Long-term follow-up results of cytarabine-containing chemotherapy for acute promyelocytic leukemia

Background/Aims@#We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL). @*Methods@#We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up. @*Results@#The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis. @*Conclusions@#Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.

The Abuse Potential of α-Piperidinopropiophenone (PIPP) and α-Piperidinopentiothiophenone (PIVT), Two New Synthetic Cathinones with Piperidine Ring Substituent

A diversity of synthetic cathinones has flooded the recreational drug marketplace worldwide. This variety is often a response to legal control actions for one specific compound (e.g. methcathinone) which has resulted in the emergence of closely related replacement. Based on recent trends, the nitrogen atom is one of the sites in the cathinone molecule being explored by designer type modifications. In this study, we designed and synthesized two new synthetic cathinones, (1) α-piperidinopropiophenone (PIPP) and (2) α-piperidinopentiothiophenone (PIVT), which have piperidine ring substituent on their nitrogen atom. Thereafter, we evaluated whether these two compounds have an abuse potential through the conditioned place preference (CPP) in mice and self-administration (SA) in rats. We also investigated whether the substances can induce locomotor sensitization in mice following 7 days daily injection and challenge. qRT-PCR analyses were conducted to determine their effects on dopamine-related genes in the striatum. PIPP (10 and 30 mg/kg) induced CPP in mice, but not PIVT. However, both synthetic cathinones were not self-administered by the rats and did not induce locomotor sensitization in mice. qRT-PCR analyses showed that PIPP, but not PIVT, reduced dopamine transporter gene expression in the striatum. These data indicate that PIPP, but not PIVT, has rewarding effects, which may be attributed to its ability to affect dopamine transporter gene expression. Altogether, this study suggests that PIPP may have abuse potential. Careful monitoring of this type of cathinone and related drugs are advocated.

Profound Hearing Loss after Endolymphatic Sac Decompression in Intractable Meniere's Disease / 대한평형의학회지

Endolymphatic sac decompression (ESD) is indicated in intractable Meniere's disease patients with serviceable hearing. A 43-year-old man presented with recurrent vertigo and fluctuating right hearing loss that had been intractable to medical treatment. ESD was performed for the purpose of vertigo control with hearing preservation. Positional vertigo with profound hearing loss developed immediate after surgery and positional vertigo was resolved within days. Following paralytic vestibulopathy with positive sign on head thrust test also resolved after 2 weeks, while sensorineural hearing loss was not recovered to preoperative level during 1 year of follow up. Recurrent vertigo attacks were developed again 1 year after the operation. Hearing preservation was not always guaranteed in ESD. Furthermore, chance of hearing loss should be included in informed consent though the procedure is purposed for hearing preservation.

The Nature of Acute Pain in Herpes Zoster and Its Relationship to the Patients' Activities of Daily Living / 대한피부과학회지

BACKGROUND: Acute pain can severely interfere with the activities of daily living (ADL) of patients with herpes zoster, but until now, little has been published in the Korean medical literature about the impact of herpes zoster on the ADL. OBJECTIVE: The objective of this study was to identify the nature and severity of the acute pain of herpes zoster patients and its relationship to the patients' ADL. METHODS: 86 patients with herpes zoster and who visited our hospital from August 2009 to June 2010 were evaluated in regard to age, gender, the dermatomal distribution, the associated diseases, the severity and nature of the pain and the ADL. The nature of the pain was measured by the Short Form McGill Pain Questionnaire. To access interference of pain with the ADL, we used the Zoster Brief Pain Inventory (ZBPI) and the Zoster Impact Questionnaire (ZIQ). RESULTS: The mean onset of pain was 5.6+/-3.2 days before the visit, and the mean onset of skin lesion was 4.2+/-2.2 days before the visit. The most common nature of the pain of herpes zoster was shooting pain (55.8%), followed by stabbing pain (54.7%). But there was no significant difference of gender, age, the dermatomal distribution, the clinical severity and the associated disease according to the severity and nature of the pain. For every 1.0 point increase in the pain score, there was a 0.257 and 0.105 point increase in the ZBPI and ZIQ scores, respectively, on the multivariate linear regression model. CONCLUSION: This study will help to choose appropriate treatments to reduce the pain of patients who suffer with herpes zoster.

The Nature of Acute Pain in Herpes Zoster and Its Relationship to the Patients' Activities of Daily Living / 대한피부과학회지

BACKGROUND: Acute pain can severely interfere with the activities of daily living (ADL) of patients with herpes zoster, but until now, little has been published in the Korean medical literature about the impact of herpes zoster on the ADL. OBJECTIVE: The objective of this study was to identify the nature and severity of the acute pain of herpes zoster patients and its relationship to the patients' ADL. METHODS: 86 patients with herpes zoster and who visited our hospital from August 2009 to June 2010 were evaluated in regard to age, gender, the dermatomal distribution, the associated diseases, the severity and nature of the pain and the ADL. The nature of the pain was measured by the Short Form McGill Pain Questionnaire. To access interference of pain with the ADL, we used the Zoster Brief Pain Inventory (ZBPI) and the Zoster Impact Questionnaire (ZIQ). RESULTS: The mean onset of pain was 5.6+/-3.2 days before the visit, and the mean onset of skin lesion was 4.2+/-2.2 days before the visit. The most common nature of the pain of herpes zoster was shooting pain (55.8%), followed by stabbing pain (54.7%). But there was no significant difference of gender, age, the dermatomal distribution, the clinical severity and the associated disease according to the severity and nature of the pain. For every 1.0 point increase in the pain score, there was a 0.257 and 0.105 point increase in the ZBPI and ZIQ scores, respectively, on the multivariate linear regression model. CONCLUSION: This study will help to choose appropriate treatments to reduce the pain of patients who suffer with herpes zoster.

Calcifying Aponeurotic Fibroma with Gait Disturbance Caused by Trauma / 대한피부과학회지

A calcifying aponeurotic fibroma is a rare benign fibrous tumor with well-characterized histopathological features. Calcifying aponeurotic fibromas are generally slowly-growing lesions related to tendons and aponeuroses in the distal extremities of children and adolescents. While the neoplasm is usually asymptomatic, cases of progressive local discomfort have been reported. The exact etiology of this neoplasm is unknown, and there is frequently no history of trauma or local irritating factors. A 10-year-old female presented with a 2.5x2.0 cm, subcutaneous mass on the right sole 4 years after she bumped against a table. The histopathologic examination showed a poorly circumscribed fibrotic mass with foci of chondroid differentiation and calcification. These findings were consistent with the diagnosis of a calcifying aponeurotic fibroma. After total excision of the mass, there has been no recurrence of the lesion in 8 months of follow-up.

Hypertrophic Lichen Planus That Occurred in an Elderly Woman / 대한피부과학회지

Lichen planus is a chronic papulosquamous disease that affects the skin and mucous membranes. It is characterized by pruritic violaceous papules that are most commonly seen on the extremities of middle aged adults, but the elderly are rarely affected. Hypertrophic lichen planus usually occurs on the extremities and especially on the shins, and it tends to be the most pruritic variant. An 82-year-old female presented with severe pruritic multiple papules and plaques on the both the upper and lower extremities for 1 year. At first, the lesions began as small, polygonal papules that formed verrucous plaques over several months. Histopathologic examination showed marked hyperkeratosis, irregular acanthosis and wedge-shaped hypergranulosis. The vacuolar alteration and the lymphocytic inflammatory infiltrate were accentuated at the base of the rete ridge. From these findings, we diagnosed these lesions as hypertrophic lichen planus. The lesions were treated with topical steroid and intralesional injection of steroid, and they were slightly improved after 2 months of therapy.

The Analysis of Prognostic Factor and Treatment Outcome of Malignancies of the External Auditory Canal / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The purpose of this study was to analyze the survival rate treatment modalities and certain prognostic factors in patients with external auditory canal (EAC) cancer using the University of Pittsburgh TNM Staging System. SUBJECTS AND METHOD: Medical records and radiological findings of 33 patients treated for EAC cancer between January 1995 and January 2009 were reviewed retrospectively. The median follow-up period was 65 months (range, 12-182 months). RESULTS: The most common histological type was squamous cell carcinoma (19 patients; 57.6%), followed by adenoid cystic carcinoma (9 patients; 27.3%), basal cell carcinoma (1 patient), adenocarcinoma (1 patient), rhabdomyosarcoma (1 patient), undifferentiated carcinoma (1 patient) and malignant small round cell tumor (1 patient). Seven of 33 patients died of EAC cancer and the overall survival rate was 70.6%. There was a significant difference in the survival rate with respect to TNM stages (p=.031). The 10 yr-survival rate of patients with stage I disease was 100.0%, whereas those of stages II, III and IV were 50.0, 80.0 and 58.3%, respectively. Five of 7 patients with recurrence had a history of initial positive resection margin. CONCLUSION: Early detection and proper surgical treatment with sufficient resection margin are essential for disease-specific survival and prevention of recurrence in patients with EAC cancer.

Schwannoma Mimicking Laryngocele

A schwannoma of the larynx is a rare benign tumor that usually presents as a submucosal mass in the pyriform sinus and the aryepiglottic space, and this type of schwannoma constitutes a diagnostic and therapeutic challenge for otolaryngologists. We present here two cases of supraglottic schwannomas that were misdiagnosed as laryngoceles. Both were excised through a lateral thyrotomy approach without a tracheostomy, and the laryngeal function was successfully maintained. We discuss the clinical and imaging findings and the management of this rare neoplasm with focusing on the differential diagnosis of laryngeal schwannoma and laryngocele. We also review the relevant medical literature.

Erratum: Correction for incorrectly displaying several words

The publisher wishes to apologize for incorrectly displaying several words. We correct the words from inclu-des (page 104, line 11, right column) to includes, from eventualry (page 105, line 24, left column) to eventually, from approprlate (page 105, line 28, left column) to appropriate, and from Early (page 105, 6 line, right column) to early.

Juvenile Hyaline Fibromatosis

Juvenile hyaline fibromatosis (JHF) is a rare, progressive autosomal recessive disease that's characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. We present here the case of a 2-yr-old boy with JHF along with a review of the relevant literature. This case demonstrates that JHF should be considered in the differential diagnosis when multiple subcutaneous nodules are observed in the face, head and neck.

Effect of Botulinum Toxin A on Trismus after Traumatic Brain Injury: A case report

We report a case of severe trismus following traumatic brain injury (TBI), which was treated successfully with botulinum toxin A. Its effect evidenced long-term resolution, for over one year. A 36 year-old man with a multifocal intracranial hemorrhage was admitted for quadriplegia with dysphagia and trismus. During ten months, a nasogastric tube was placed for feeding after TBI, and at that time his upper- to-lower interincisal distance was only 1.2 cm upon voluntary mouth opening. Botulinum toxin A 450 U (Dysport(R)) was injected into both masseter, medial pterygoid and left temporalis muscles under electromyographic guidance. The interincisal distance began to improve one week after injection. He successfully underwent percutaneous endoscopic gastrostomy, dysphagia rehabilitation therapy, and dental prosthesis. More than one month after injection, oral feeding proved possible. After 1 year his interincisal distance was maintained at 2.9 cm.

The Ultrasonographic Findings Related to Pain and FunctionalStatus in Patients with Knee Osteoarthritis

OBJECTIVE: To evaluate the usefulness of ultrasonographic (US) examination in patients with knee osteoarthritis (OA) and determine US findings associated with pain and functional status. METHOD: 45 patients with primary knee OA classified by the American College of Rheumatology (ACR) criteria were recruited. The severity of pain and functional status were measured by Lequesne index and Western Ontario and McMaster Universities Osteoarthritis Scores (WOMAC). All patients underwent US examination of their knees and plain radiography for Kellgren and Lawrence (KL) grade. RESULTS: Even in mild OA cases (KL G1, 2), patients had evidence of distended suprapatellar pouch (effusion) (32%), synovial thickening (12%), cartilage degeneration (32%) and medial capsular distension (72%). The severity of pain and functional status were found to be correlated with following US findings: amount of suprapatellar effusion (gamma=0.514, p<0.01), degree of synovial thickness (gamma=0.520, p<0.01), cartilage degeneration grade (gamma=0.594, p<0.01), length of medial capsular distension (gamma=0.426, p<0.01). However, the length of medial and lateral osteophytes, size of Baker's cyst, and clinical parameter such as age, disease duration and BMI score were not correlated with the severity of pain and functional status in OA patients. Following multiple regression analysis, the amount of effusion, synovial thickness and length of medial capsular distension were correlated with Lequesne and WOMAC functional status score (gamma2=0.635, p<0.05). CONCLUSION: Ultrasonographic assessment was useful for diagnosing knee OA. The severity of pain and function were highly associated with the amount of suprapatellar effusion, degree of synovial thickness, the length of medial capsular distension and grade of cartilage degeneration.

Clinical Manifestations and Surgical Results of Malleostapedotomy and Malleostapedectomy / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Incus stapedotomy is usually performed for the patients with otosclerosis and congenital ossicular fixation. However, for the patients whose incus is absent, anomalous or fixed, the piston wire cannot be placed around the incus. For these cases, malleostapedotomy or malleostapedectomy could be a proper treatment instead of incus stapedotomy. The aim of this study was to evaluate the clinical manifestation and treatment outcome of malleostapedotomy and malleostapedectomy. SUBJECTS AND METHOD: From January 1993 through March 2007, there were four malleostapedotomies and three malleostapedectomies. The medical records of these patients were retrospectively reviewed. The length of piston, ossicular condition, hearing improvement and postoperative complications were investigated. RESULTS: Conventional incus stapedotomy was not possible due to incus anomaly in three cases, incus fixation in two cases, incus removal during the previous surgery in one case and incus defect due to previous surgery in one case. The median length of prosthesis was 5.5 mm. The median preoperative air-bone gap was 42 dB and postoperative air-bone gap was 12 dB. The postoperative air-bone gap level was smaller than 10 dB in three patients, 11-20 dB in one patient, 21-30 dB in one patients, and 31-49 dB in one patient. Except for one case that had extrusion of the prosthesis after eight years, there was no intraoperative or postoperative complication. CONCLUSION: Malleostapedotomy or malleostapedectomy may be a good alternative surgical procedure to routine incus stapedotomy in cases of absence, anomaly or fixation of the incus.

Clinical Experiences of Diagnosis and Treatment for Wegener's Granulomatosis / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Because most patients with Wegener's granulomatosis (WG) visit otolaryngologists complaining of head and neck symptoms, otolaryngologists may play an important role in early diagnosis. In this study, we investigated clinical features, laboratory findings and treatment outcomes of 33 patients with WG, and propose a new algorithm for the diagnosis of WG. SUBJECTS AND METHOD: Thirty-three patients with WG who visited the clinic between January 1980 and December 2007 were included. The medical records of 13 male and 20 female patients were retrospectively reviewed. The mean follow-up duration was 68 months. RESULTS: Most WG patients had ENT symptoms such as nasal obstruction, rhinorrhea, otorrhea, hearing loss, dyspnea and hoarseness. The mean duration from ENT examination to diagnosis was 6.7 months (3 days- 102 months) and tissue biopsy was performed on an average of 2.1 times to confirm the diagnosis. While 16 patients could be diagnosed by the first biopsy, the others were required to repeat the biopsy. ANCA was positive in 17 patients (50%). Twentysix patients (78.7%) had multiple organ involvements such as lung, kidney, joint and skin. Twenty-eight patients received a combination of prednisolone and cyclophosphamide with the remission rate of 87%. CONCLUSION: Based on this study, we propose a better algorithm for the diagnosis of WG to avoid unnecessary delay in the diagnosis and treatment of WG.

Influence of Associated Medical Diseases and Complications on Functional Improvement after Rehabilitation in Patients with Stroke

OBJECTIVE: To investigate the influence of associated medical diseases and complications on functional improvement after in-patient through stroke rehabilitation. METHOD: We performed a retrospective analysis on medical records of 183 stroke patients who had admitted to the department of rehabilitation medicine. Functional Independence Measure (FIM), Modified Barthel Index (MBI) at admission and discharge were used to assess the functional status. We investigated medical diseases, such as hypertension, diabetes, myocardial infarct, atrial fibrillation osteoarthritis, rheumatoid arthritis, previous history of stroke and complications such as dementia, post-stroke depression, central post-stroke pain, complex regional pain syndrome, neglect and aphasia. RESULTS: Post-stroke patients with myocardial infarct, atrial fibrillation, osteoarthritis, dementia, aphasia and neglect significantly showed lower gain of FIM and MBI, lower FIM and MBI efficacy during inpatient rehabilitation compared to without those (p0.05). Total numbers of associated medical diseases and complications negatively affect on FIM and MBI efficacy (p<0.05). CONCLUSION: Therefore, it may be important to early detect and manage associated medical diseases and complications in post-stroke patients during rehabilitation, which improve the overall functional recovery of the patients.

A Case of Ganglioneuroblastoma Presenting as Weakness of the Lower Extremities

Ganglioneuroblastoma is a rare tumor originating from the sympathetic chain. It is intermediate between the highly malignant neuroblastoma and the benign ganglioneuroma. The predilection sites of ganglioneuroblastoma are the retroperitoneum and mediastinum but involvement of the structures around the spinal cord is infrequent. A 4 year-old girl who had progressive weakness in both lower extremities had a tiptoe gait for 2 months. A magnetic resonance imaging (MRI) study revealed a heterogeneous enhancing mass in the right mediastinum and paraspinal areas. A biopsy of the mass confirmed a ganglioneuroblastoma with many neuroblasts and nodules of ganglion cells. This is an unusual case of a child with spastic paraplegia caused by ganglioneuroblastoma of the thoracic cavity.

Resolution of Canal Paresis after Treatment in Benign Paroxysmal Positional Vertigo of the Horizontal Canal

Canal paresis in patients with benign paroxysmal positional vertigo (BPPV) has been variously reported from 26 to 50%. In such cases, BPPVs are developed secondary to accompanying vestibulopathy or other underlying inner ear diseases. Also the side of canal paresis is regarded as involved ear in the lateralization of horizontal canal BPPV (HC-BPPV), in which lateralization is sometimes ambiguous just by Eward's second law. In this case, authors report a woman who had HC-BPPV and pathologic canal paresis which resolved after canalith repositioning. This case may imply that dislodged particles block the endolymphatic flow resulting canal paresis.

Fractal and Complexity Measures of Heart Rate Dynamics in Patients with Normal and Left Ventricular Dysfunction: The Role of New Noninvasive Markers for Cardiac Risk Stratification

BACKGROUND AND OBJECTIVES: The traditional indexes of heart rate (HR) variability may lack the ability to detect subtle, but important changes in HR behavior. Nonlinear heart rate variability (HRV) analysis methods that are based on chaos theory can reveal subtle abnormalities in the HR dynamics of patients with cardiovascular diseases. Therefore, we tested the validity of nonlinear analysis methods as markers to differentiate normal and abnormal HR dynamics in the cardiovascular disease state. SUBJECTS AND METHODS: One-hundred patients were studied: 70 patients with left ventricular dysfunction (LVD), including 40 post-myocardial infarct patients (PMI) and 30 dilated cardiomyopahty patients (DCM), and 30 age and gender-matched controls. One-hour, 6-hours (day and night each) and 24 hours of R-R interval data from 24-hour Holter recordings were subjected to the conventional time and frequency-domain analysis. The ApEn, short-term (alpha1) and long-term (alpha2) scaling exponents of the detrended fluctuation analysis (DFA) and the power-law exponent (beta) were also measured. RESULTS: Conventional linear measures did not show a significant difference except for the VLF, lnLF and the LF/HF ratio between the controls and the LVD patients. Among the analyzed parameters, beta, beta2 and alpha1 were the most powerful discriminators. The beta of the normal and LVD patients was -1.10+/-0.29 and -0.70+/-0.40, respectively (p<0.001), and the alpha1 was 1.08+/-0.23 and 0.81+/-0.28, respectively (p<0.001). The beta, beta2 and alpha1 can discriminate the etiologic cause of LVD. The length of the R-R interval data did not affect the result, and a significant correlation was observed. The individual values of the fractal and complexity measures were more stable than those of the conventional linear measures. CONCLUSION: We conclude that the measures derived from fractal and complexity methods are useful for detecting altered HR dynamics of LVD and for improving the shortcomings of the conventional measures.

A Case of Small Cell Variant of T-Cell Prolymphocytic Leukemia

T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy that has an aggressive clinical course and it is a distinct clinico-biological entity from other T-cell disorders. It is now apparent that this disease represents a separate entity from CLL. Clinically, T-PLL presents with hepatosplenomegaly, lymphadenopathy, skin lesion, and marked lymphocytosis exceeding 100x109/L. Because its clinical course is aggressive, the treatment is difficult. We report a case of small cell variant of T-cell with a review of literatures.