Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature.

BACKGROUND: Pure red cell aplasia (PRCA) is less common blood disorder; the causes and the treatments of PRCA are varied. METHODS: We conducted a retrospective study during January 2010-December 2017, to explore the etiologies and to evaluate the response and treatment burden in adult patients with PRCA. RESULTS: Of 32 PRCA patients, median age was 57 years (18-90 years). Median hemoglobin level and reticulocyte count at the time of diagnosis were 5.6 g/dL (3.3-7.3 g/dL) and 0.3% (0.1-0.7%), respectively. Median time to hematologic recovery was 12 weeks (3-72 weeks), and median number of red blood cell transfusion (RBC) was 20 units (4-100 units). Causes of PRCA were erythropoiesis-stimulating agent (ESA) (47%), parvovirus B19 infection (19%), thymoma (13%), zidovudine (6%), primary autoimmune PRCA (6%), Kaposi's sarcoma (3%), systemic lupus erythematosus (3%), and ABO-mismatched stem cell transplantation (3%). Only 9 out of 24 treated patients achieved hematologic response within 8 weeks of treatment. Intravenous immunoglobulin therapy provided 100% response rate in patients with parvovirus B19-associated PRCA and primary autoimmune PRCA. Low response rate was found in patients receiving immunosuppressants and chemotherapy for the treatment of ESA and thymoma-associated PRCA, respectively. CONCLUSIONS: Treatment outcome of PRCA depended upon the causes and the types of treatment, and the burden of RBC transfusion was very high in patients with ESA and thymoma-associated PRCA.

Comparison of different transbronchial biopsy sampling techniques for the diagnosis of peripheral pulmonary lesions with radial endobronchial ultrasound-guided bronchoscopy: A prospective study.

BACKGROUND: Data regarding the transbronchial biopsy (TBB) techniques in radial endobronchial ultrasound (R-EBUS)-guided bronchoscopy are limited. The purpose of this study was to compare three R-EBUS-guided TBB techniques for the diagnosis of peripheral pulmonary lesions (PPLs). METHODS: A prospective pilot study was conducted including 90 patients with positive bronchus sign PPLs, who underwent R-EBUS-guided TBB. TBB techniques were performed in all patients using small biopsy forceps with a guide sheath (GS). These samples were submitted for both cell block histology (CB) and conventional histology (SB). Standard biopsy forceps were used to collect further samples that were submitted for conventional histology (LB). The diagnostic yields of the three techniques were compared. RESULTS: The mean diameter of the PPLs was 25.5 ± 8.2 mm and the final diagnoses included 70 malignant and 20 benign lesions. The overall diagnostic yield of R-EBUS-guided bronchoscopy was 82.2%. Although the difference was not statistically significant, CB provided the highest yield of the three TBB techniques: 68.9%, 65.6%, and 62.2% for CB, SB, and LB, respectively (P = 0.20). When the GS was removed and standard biopsy forceps were introduced, misplacement (detected by fluoroscopy) was observed in 24 cases, and LB provided a diagnosis in 11 cases. PPLs ≤20 mm were associated with misplacement (P = 0.003). After the exclusion of misplaced cases, the diagnostic yields were 69.7%, 71.2%, and 68.2% for CB, SB, and LB, respectively (P = 0.65). CONCLUSIONS: Neither the size of biopsy forceps nor the histology process affected the diagnostic yield of R-EBUS-guided bronchoscopy.

Non-Hodgkin lymphoma in South East Asia: An analysis of the histopathology, clinical features, and survival from Thailand.

Systemic reports on the descriptive epidemiology of non-Hodgkin lymphoma (NHL) from Southeast Asia are scarce. A nationwide multi-institutional registry was conducted to compare the histopathology, clinical features, and survival of Thai adult patients with NHL using large registries, especially those from Far East Asia (FEA). Using a web-based registry system, 13 major medical centers from the 4 geographic regions of Thailand prospectively collected, from 2007 to 2014, the diagnostic pathology, according to the World Health Organization classification, 2008, clinical features and survival of 4056 patients who were newly diagnosed with NHL. The median age of the patients was 56 years (range, 16-99 years). The male-to-female ratio was 1.3:1. From the total of 4056 patients, T/NK-cell lymphoma (TNKCL) accounted for 12.6% of cases, and 5.1% had human immunodeficiency virus-associated lymphoma. The four leading histological subtypes were diffuse large B-cell lymphoma, not otherwise specified (58.1%); follicular lymphoma (5.6%); extranodal mucosa-associated lymphoid tissue lymphoma (5.2%); and peripheral T-cell lymphoma, not otherwise specified (4.0%). With a median follow-up duration of 46.1 months, the median overall survival of B-cell NHL was significantly longer than that of patients with TNKCL (76.5 vs 28.8 months, P = .0001). Compared to FEA, the Thai registry had an approximately one-half lower relative frequency of TNKCL; the prevalence of extranodal mucosa-associated lymphoid tissue lymphoma was much lower than in Korea, and the frequency of extranodal TNKCL, nasal type, was strikingly low compared to China. It is concluded that while the median age of Thai patients with NHL was approximately a decade younger than for Caucasians, the long-term survival rates for most histological subtypes were comparable. While the histological distribution generally complied with the characteristic Asian features, some differences from FEA were observed.

Comparison of diagnostic performances among bronchoscopic sampling techniques in the diagnosis of peripheral pulmonary lesions.

BACKGROUND: There are many sampling techniques dedicated to radial endobronchial ultrasound (R-EBUS) guided flexible bronchoscopy (FB). However, data regarding the diagnostic performances among bronchoscopic sampling techniques is limited. This study was conducted to compare the diagnostic yields among bronchoscopic sampling techniques in the diagnosis of peripheral pulmonary lesions (PPLs). METHODS: A prospective study was conducted on 112 patients who were diagnosed with PPLs and underwent R-EBUS-guided FB between Oct 2012 and Sep 2014. Sampling techniques-including transbronchial biopsy (TBB), brushing cell block, brushing smear, rinsed fluid of brushing, and bronchoalveolar lavage (BAL)-were evaluated for the diagnosis. RESULTS: The mean diameter of the PPLs was 23.5±9.5 mm. The final diagnoses included 76 malignancies and 36 benign lesions. The overall diagnostic yield of R-EBUS-guided bronchoscopy was 80.4%; TBB gave the highest yield among the 112 specimens: 70.5%, 34.8%, 62.5%, 50.0% and 42.0% for TBB, brushing cell block, brushing smear, rinsed brushing fluid, and BAL fluid (BALF), respectively (P<0.001). TBB provided high diagnostic yield irrespective of the size and etiology of the PPLs. The combination of TBB and brushing smear achieved the maximum diagnostic yield. Of 31 infectious PPLs, BALF culture gave additional microbiological information in 20 cases. CONCLUSIONS: TBB provided the highest diagnostic yield; however, to achieve the highest diagnostic performance, TBB, brushing smear and BAL techniques should be performed together.

Peniciliosis endobronquial: presentación de un caso y revisión de la literatura / No disponible

La peniciliosis es una infección oportunista que se da en pacientes con infección por el VIH y otros pacientes inmunodeprimidos, sobre todo en el Sudeste Asiático, el sur de China, Hong Kong y Taiwán. Se producen manifestaciones respiratorias en alrededor de una tercera parte de los pacientes. Presentamos aquí el caso de un paciente de 26 años de edad inmunodeprimido, sin VIH, que comenzó con una peniciliosis endobronquial que obstruía las vías aéreas, junto con una revisión de la literatura de este trastorno muy poco frecuente

Penicilliosis is an opportunistic infection in HIV-infected and other immunocompromised patients mostly in Southeast Asia, Southern China, Hong Kong, and Taiwan, with respiratory manifestations in about onethird of patients. We report the case of a 26-year-old non-HIV immunocompromised patient presenting with an airway obstruction caused by penicilliosis, together with a review of the literature of this rare condition

Airway obstruction caused by penicilliosis: a case report and review of the literature.

Penicilliosis is an opportunistic infection in HIV-infected and other immunocompromised patients mostly in Southeast Asia, Southern China, Hong Kong, and Taiwan, with respiratory manifestations in about one-third of patients. We report the case of a 26-year-old non-HIV immunocompromised patient presenting with an airway obstruction caused by penicilliosis, together with a review of the literature of this rare condition.

Esthesioneuroblastoma metastasis to the breast: A case report and review of the literature.

Metastasis to the breast from an extramammary malignant neoplasm, including esthesioneuroblastoma, is uncommon. The present study describes a rare case of sinonasal esthesioneuroblastoma, Hyams' histologic grade 2, Kadish's stage C, T4N0M0, in a 30-year-old female. The patient underwent a radical ethmoidectomy with external beam radiotherapy, followed by chemotherapy including five cycles of cisplatin and etoposide. One year after the initial diagnosis, the patient presented to the hospital with the chief complaint of a rapidly enlarging lump in the right breast. A fine needle aspiration was performed and immunocytochemistry revealed a metastatic esthesioneuroblastoma. The patient received palliative chemotherapy and radiotherapy; however, the patient developed a local recurrence with systemic metastasis and succumbed to the disease seven months later.

Diagnóstico de lesiones pulmonares periféricas con broncoscopia bajo guía de ecografía endobronquial radial / Diagnosis of Peripheral Pulmonary Lesions With Radial Probe Endobronchial Ultrasound-Guided Bronchoscopy

Introducción: El diagnóstico de las lesiones pulmonares periféricas (LPP) constituye un verdadero reto para los neumólogos. La ecografía endobronquial radial (R-EBUS) es una técnica que se ha desarrollado para mejorar el rendimiento diagnóstico. El objetivo de este estudio fue evaluar la efectividad de la R-EBUS en el diagnóstico de las LPP. Métodos: Se llevó a cabo un estudio retrospectivo en 174 pacientes a los que se habían diagnosticado LPP y a los que se practicó una broncoscopia bajo guía de EBUS. Para el diagnóstico se evaluó el examen histológico de las muestras obtenidas mediante biopsia pulmonar transbronquial (BPTB) y los exámenes citológicos del frotis de cepillado, líquido de irrigación del cepillado y líquido de lavado broncoalveolar (LLBA). Resultados: El diámetro medio de las LPP fue de 25,1 ± 10,7 mm. Los diagnósticos finales fueron 129 enfermedades malignas y 45 lesiones benignas. El rendimiento diagnóstico global de la broncoscopia bajo guía de EBUS fue del 79,9%. Ni el tamaño ni la etiología de las LPP mostraron influencia alguna en el rendimiento diagnóstico de la broncoscopia bajo guía de EBUS (82,9% frente al 74,6% para las LPP de tamaño > 20 mm y las LPP de tamaño ≤ 20 mm; p = 0,19, y 82,9% frente al 71,1% para las enfermedades malignas y benignas; p = 0,09). La BPTB fue la que obtuvo el máximo rendimiento de entre estas diversas muestras (69,0, 50,6, 42,0 y 44,3% para las muestras de BPTB, frotis de cepillado, líquido de irrigación de cepillado y LLBA, respectivamente; p < 0,001). La combinación de BPTB, frotis de cepillado y LLBA aportó el máximo rendimiento diagnóstico, mientras que el líquido de irrigación de cepillado no añadió una mejora adicional de los resultados. Conclusión: La broncoscopia bajo guía de R-EBUS es una técnica útil en el diagnóstico de las LPP. Para alcanzar el máximo rendimiento diagnóstico deben utilizarse conjuntamente la BPTB, el frotis de cepillado y el lavado broncoalveolar

Introduction: The diagnosis of peripheral pulmonary lesions (PPLs) is a challenging task for pulmonologists. Radial probe endobronchial ultrasound (R-EBUS) has been developed to enhance diagnostic yield. The objective of this study was to evaluate the effectiveness of R-EBUS in the diagnosis of PPLs. Methods: A retrospective study was conducted on 174 patients diagnosed with PPLs who underwent EBUS-guided bronchoscopy. Histological examination of specimens obtained by transbronchial lung biopsy (TBLB) and cytological examinations of brushing smear, brush rinse fluid and bronchoalveolar lavage fluid (BALF) were evaluated for the diagnosis. Results: The mean diameter of the PPLs was 25.1 ± 10.7 mm. The final diagnoses included 129 malignancies and 45 benign lesions. The overall diagnostic yield of EBUS-guided bronchoscopy was 79.9%. Neither size nor etiology of the PPLs influenced the diagnostic performance of EBUS-guided bronchoscopy (82.9% vs 74.6% for PPLs >20 mm and PPLs ≤ 20 mm; P=0.19, and 82.9% vs 71.1% for malignancy and benign diseases; P = 0.09). TBLB rendered the highest yield among these specimens (69.0%, 50.6%, 42.0%, and 44.3% for TBLB, brushing smear, brush rinse fluid, and BALF, respectively; P < 0.001). The combination of TBLB, brush smear, and BALF provided the greatest diagnostic yield, while brush rinse fluid did not add benefits to the outcomes. Conclusion: R-EBUS-guided bronchoscopy is a useful technique in the diagnosis of PPLs. To achieve the highest diagnostic performance, TBLB, brushing smear and bronchoalveolar lavage should be performed together

Interobserver variation in classifying lymphomas among hematopathologists.

BACKGROUND: Lymphomas are common malignancies that have various subtypes with many overlapping histologic, immunophenotypic and genetic features. Therefore, discordance in classifying lymphoma among pathologists may be encountered. But this issue is not well characterized. We conducted the present study to demonstrate discordances among Thai hematopathologists as well as to highlight common arguing points for classifying lymphomas. METHODS: The 117 lymphoma cases were randomly retrieved and individually reviewed by 7 hematopathologists, members of the "Thai Hematopathologist Group," without knowing the original diagnoses. The consensus diagnoses were given from a discussion by all members. In each case, the diagnosis from each participant was compared with the consensus diagnosis and classified into 4 categories as follow: 1) concordance, 2) minor discordance, 3) major discordance and 4) serious discordance. RESULTS: There were approximately 11% discordances between original and consensus diagnoses. The average discordances among all pathologists according to minor, major and serious discordances were 10%, 3.5% and 0.3%, respectively. Diffuse large B-cell lymphoma had the least discordance (7%). Small biopsies had been found to increase discordances in some lymphoma subtypes. CONCLUSIONS: The present study reveals some degrees of interobserver variation in classifying of lymphoma by using the 2008 WHO classification among hematopathologists. Some types of lymphomas on small biopsies were found to have a significant higher discordance rate. This study also described some common diagnostic discordances regarded as potential pitfalls in classifying lymphomas. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_162.

Diagnosis of peripheral pulmonary lesions with radial probe endobronchial ultrasound-guided bronchoscopy.

INTRODUCTION: The diagnosis of peripheral pulmonary lesions (PPLs) is a challenging task for pulmonologists. Radial probe endobronchial ultrasound (R-EBUS) has been developed to enhance diagnostic yield. The objective of this study was to evaluate the effectiveness of R-EBUS in the diagnosis of PPLs. METHODS: A retrospective study was conducted on 174 patients diagnosed with PPLs who underwent EBUS-guided bronchoscopy. Histological examination of specimens obtained by transbronchial lung biopsy (TBLB) and cytological examinations of brushing smear, brush rinse fluid and bronchoalveolar lavage fluid (BALF) were evaluated for the diagnosis. RESULTS: The mean diameter of the PPLs was 25.1 ± 10.7 mm. The final diagnoses included 129 malignancies and 45 benign lesions. The overall diagnostic yield of EBUS-guided bronchoscopy was 79.9%. Neither size nor etiology of the PPLs influenced the diagnostic performance of EBUS-guided bronchoscopy (82.9% vs. 74.6% for PPLs>20mm and PPLs≤20mm; p=0.19, and 82.9% vs. 71.1% for malignancy and benign diseases; p=0.09). TBLB rendered the highest yield among these specimens (69.0%, 50.6%, 42.0%, and 44.3% for TBLB, brushing smear, brush rinse fluid, and BALF, respectively; p<0.001). The combination of TBLB, brush smear, and BALF provided the greatest diagnostic yield, while brush rinse fluid did not add benefits to the outcomes. CONCLUSION: R-EBUS-guided bronchoscopy is a useful technique in the diagnosis of PPLs. To achieve the highest diagnostic performance, TBLB, brushing smear and bronchoalveolar lavage should be performed together.

Fungal appendicitis: a case series and review of the literature.

Appendicitis is a condition characterized by inflammation of the vermiform appendix, which is commonly caused by bacterial infections and rarely caused by fungal organisms. In the present study, we reviewed the prevalence, clinicopathological features, and therapeutic management of fungal appendicitis. During July 2010 to June 2011, the pathology of 262 resected vermiform appendices was reviewed. Fungal appendicitis occurred in 1.15%, including two cases of Candida spp and one case of Aspergillus spp infection. All patients were immunocompromised and presented with the classical signs and symptoms of appendicitis with the onset of illness less than two days. They were considered for acute appendicitis and underwent appendectomy. The histopathology of the resected vermiform appendix showed fungal organisms with suppurative inflammation and secondary periappendiceal peritonitis. The curative treatment was presented in 1-out-of-3 cases. One patient was alive during a follow-up of eight months. Two patients died, and an autopsy was performed in one case. Although fungal appendicitis was uncommon, the disease might occur among immunosuppressed patients who have developed classical signs and symptoms of appendicitis. Early diagnosis and prompt surgery with medical treatment are associated with a survival advantage.

Extranodal NK/T-cell lymphoma, nasal type, includes cases of natural killer cell and αß, γδ, and αß/γδ T-cell origin: a comprehensive clinicopathologic and phenotypic study.

Extranodal NK/T-cell lymphoma (ENKTL), nasal type, may be of NK or T-cell origin; however, the proportion of T-ENKTLs and whether they are of αß or γδ type remains uncertain. To elucidate the cell of origin and detailed phenotype of ENKTL and assess any clinicopathologic associations, 67 cases of ENKTL from Thailand were investigated, together with 5 γδ enteropathy-associated T-cell lymphomas (EATLs) for comparison. In all, 70% of the ENKTL were T-cell receptor (TCR) ß,γ and, in cases tested, δ negative (presumptive NK origin); 5% were TCR γδ, 3% were TCR αß, 1% were TCR αß/γδ, and 21% were indeterminate. Out of 17 presumptive NK-ENKTLs tested, 3 had clonal TCR rearrangements. All cases were EBV and TIA-1; >85% were positive for CD3, CD2, granzyme B, pSTAT3, and Lsk/MATK; and all were CD16. Presumptive NK-ENKTLs had significantly more frequent CD56 (83% vs. 33%) and CXCL13 (59% vs. 0%) but less frequent PD-1 (0% vs. 40%) compared with T-ENKTLs. Of the NK-ENKTLs, 38% were Oct-2 compared with 0% of T-ENKTLs, and 54% were IRF4/MUM1 compared with 20% of T-ENKTLs. Only αß T-ENKTLs were CD5. Intestinal ENKTLs were EBV and had significantly more frequent CD30, pSTAT3, and IRF4/MUM1 expression but less frequent CD16 compared with γδ EATL. Significant adverse prognostic indicators included a primary non-upper aerodigestive tract site, high stage, bone marrow involvement, International Prognostic Index ≥2, lack of radiotherapy, Ki67 >40%, and CD25 expression. The upper aerodigestive tract ENKTLs of T-cell origin compared with those of presumptive NK origin showed a trend for better survival. Thus, at least 11% of evaluable ENKTLs are of T-cell origin. Although T-ENKTLs have phenotypic and some possible clinical differences, they share many similarities with ENKTLs that lack TCR expression and are distinct from intestinal γδ EATL.

Infection-associated hemophagocytic syndrome among patients with dengue shock syndrome and invasive aspergillosis: a case series and review of the literature.

The authors report four autopsy cases of previously healthy children with dengue shock syndrome complicated with infection-associated hemophagocytosis and invasive aspergillosis. Hemophagocytosis is confirmed by histopathology of autopsied reticuloendothelial organs. All four children were identified to have invasive aspergillosis by histopathology and three cases were positive on fungal culture for Aspergillus spp. Regarding the cause of death among the four children without pre-existing underlying disease, three cases were directly ascribable to invasive aspergillosis and the remaining case was ascribed to dengue shock syndrome. The transmigration of preexisting fungi from the respiratory mucosa damaged by the dengue shock process is postulated as the pathogenesis of invasive aspergillosis. The main predisposing factor was found to be prolonged dengue shock syndrome. We reviewed the clinicopathologic features and therapeutic management of infection-associated hemophagocytic syndrome in patients with dengue shock syndrome and invasive aspergillosis.

Double check up of malignancy biopsy specimens for patient safety.

BACKGROUND: The diagnostic of malignancy in biopsy specimens is very important because it guides to selected treatment option and prognostic prediction. However biopsy specimens usually have small pieces leading to variations of the interpretation by anatomical pathologists. OBJECTIVE: To detect and correct the errors or the significant discrepancies in the diagnosis of biopsy specimens before sign-out and to determine the frequency of anatomic pathology significant discrepancies. DESIGN: The application of the mutually agreed work instructions (record) for the detection of errors or the significant discrepancies and their process of sign-out. The record of biopsy specimen that received a secondary check (1959 cases, 2005-2007) was analyzed. RESULTS: After a secondary check, 53 cases of non-malignancy for any reason by a second pathologist were included. However when using our definition on significant discrepancies, only 37 cases were considered. Another seven cases with the opinions with malignancy that were of different cell types that do harm to the patients were added. Therefore, 44 cases (2.25%) had truly significant discrepancies. CONCLUSION: The truly significant discrepancy frequency was 2.25% during the process of pre-sign-out secondary check of malignancy of biopsy specimens. The project has been applied as a routine daily work. It can be an innovative safety program for patient in Thailand.

Eosinophilic appendicitis caused by Schistosoma japonicum: a case report and review of the literature.

Parasitic appendicitis is uncommon. The authors reviewed the pathology of 4,130 appendices resected over the past 10 years (2000 to 2009). Only one case of eosinophilic appendicitis caused by Schistosoma japonicum was identified. The overall prevalence of schistosomal appendicitis was 0.024%. The case was a 61-year-old woman who presented with right lower quadrant abdominal pain. She had been a farmer in Chumphon and Surat Thani Provinces, which are endemic for schistosomiasis in Thailand. Physical, laboratory and ultrasound examinations were suggestive of acute appendicitis. She underwent emergency appendectomy. Intraoperative findings revealed a ruptured appendix with a fecalith in the appendiceal lumen. The histopathologic diagnosis was suppurative eosinophilic appendicitis with schistosomal ova in the mucosa, submucosa, muscular layer and vascular lumens, identified as S. japonicum eggs. The patient was treated for the parasite with praziquantal. We briefly review the clinicopathologic features and pathogenesis of schistosomal appendicitis.

Endoscopic removal of one live biliary Fasciola gigantica.

Biliary fascioliasis was diagnosed in a woman from Angthong province, who presented herself with anorexia, weight loss, and jaundice for one month. Intraoperative retrograded cholangiopancreatography revealed a filling defect considered as a stone and bile sludge. By the sphincterotomy and balloon-extraction technique, one live fluke was removed and identified as Fasciola gigantica. A single dose of praziquantel, 25 mg/kg of body weight/day, was given although the postoperative stool examination revealed no Fasciola spp. egg. The patient was doing well after the fluke removal. According to a previous review, in this country, Ayutthaya is the southernmost province for the distribution of fascioliasis.