominent in the lower extremities. Multiple small shallow ulcers with crusts were found in places. In addition, gangrene was observed in both ear helices, both index fingers, and several toes. The patient had monoclonal gammopathy consisting of IgG and kappa (3.95 g/dL), cryoglobulinemia, and bone marrow plasmacytosis (42%). A biopsy of a discolored skin patch on the lower leg revealed leukocytoclastic vasculitis. She was diagnosed with multiple myeloma associated with cryoglobulinemia. Immediate plasmapheresis halted the progression of the skin lesions and digital gangrene. Two cycles of thalidomide plus dexamethasone therapy led to a partial response. This case highlights the need to search for cryoglobulinemia and multiple myeloma when we see livedo reticularis or multiple skin ulcers with obscure causes.
Biopsy ; Bone Marrow ; Cryoglobulinemia ; Dexamethasone ; Ear ; Female ; Fingers ; Gangrene ; Humans ; Immunoglobulin G ; Leg ; Livedo Reticularis ; Lower Extremity ; Middle Aged ; Multiple Myeloma* ; Paraproteinemias ; Plasmapheresis ; Skin ; Skin Ulcer ; Thalidomide ; Toes ; Ulcer ; Vasculitis*

To determine the approximate incidence and clinical features of pernicious anemia in a Korean population, we retrospectively analyzed clinical data for patients with pernicious anemia who were diagnosed between 1995 and 2010 at five hospitals in Chungnam province. Ninety-seven patients were enrolled, who accounted for 24% of patients with vitamin B12 deficiency anemia. The approximate annual incidence of pernicious anemia was 0.3 per 100,000. The median age was 66 (range, 32-98) yr, and the male/female ratio was 1.25. Anemia-associated discomfort was the most common symptom (79.4%), followed by gastrointestinal and neurological symptoms (78.4% and 38.1%, respectively). Pancytopenia was found in 36 patients (37.1%), and autoimmune disorders were found in 15 patients (15.5%). Antibody to intrinsic factor was detected in 62 (77.5%) of 80 patients examined, and antibody to parietal cells was detected in 35 (43.2%) of 81 patients examined. Of the 34 patients who underwent tests for Helicobacter pylori, 7 (12.5%) were positive. The anemia-associated and gastrointestinal symptoms resolved completely in all patients after intramuscular injection of cobalamin, whereas neurological symptoms remained in some. In conclusion, pernicious anemia is less frequent in Koreans than in Western populations; however, the clinical features of this disorder in Koreans do not differ from those of Western cases.
Adult ; Aged ; Anemia, Pernicious/complications/*diagnosis/epidemiology ; Asian Continental Ancestry Group ; Autoimmune Diseases/complications/epidemiology ; Female ; Gastrointestinal Diseases/complications/drug therapy/epidemiology ; Helicobacter Infections/diagnosis ; Helicobacter pylori ; Humans ; Isoantibodies/blood ; Male ; Middle Aged ; Nervous System Diseases/complications/epidemiology ; Parietal Cells, Gastric/immunology ; Republic of Korea/epidemiology ; Retrospective Studies ; Vitamin B 12/blood/therapeutic use

Multiple solitary plasmacytoma is a very rare disease entity, which occurs in up to 5% of patients with solitary plasmacytomas. We report an atypical case of multiple solitary plasmacytoma that recurred in multiple visceral organs without any evidence of bone marrow involvement. A 68-year-old male presented with voiding difficulty. Twenty months earlier, he had been placed on local radiotherapy for solitary plasmacytomas in the right 6th rib and right iliac bone. Recurrences were noted 14 and 12 months later in several ribs and the 5th cervical vertebra, respectively. These were well controlled with local radiotherapy and conventional systemic chemotherapy. He had multiple soft tissue masses in the stomach, pancreas, pelvic cavity, and right buttock. An endoscopic biopsy of the gastric mass confirmed the diagnosis of plasmacytoma. Local radiotherapy to the pelvic mass and systemic therapy consisting of bortezomib and dexamethasone were given, and he has been well for 8 months.
Aged ; Biopsy ; Bone Marrow ; Boronic Acids ; Buttocks ; Dexamethasone ; Humans ; Male ; Pancreas ; Plasmacytoma ; Pyrazines ; Rare Diseases ; Recurrence ; Ribs ; Spine ; Stomach ; Viscera ; Bortezomib