A Case of Chronic Myeloid Leukemia with Multiple Chloromas Treated Successfully with Dasatinib / 계명의대학술지

Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder of the primitive hematopoietic stem cells. CML is characterized by the overproduction of myeloid cells, which results in marked splenomegaly and leukocytosis. CML presented by multiple chloromas is extremely rare. Multiple chloromas in the skin and brain are quite rare as the initial presentation of CML. These rare manifestation should alert clinicians to include CML in the differential diagnosis of patients presenting with multiple non-pruritic skin nodules or neurologic symptoms. Dasatinib has promising therapeutic potential for managing intracranial leukemic disease. Here, we report the case of a patient who visited the hospital with multiple chloroma which is unusual presentation of CML, and treated with dasatinib successfully.

A Review of the Electrocardiography Changes and Clinical Manifestations of Scrub Typhus in a Single Center / 대한내과학회지

BACKGROUND/AIMS: Most scrub typhus patients have normal electrocardiography (ECG) findings. Recently, however, several cases with ECG changes were reported. However, few studies have examined the ECG changes in scrub typhus. METHODS: The ECG and demographic data of patients infected with Orientia tsutsugamushi seen at Dongsan Medical Center from January 2008 to June 2012 were reviewed retrospectively. Patients with pre-existing cardiac disease were excluded. The initial and follow-up ECG findings, demographic characteristics, and laboratory findings was examined. RESULTS: The group in which the initial ECG showed an arrhythmia had a higher blood urea nitrogen (BUN) level than the normal and ischemic groups (median 26.0 vs. 15.0 vs. 15.5 mg/dL, p = 0.041). The group with a sustained abnormality in the follow-up ECG had higher BUN (median 17.0 vs. 15.0 mg/dL, p = 0.022) and creatinine (median 1.30 vs. 0.90 mg/dL, p = 0.026) levels than the normal ECG group. In addition, the group with a sustained abnormality in the follow-up ECG had a higher rate of intensive care unit (ICU) admission (30.8% vs. 5.3%, p = 0.015) and longer treatment period (average 9 vs. 6 days, p = 0.027) than the other groups. CONCLUSIONS: In our study, more than 30% of the patients with scrub typhus showed ECG abnormalities. Those with an arrhythmic ECG change on presentation had high BUN levels. The sustained ECG abnormality group had high BUN and creatinine levels, and an increased ICU admission rate and length of stay. We should pay attention to the ECG findings on presentation and during the treatment of scrub typhus.

The Efficacy and Perioperative Complications Associated with Lumbar Spinal Fusion Surgery, Focusing on Geriatric Patients in the Republic of Korea

OBJECTIVE: The purpose of this study was to examine the efficacy and perioperative complications associated with lumbar spinal fusion surgery, focusing on geriatric patients in the Republic of Korea. METHODS: We retrospectively investigated 485 patients with degenerative spinal diseases who had lumbar spinal fusion surgeries between March 2006 and December 2010 at our institution. Age, sex, comorbidity, American Society of Anesthesiologists (ASA) class, fusion segments, perioperative complications, and outcomes were analyzed in this study. Risk factors for complications and their association with age were analyzed. RESULTS: In this study, 81 patients presented complications (16.7%). The rate of perioperative complications was significantly higher in patients 70 years or older than in other age groups (univariate analysis, p=0.015; multivariate analysis, p=0.024). The perioperative complications were not significantly associated with the other factors tested (sex, comorbidity, ASA class, and fusion segments). Post-operative outcomes of lumbar spinal fusion surgeries for the patients were determined on the basis of MacNab's criteria (average follow up period : 19.7 months), and 412 patients (85.0%) were classified as having "excellent" or "good" results. CONCLUSION: Increasing age was an important risk factor for perioperative complications in patients undergoing lumbar spinal fusion surgery, whereas other factors were not significant. However, patients' satisfaction or return to daily activities when compared with younger patients did not show much difference. We recommend good clinical judgment as well as careful selection of geriatric patients for lumbar spinal fusion surgery.

Giant Cystic Cerebral Cavernous Malformation with Multiple Calcification: Case Report

Cerebral cavernous malformation with giant cysts is rare and literature descriptions of its clinical features are few. In this case study, the authors describe the clinical symptoms, radiological findings, and pathological diagnosis of cerebral cavernous malformations with giant cysts, reviewing the relevant literature to clearly differentiate this from other disease entities. The authors present a case of a 19-year-old male with a giant cystic cavernous malformation, who was referred to the division of neurosurgery due to right sided motor weakness (grade II/II). Imaging revealed a large homogenous cystic mass, 7.2x4.6x6 cm in size, in the left fronto-parietal lobe and basal ganglia. The mass had an intra-cystic lesion, abutting the basal portion of the mass. The initial diagnosis considered this mass a glioma or infection. A left frontal craniotomy was performed, followed by a transcortical approach to resect the mass. Total removal was accomplished without post-operative complications. An open biopsy and a histopathological exam diagnosed the mass as a giant cystic cavernous malformation. Imaging appearances of giant cavernous malformations may vary. The clinical features, radiological features, and management of giant cavernous malformations are described based on pertinent literature review.

Clinical Outcome of Rituximab-Based Therapy (RCHOP) in Diffuse Large B-Cell Lymphoma Patients with Bone Marrow Involvement / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: We investigated the clinical outcome of bone marrow (BM) involvement in patients with diffuse large B-cell lymphoma (DLBCL) who received rituximab-based therapy. MATERIALS AND METHODS: A total of 567 consecutive patients with newly diagnosed DLBCL treated with rituximab-CHOP (RCHOP) between November 2001 and March 2010 were included in the current study. All of the patients underwent a BM study at the initial staging and the clinical characteristics and prognosis of these patients with or without BM involvement were analyzed retrospectively. RESULTS: The total cohort included 567 patients. The overall incidence of BM involvement was 8.5%. With a median follow-up duration of 33.2 months (range, 0.1 to 80.7 months) for patients who were alive at the last follow-up, the five-year overall survival (OS) and event-free survival (EFS) rate in patients without BM involvement (76.3% and 67.5%, p<0.001) was statistically higher than that in patients with BM involvement (44.3% and 40.1%, p<0.001). In multivariate analysis, among total patients, BM involvement showed a significant association with OS and EFS. In univariate and multivariate analyses, even among stage IV patients, a significant association with worse EFS was observed in the BM involvement group. CONCLUSION: BM involvement at diagnosis affected the survival of patients with DLBCL who received RCHOP. Although use of RCHOP can result in significant improvement of the therapeutic effect of DLBCL, BM involvement is still a negative prognostic factor of DLBCL patients in the era of rituximab.

The Role of Hyperthyroidism as the Predisposing Factor for Superior Sagittal Sinus Thrombosis

Superior sagittal sinus thrombosis (SSST) is an uncommon cause of stroke, whose symptoms and clinical course are highly variable. It is frequently associated with a variety of hypercoagulable states. Coagulation abnormalities are commonly seen in patients with hyperthyroidism. To the best of our knowledge, there are few reports on the association between hyperthyroidism and cerebral venous thrombosis. We report on a 31-year-old male patient with a six-year history of hyperthyroidism who developed seizure and mental deterioration. Findings on brain computed tomography (CT) showed multiple hemorrhages in the subcortical area of both middle frontal gyrus and cerebral digital subtraction angiography (DSA) showed irregular intra-luminal filling defects of the superior sagittal sinus. These findings were consistent with hemorrhagic transformation of SSST. Findings on clinical laboratory tests were consistent with hyperthyroidism. In addition, our patient also showed high activity of factors IX and XI. The patient received treatment with oral anticoagulant and prophylthiouracil. His symptoms showed complete improvement. A follow-up cerebral angiography four weeks after treatment showed a recanalization of the SSS. In conclusion, findings of our case indicate that hypercoagulability may contribute to development of SSST in a patient with hyperthyroidism.

Comparison of Posterior Fixation Alone and Supplementation with Posterolateral Fusion in Thoracolumbar Burst Fractures

OBJECTIVE: We compared the radiological and clinical outcomes between patients who underwent posterior fixation alone and supplemented with fusion following the onset of thoracolumbar burst fractures. In addition, we also evaluated the necessity of posterolateral fusion for patients treated with posterior pedicle screw fixation. METHODS: From January 2007 to December 2009, 46 consecutive patients with thoracolumbar burst fracture were included in this study. On the basis of posterolateral fusion, we divided our patients into the non-fusion group and the fusion group. The radiological assessment was performed according to the Cobb's method, and results were obtained at immediately, 3, 6, 12 months after surgery. The clinical outcomes were evaluated using the modified Mcnab criteria at the final follow-up. RESULTS: The demographic data and the mean follow-up period were similar between the two groups. Patients of both groups achieved satisfactory clinical outcomes. The mean loss of kyphosis correction showed that patients of both groups experienced loss of correction with no respect to whether they underwent the posterolateral fusion. There was no significant difference in the degree of loss of correction at any time points of the follow-up between the two groups. In addition, we also compared the effect of fixed levels (i.e., short versus long segment) on loss of correction between the two groups and there was no significant difference. There were no major complications postoperatively and during follow-up period. CONCLUSION: We suggest that posterolateral fusion may be unnecessary for patients with thoracolumbar burst fractures who underwent posterior pedicle screw fixation.

Comparative Study of Posterior Lumbar Interbody Fusion via Unilateral and Bilateral Approaches in Patients with Unilateral Leg Symptoms

OBJECTIVE: We investigated the clinical and radiological advantages of unilateral laminectomy in posterior lumbar interbody fusion (PLIF) procedure comparing with bilateral laminectomy, under the same procedural condition including bilateral instrumentation and insertion of two cages, in patients with degenerative lumbar disease with unilateral leg symptoms. METHODS: We retrospectively reviewed 124 consecutive cases of PLIF via unilateral or bilateral approach between January 2006 and April 2010. In 80 cases (bilateral group), two cages were inserted via bilateral laminectomy, and in 44 cases (unilateral group), via unilateral laminectomy. The average follow-up duration was 29.5 months. The clinical outcomes were evaluated with the Visual Analogue Scale (VAS) and the Oswestry disability index (ODI). The fusion rates and disc space heights were determined by dynamic standing radiographs and/or computed tomography. Operative times, intra-operative and post-operative blood losses and hospitalization periods were also evaluated. RESULTS: In clinical evaluation, the VAS and ODI scores showed excellent outcomes in both groups. There were no significant differences in term of fusion rate, but the perioperative blood loss and the operative time of the unilateral group were lower than that of the bilateral group. CONCLUSION: Unilateral laminectomy can minimize the operative time and perioperative blood loss in PLIF procedure. However, the different preoperative disc height between two groups is a limitation of this study. Despite this limitation, solid fusion and satisfactory symptomatic improvement could be achieved uniquely by our surgical method. This surgical method can be an alternative surgical technique in patients with unilateral leg pain.

A case of thyrotoxicosis after sunitinib treatment / 대한내과학회지

Sunitinib is a tyrosine-kinase inhibitor used to treat metastatic renal cell carcinoma and imatinib-resistant gastrointestinal stromal tumors. Hypothyroidism is a common side effect of sunitinib therapy. Nevertheless, overt thyrotoxicosis induced by destructive thyroiditis after sunitinib treatment is very rare. Here, we report a patient with overt thyrotoxicosis related to sunitinib therapy.

Diagnostic Utility of AMACR and Claudin-7 for the Classification of Renal Cell Carcinoma

BACKGROUND: The histologic classification of renal cell carcinoma (RCC) is based on the cytoarchitectural features, yet sometimes this requires correlation with the immunophenotype. Alpha-methylacyl-CoA racemase (AMACR) and claudin-7 have recently been introduced as useful markers that are frequently expressed in papillary RCC (PRCC) and chromophobe RCC (ChRCC), respectively. The aims of this study are to evaluate the expressions of AMACR and claudin-7 in RCCs and to investigate whether they are helpful for making the histological classification of RCCs. METHODS: Immunohistochemistry for CD10, RCC marker, cytokeratin (CK)7, CD117, AMACR and claudin-7 was performed for 104 RCCs, and these consisted of 54 clear cell RCCs (CCRCC), 26 PRCCs and 24 ChRCCs. RESULTS: For diagnosing PRCC, the sensitivity and specificity of AMACR were 92.3% and 71.8%, respectively, and using AMACR(+)/CK7(+), the specificity was increased by 23.1% to 94.9%. For diagnosing ChRCC, the sensitivity and specificity of claudin-7 were 91.7% and 78.8%, respectively, and using claudin-7(+)/AMACR(-), the specificity was significantly improved (to 96.3%). For diagnosing CCRCC, CK7(-)/claudin-7(-)/CD117(-) was the most useful immunohistochemical panel (sensitivity, 96.3%; specificity, 98%). CONCLUSIONS: AMACR and claudin-7 are helpful markers for the histologic classification of RCCs, and their diagnostic utility is strengthened when they are used as an immunohistochemical panel, AMACR(+)/CK7(+) for PRCC, claudin-7(+)/AMACR(-) for ChRCC and CK7(-)/claudin-7(-)/CD117(-) for CCRCC.

Comparison of Fusion with Cage Alone and Plate Instrumentation in Two-Level Cervical Degenerative Disease

OBJECTIVE: This study assessed the efficacy of anterior cervical discectomy and fusion (ACDF) with cage alone compared with ACDF with plate instrumentation for radiologic and clinical outcomes in two-level cervical degenerative disease. METHODS: Patients with cervical degenerative disc disease from September 2004 to December 2009 were assessed retrospectively. A total of 42 patients received all ACDF at two-level cervical lesion. Twenty-two patients who underwent ACDF with cage alone were compared with 20 patients who underwent ACDF with plate fixation in consideration of radiologic and clinical outcomes. Clinical outcomes were assessed using Robinson's criteria and posterior neck pain, arm pain described by a 10 point-visual analog scale. Fusion rate, subsidence, kyphotic angle, instrument failure and the degenerative changes in adjacent segments were examined during each follow-up examination. RESULTS: VAS was checked during each follow-up and Robinson's criteria were compared in both groups. Both groups showed no significant difference. Fusion rates were 90.9% (20/22) in ACDF with the cage alone group, 95% (19/20) in ACDF with the plate fixation group (p = 0.966). Subsidence rates of ACDF with cage alone were 31.81% (7/22) and ACDF with plate fixation were 30% (6/20) (p = 0.928). Local and regional kyphotic angle difference showed no significant difference. At the final follow-up, adjacent level disease developed in 4.54% (1/22) of ACDF with cage alone and 10% (2/20) of ACDF with plate fixation (p = 0.654). CONCLUSION: In two-level ACDF, ACDF with cage alone would be comparable with ACDF with plate fixation with regard to clinical outcome and radiologic result with no significant difference. We suggest that the routine use of plate and screw in 2-level surgery may not be beneficial.

A Solitary Skull Lesion of Syphilitic Osteomyelitis

We experienced a rare case of solitary syphilitic osteomyelitis of the skull without any other clinical signs or symptoms of syphilis. A 20-year-old man was referred due to intermittent headache and mild tenderness at the right parietal area of the skull with a palpable coin-sized lesion of softened cortical bone. On radiological studies, the lesion was a radiolucent well enhanced mass (17 mm in diameter). The erythrocyte sedimentation rate (52 mm/h) and C-reactive protein (2.24 mg/dL) were elevated on admission. Serum venereal disease research laboratory (VDRL) and Treponema pallidum haemagglutination assay (TPHA) tests were positive. There were no clinical signs or symptoms of syphilis. After treatment with benzathine penicillin, we removed the lesion and performed cranioplasty. The pathologic finding of the skull lesion was fibrous proliferation with lymphoplasmocytic infiltration forming an osteolytic lesion. In addition, a spirochete was identified using the Warthin-starry stain. The polymerase chain reaction study showed a positive band for Treponema pallidum. Solitary osteomyelitis of the skull can be the initial presenting pathological lesion of syphilis.

Time to Recover Consciousness in Patients with Diffuse Axonal Injury : Assessment with Reference to Magnetic Resonance Grading

OBJECTIVE: This study was conducted to investigate the correlation between the degrees of injury on brain magnetic resonance imaging (MRI) and the time interval to recovery of consciousness in patients with diffuse axonal injury. METHODS: From January 2004 to December 2008, 25 patients with diffuse axonal injury were treated at our hospital. We retrospectively investigated the patients' medical records and radiological findings. We divided the patients into three groups according to the grade of MRI finding : grade I, small scattered lesions on the white matter of the cerebral hemisphere; grade II, focal lesions on the corpus callosum; and grade III, additional focal lesions on the brain stem. RESULT: Seven patients belonged to the grade I group; 10 to the grade II group; and 8 to the grade III group. The mean Glasgow Coma Scale (GCS) score of all patients at the time of admission was 7.28. Recovery of consciousness was observed in 23 of the 25 patients; the remaining two patients never regained consciousness. The time interval to recovery of consciousness (awake status) ranged from 1 day to 125 days (mean 22.1 days) : grade I group patients, within approximately 1 week (mean 3.7 days); grade II group patients, within approximately 2 weeks (mean 12.5 days); and grade III group patients, within approximately 2 months (mean 59.5 days). CONCLUSION: Our study results suggest a correlation between the mean time interval to recovery of consciousness in patients with diffuse axonal injuries and the degrees of brain injuries seen on MRI. Patients with grade I and II diffuse axonal injuries recovered consciousness within 2 weeks, while patients with grade III injuries required approximately 2 months.

Small lymphocytic lymphoma that developed in a patient with long-standing cold agglutinin disease / 대한내과학회지

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia (AIHA) induced by cold antibodies. CAD constitutes 16~32% of AIHA cases and the causative cold autoantibodies are commonly specific against blood group I or i antigens. CAD is closely related to lymphoid neoplasia and infections such as Mycoplasma and Epstein-Barr virus (EBV). Primary CAD has a tendency to develop into malignant lymphoma over its course. We experienced a case of small lymphocytic lymphoma during the treatment of primary CAD over 11 years. The patient was a 67-year-old man who complained of a neck mass. Node biopsy revealed diffuse neoplastic proliferation of small monotonous lymphoid cells with polygonal or oval nuclei;these cells were CD20- and CD5-positive. Follow-up chest and neck computed tomography after treatment with chlorambucil and prednisolone showed partial resolution and stable disease status for over 2 years. We report a case of small lymphocytic lymphoma that developed in a patient with long-standing cold agglutinin disease for 11 years and review the literature.

Misidentification of Candida parapsilosis as Large Platelets in an Automated Blood Analyzer / 대한임상미생물학회지

We experienced a case in which yeasts in blood sample from a patient with cervical cancer with hepatic metastasis and multiple intraperitoneal cysts interfered with platelet morphology flag in automated blood analyzer. The peripheral blood smear was performed to confirm the flag and revealed intracellular and extracellular yeasts, which were subsequently identified as Candida parapsilosis by blood culture.

A Case of Atypical CML with Micro BCR/ABL Rearrangement / 대한혈액학회지

Microtype BCR/ABL rearrangement is extremely rare and has been known to be associated with neutrophilic chronic myeloid leukemia (N-CML). However, there is more to be understood regarding this phenotype. We report a case of atypical CML that exhibited micro BCR/ABL rearrangement with predominant thrombocytosis. Our patient showed thrombocytosis (1,464x10(9)/L) and megakaryocytosis in the peripheral blood and bone marrow. However, neither leukocytosis nor neutrophilia was observed (white blood cells (WBC), 5.02x10(9)/L neutrophils, 45%). Bone marrow aspirate revealed increased cellularity: 12% basophils, 6% eosinophils, and 9% blasts. The 46,XX,t(9;22)(q34;q11.2),i(17q) chromosome complement was observed in 4 of 20 metaphase cells, and standard BCR/ABL fusion signals were observed in 10% of interphase cells on fluorescence in situ-hybridization (FISH) analysis. Reverse transcriptase- polymerase chain reaction (RT-PCR) was used to acquire the BCR/ABL fusion transcript, the identity of which was confirmed via sequence analysis. Hematologic remission was achieved 2 months after imatinib therapy initiation, and molecular remission was achieved 2 months after hematologic remission. The patient is currently undergoing regular follow-up visits and is in good health. However, further long-term follow up is warranted. The incorporation of imatinib into therapeutic strategies may be further established through the study of more cases of micro BCR/ABL.

Spontaneous Spinal Epidural Hematoma

Spontaneous spinal epidural hematoma (SSEH) is a relatively rare but significant spinal condition. Urgent surgical evacuation of a hematoma is generally indicated to prevent serious permanent neurological deficits. We encountered three cases of spontaneous spinal epidural hematomas associated with motor weakness that were treated successfully by surgical intervention.

Diagnosis and treatment of purpura / 대한내과학회지

No abstract available.

Low Molecular Weight Heparin Treatment in Pregnant Women with a Mechanical Heart Valve Prosthesis

No definitive recommendation is available concerning optimal antithrombotic therapy in pregnant women with a mechanical heart valve. The purpose of the current study was to evaluate the clinical results of nadroparin treatment with respect to pregnancy outcome and maternal complications. From 1997 to 2005, 31 pregnancies were reviewed in 25 women. Nadroparin (7,500 U, twice daily) was used in 23 pregnancies between 6 and 12 weeks of gestation and close-to-term only, and coumarin derivatives were used with aspirin at other times. Eight pregnant women treated with coumarin derivatives throughout pregnancy were compared to evaluate the safety and efficacy of nadroparin. No maternal death or bleeding complication occurred in either of the two groups, and frequencies of maternal thromboembolism including valve thrombosis (8.7% vs. 12.5%, p>0.05) were similar. However, the frequencies of live born (91.3% vs. 50%, p=0.01) and healthy babies (90.4% vs. 25%, p<0.01) were significantly higher, and the fetal loss rate was significantly lower (8.7% vs. 50%, p=0.01) in the nadroparin-treated group. Regarding the efficacy and safety of antithrombotic treatment in pregnant women with prosthetic heart valves, nadroparin treatment during the first trimester is an acceptable regimen and produces better results than coumarin derivatives.

Paclitaxel for elderly patients with advanced NSCLC / 대한내과학회지

BACKGROUND: Combination chemotherapy including platinum is based on treatment of advanced non-small cell lung cancer (NSCLC). But combination chemotherapy is not tolerable in elderly patients. Paclitaxel is one of the most active single chemotherapeutic agent in advanced NSCLC. We evaluated the efficacy and safety of single paclitaxel chemotherapy in elderly with advanced NSCLC. METHODS: From September 2002 to May 2004, a total 24 patients aged 70 years and older with advanced NSCLC were enrolled in this study. Treatment was consisted with paclitaxel 135 mg/m2 intravenously for 3hrs on day 1. Chemotherapy repeated every three weeks until disease progression or severe toxicity developed. RESULTS: Of the 24 patents, only 18 patient can be evaluated and 4 partial remission, 11 stable diseases and 3 progressive diseases were observed. Based on an intent-to-treatment analysis, The overall response rate was 17%. The estimated median survival and median time to progression were 44 weeks and 18 weeks, respectively. The major toxicity were grade 3 or 4 neutropenia (6%). Other toxicity were myalgia, neuropathy, nausea and oral mucositis, but all of them were usually mild (grade 1, 2) and recovered spontaneously. There were no treatment- related deaths. CONCLUSIONS: This single low dose paclitaxel chemotherapy is highly tolarable with activity comparable to that of conventional dose regimens especially in elderly advanced non-small cell lung cancer.