Secondary Pulmonary Alveolar Proteinosis in Hematologic
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Supplementary Figure 1. Murine and human GATA2 promoter alignment shows highly conserved regions. Brackets delimit the highly conserved regions in both genomes, as graphics obtained from ECR Browser show: large areas marked in red indicate highly similar intergenic sequences, repetitive sequences in green, exonic sequences in yellow, and intronic sequences in salmon color. Horizontal axis represents base positions in the genome and the vertical axis represents the percentage identity between the human and mouse genomes. GATA2 binding sites are indicated in red within the brackets, and they were located using the TFSearch, Match and MatInspector transcription factor binding site finder algorithms. Comparison of GATA2 binding sites between both genomes and several surrounding nucleotides is shown in the middle, where GATA2 binding site core sequences are shown in italic and underlined. Two GATA2 binding sites at -2.8 belonged to a highly conserved region spanning 389 bp, with 87.7% homology with the human GATA2 promoter. Similarly, other two GATA2 binding sites at -1.8 were located within a 282 bp region with 75.5% homology. Additionally, a third 142 bp region was taken into account containing the -2992 GATA2 binding site, which showed 68.3% homology. Supplementary Table I: List of reported germline GATA2 mutations and associated syndromes. Mutation (DNA)* Affected exon Mutation (protein) Mutation type Affected ZF Reference Cases/families Associated syndrome c.1-200_871+527del2033 4, 5 p.Met1_Ser290del In-frame deletion None 4,7,20,S1 2/1 -
Letters to the Editor
LETTERS TO THE EDITOR was discharged on long-term therapy with clar - + MonoMAC versus idiopathic CD4 lymphocytopenia . ithromycin, ethambutol, and rifampin. His skin lesions Comment to Haematologica. 2011;96(8):1221-5 resolved within a month of starting therapy. The cause of pancytopenia was initially thought to be due to BM sup - pression associated with disseminated MAC infection. We read with interest a recent article in Haematologica However, pancytopenia did not resolve and a BM biopsy describing a novel defined disorder known as MonoMAC was performed. This showed a markedly hypocellular (monocytopenia with Mycobacterium avium complex). 1 It is not infrequent for patients with MonoMAC to present with a low CD4 count, leading to a consideration of idio - pathic CD4-positive (CD4 +) lymphocytopenia (ICL). 2 Table 1. Summary of patient’s laboratory findings. Here we describe a case of MonoMAC who was initially Patient Reference range thought to have ICL and aplastic anemia. However, the combination of pancytopenia, profound monocytopenia, WBC 1.2 ¥10 9/L 4.1-10.9 ¥10 9/L and bone marrow (BM) findings support a diagnosis of Absolute neutrophil count 876 cells/ mL 1400-6500 cells/ mL MonoMAC. Absolute lymphocyte count 144 cells/ mL 1200-3400 cells/ mL A 24-year old Hispanic male presented with 2 ulcers on the lower extremities that he had had for two months. Absolute monocyte count 12 cells/ mL 300-820 cells/ mL These ulcers were 1.0 to 3.5 cm in size at their widest Hemoglobin 5.2 g/dL 14.0-18.0 g/dL point and had slightly raised erythematous borders with Hematocrit 16.2% 40-54% pink-glistening to black eschar bases (Figure 1A). -
Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-Cov-2) and Coronavirus Disease 19 (COVID-19) – Anatomic Pathology Perspective on Current Knowledge Sambit K
Mohanty et al. Diagnostic Pathology (2020) 15:103 https://doi.org/10.1186/s13000-020-01017-8 REVIEW Open Access Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and coronavirus disease 19 (COVID-19) – anatomic pathology perspective on current knowledge Sambit K. Mohanty1,2†, Abhishek Satapathy2†, Machita M. Naidu2, Sanjay Mukhopadhyay3, Shivani Sharma1, Lisa M. Barton4, Edana Stroberg4, Eric J. Duval4, Dinesh Pradhan5, Alexandar Tzankov6 and Anil V. Parwani7* Abstract Background: The world is currently witnessing a major devastating pandemic of Coronavirus disease-2019 (COVID- 19). This disease is caused by a novel coronavirus named Severe Acute Respiratory Syndrome Coronavirus-2 (SARS- CoV-2). It primarily affects the respiratory tract and particularly the lungs. The virus enters the cell by attaching its spike-like surface projections to the angiotensin-converting enzyme-2 (ACE-2) expressed in various tissues. Though the majority of symptomatic patients have mild flu-like symptoms, a significant minority develop severe lung injury with acute respiratory distress syndrome (ARDS), leading to considerable morbidity and mortality. Elderly patients with previous cardiovascular comorbidities are particularly susceptible to severe clinical manifestations. Body: Currently, our limited knowledge of the pathologic findings is based on post-mortem biopsies, a few limited autopsies, and very few complete autopsies. From these reports, we know that the virus can be found in various organs but the most striking tissue damage involves the lungs resulting almost always in diffuse alveolar damage with interstitial edema, capillary congestion, and occasional interstitial lymphocytosis, causing hypoxia, multiorgan failure, and death. A few pathology studies have also reported intravascular microthrombi and pulmonary thrombembolism. -
Pandemic Influenza A/H1N1: Comparative Analysis of Microscopic Lung Histopathological Findings
ARTIGO ORIGINAL Influenza pandêmica A/H1N1: análise comparativa de alterações histopatológicas pulmonares Pandemic influenza A/H1N1: comparative analysis of microscopic lung histopathological findings Roberta Marchiori1, Carla Sakuma de Oliveira Bredt2, Marcos Menezes Freitas de Campos1, Fábio Negretti1, Péricles Almeida Delfino Duarte1 RESUMO Care Unit of a university hospital in 2009. Nasopharyngeal aspirate Objetivo: Analisar as alterações histológicas pulmonares de quatro specimens were collected from the patients and were analyzed by real-time polymerase chain reaction. Lung biopsy was performed casos fatais de influenza pandêmica H1N1, correlacionando-os a post mortem; a score of intensity for pathological changes was características clínico-epidemiológicas. Métodos: Estudo retrospectivo applied. Results: Three patients had positive real-time polymerase e descritivo de dados de prontuários de quatro pacientes que chain reaction (although all of them had a clinical diagnose of faleceram por influenza H1N1 na Unidade de Terapia Intensiva de um influenza H1N1). The main histopathological changes were: exudative hospital universitário, em 2009. Os pacientes haviam sido submetidos diffuse alveolar damage with atelectasis; varying degrees of alveolar a aspirado de nasofaringe e as amostras foram analisadas pelo hemorrhage and edema, necrosis and sloughing of the respiratory método de reação em cadeia da polimerase em tempo real. Biópsia epithelium in several bronchioli; and thrombus formation. One of the pulmonar foi realizada no dia do óbito; um escore de intensidade das patients (the pregnant one) presented histopathological findings of alterações histopatológica foi aplicado. Resultados: Três pacientes cytomegalic inclusion. Conclusion: The pulmonary histopathological apresentaram reação em cadeia da polimerase em tempo real com findings in patients with fatal 2009 H1N1 influenza pandemic resultado positivo (embora todos tivessem diagnóstico de influenza disclosed intense alveolar damage and hemorrhage and severe H1N1). -
A Case of Cryptogenic Organizing Pneumonia in a Seventh-Decade Woman
Saudi Journal of Medicine ISSN 2518-3389 (Print) Scholars Middle East Publishers ISSN 2518-3397 (Online) Dubai, United Arab Emirates Website: http://scholarsmepub.com/ A case of Cryptogenic Organizing Pneumonia in a seventh-decade woman. "Yahya Al-FIFI’s Diagnostic Criteria for Cryptogenic Organizing Pneumonia (COP) Without Lung Tissues Biopsies for Histopathology". Is This the Truth of the Reality Or The Reality of the Truth? Yahya Salim Yahya AL-FIFI Consultant, Internal Medicine &Infectious Diseases, Department of Medicine, Infection Diseases Division, Prince Mohammad Bin Nasser Hospital, Jizan, Jazan, Saudi Arabia. Email: [email protected] Abstract: We describe the first and rare case report of a cryptogenic organizing *Corresponding author pneumonia (COP) in a seventh decade diabetic and hypertensive woman from low Yahya Salim Yahya highlands, Jazan, Saudi Arabia. The evidence of the clinical scenario, laboratories testing, radiological images findings followed by a significant improvement due to Article History steroid treatment are quite enough to diagnose COP, irrespective of the lung tissues Received: 19.11.2017 biopsies procedures and processing accessibility for histopathology, in a timely manner Accepted: 24.11.2017 as reveals in “Yahya Al-FIFI’s diagnostic criteria for cryptogenic organizing pneumonia Published: 30.11.2017 (COP) without lung tissues biopsies for histopathology”. We started a methylprednisolone forty milligrams intravenously every eight hourly for seven days DOI: which is showing a dramatic clinical improvement within initial twenty-four hours of 10.21276/sjm.2017.2.7.4 the first seven days and complete recovery clinically and radiologically, at the end of the following fourteen days of tapering prednisolone doses without a relapse for seven months. -
Lansdell Vs. Georgia-Pacific Corporation Awcc# F007360
BEFORE THE ARKANSAS WORKERS’ COMPENSATION COMMISSION CLAIM NO. F007360 ALVIN LANSDELL, EMPLOYEE CLAIMANT GEORGIA-PACIFIC CORPORATION, SELF-INSURED EMPLOYER RESPONDENT OPINION FILED SEPTEMBER 3, 2003 Upon review before the FULL COMMISSION in Little Rock, Pulaski County, Arkansas. Claimant represented by HONORABLE GREGORY R. GILES, Attorney at Law, Texarkana, Arkansas. Respondent represented by HONORABLE MARK A. PEOPLES, Attorney at Law, Little Rock, Arkansas. Decision of the Administrative Law Judge: Affirmed as modified. OPINION AND ORDER The claimant appeals an Administrative Law Judge’s opinion filed August 21, 2002. The Administrative Law Judge found that Act 1281 of 2001 made substantive law changes to the burden of proof for occupational disease and was to be applied prospectively. The Administrative Law Judge therefore found, “Claimant has failed to prove by clear and convincing evidence that he sustained an occupational disease which arose out of and in the course of his employment.” After reviewing the entire record de novo, the Lansdell - F007360 2 Full Commission finds that our recent decision in a companion case, Sikes v. Georgia-Pacific Corporation, Workers’ Compensation Commission F000657 (July 7, 2003), is controlling in this matter as to the appropriate burden of proof. We therefore find that the Legislature meant to apply Act 1281 retroactively, so that the “preponderance of the evidence” standard of Ark. Code Ann. § 11-9-601 (e)(1)(B) applies to the instant matter. The Full Commission further finds that the claimant failed to prove by a preponderance of the evidence that he sustained a compensable occupational disease. We therefore affirm, as modified, the opinion of the Administrative Law Judge. -
Editorials & Perspectives
EDITORIALS & PERSPECTIVES Dendritic cell, monocyte, B and NK lymphoid deficiency defines the lost lineages of a new GATA-2 dependent myelodysplastic syndrome Venetia Bigley and Matthew Collin Institute of Cellular Medicine, Newcastle University, UK. E-mail: [email protected] doi:10.3324/haematol.2011.048355 (Related Original Article on page 1221) novel immunodeficiency syndrome has recently been International Prognostic Scoring System (IPSS). defined in young adults. Known variously as dendritic It is worth emphasizing at this point that DCML deficiency cell, monocyte, B and NK lymphoid (DCML deficien - patients may have significant risk to their health before addi - A1 2 2 cy), ‘autosomal dominant and sporadic monocytopenia’ or tional cytopenias evolve. Disseminated mycobacterial infec - ‘MonoMAC’ (monocytopenia with Mycobacterium avium com - tion, PAP and carcinoma in situ may all occur prior to the onset plex), 3 it is characterized by a composite mononuclear cell defi - of an obvious myelodysplastic syndrome, underscoring the ciency, atypical mycobacterial and viral infection, and progres - fact that immunodeficiency is a cardinal feature of the disor - sion to myelodysplasia and leukemia. Strikingly, a number of der. In the series of 4 subjects described in 2011, one had mild patients also develop pulmonary alveolar proteinosis (PAP). anemia (10.3 g/dL) and one mild thrombocytopenia Patients typically present in their 3 rd or 4 th decade, although his - (123 ¥10 9/L), yet 2 required hematopoietic stem cell transplan - torical blood counts may show pre-existing monocytopenia tation (one for refractory mycobacterial infection, one for PAP) for ten years or more. A substantial number of patients devel - and a third died of influenza H1N1 prior to transplantation. -
H5N1-Infected Cells in Lung with Diffuse Alveolar Damage in Exudative Phase from a Fatal Case in Vietnam
Jpn. J. Infect. Dis., 61, 157-160, 2008 Short Communication H5N1-Infected Cells in Lung with Diffuse Alveolar Damage in Exudative Phase from a Fatal Case in Vietnam Nguyen Thanh Liem, Noriko Nakajima1, Le Phuc Phat**, Yuko Sato1, Hoang Ngoc Thach, Pham Viet Hung, Luong Thi San, Harutaka Katano1, Toshio Kumasaka1,2, Teruaki Oka3, Shoji Kawachi4, Takeji Matsushita4, Tetsutaro Sata1, Koichiro Kudo4 and Kazuo Suzuki1,5* The National Hospital of Pediatrics, Hanoi, Vietnam; 1National Institute of Infectious Diseases, Tokyo 162-8640; 2Juntendo University School of Medicine, Tokyo 113-8421; 3Kanto Central Hospital, Tokyo 158-8531; 4International Medical Center of Japan, Tokyo 162-8655; and 5Chiba University Graduate School of Medicine, Chiba 260-8670, Japan (Received October 23, 2007. Accepted February 4, 2008) SUMMARY: Necropsied lung tissues of three fatal cases with avian influenza A virus (H5N1) infection in Vietnam were analyzed to detect H5N1 virus-infected cells. Formalin-fixed and paraffin-embedded lung tissue sections showed typical histological features of diffuse alveolar damage (DAD) in all cases. Immunohisto- chemistry for the influenza A virus nucleoprotein antigen revealed positive signals of bronchiolar and alveolar epithelial cells in only one patient, who exhibited DAD with an exudative phase and died on the 6th day after onset. However, no signal was detected in the other two cases of DAD with a proliferative phase. These patients died on day 16 and day 17 after onset, respectively. H5N1 virus antigens were detected predominantly in epithelial cells in terminal bronchioles and in alveoli, i.e., type I and type II alveolar pneumocytes, and in alveolar macrophages. -
PBX3 and MEIS1 Cooperate in Hematopoietic Cells to Drive Acute
Published OnlineFirst January 8, 2016; DOI: 10.1158/0008-5472.CAN-15-1566 Cancer Molecular and Cellular Pathobiology Research PBX3 and MEIS1 Cooperate in Hematopoietic Cells to Drive Acute Myeloid Leukemias Characterized by a Core Transcriptome of the MLL-Rearranged Disease Zejuan Li1, Ping Chen1, Rui Su2, Chao Hu1,2,3, Yuanyuan Li1, Abdel G. Elkahloun4, Zhixiang Zuo1,2, Sandeep Gurbuxani5, Stephen Arnovitz1, Hengyou Weng1,2, Yungui Wang1,2,3, Shenglai Li1, Hao Huang1, Mary Beth Neilly1, Gang Greg Wang6, Xi Jiang1,2, Paul P. Liu4, Jie Jin3, and Jianjun Chen1,2 Abstract Overexpression of HOXA/MEIS1/PBX3 homeobox genes is sion profiling of hematopoietic cells demonstrated that PBX3/ the hallmark of mixed lineage leukemia (MLL)-rearranged MEIS1 overexpression, but not HOXA9/MEIS1, HOXA9/PBX3, acute myeloid leukemia (AML). HOXA9 and MEIS1 are con- or HOXA9 overexpression, recapitulated the MLL-fusion–medi- sideredtobethemostcriticaltargetsofMLLfusionsandtheir ated core transcriptome, particularly upregulation of the endog- coexpression rapidly induces AML. MEIS1 and PBX3 are not enous Hoxa genes. Disruption of the binding between MEIS1 individually able to transform cells and were therefore hypoth- andPBX3diminishedPBX3/MEIS1–mediated cell transforma- esized to function as cofactors of HOXA9. However, in this tion and HOX gene upregulation. Collectively, our studies study, we demonstrate that coexpression of PBX3 and MEIS1 strongly implicate the PBX3/MEIS1 interaction as a driver of (PBX3/MEIS1), without ectopic expression of a HOX gene, is cell transformation and leukemogenesis, and suggest that this sufficient for transformation of normal mouse hematopoietic axis may play a critical role in the regulation of the core stem/progenitor cells in vitro.Moreover,PBX3/MEIS1 overex- transcriptional programs activated in MLL-rearranged and pression also caused AML in vivo, with a leukemic latency HOX-overexpressing AML. -
Pediatric Ambulatory Community Acquired Pneumonia (CAP)
ANMC Pediatric (≥3mo) Ambulatory Community Acquired Pneumonia (CAP) Treatment Guideline Criteria for Respiratory Distress Criteria For Outpatient Management Testing/Imaging for Outpatient Management Tachypnea, in breaths/min: Mild CAP: no signs of respiratory distress Vital Signs: Standard VS and Pulse Oximetry Age 0-2mo: >60 Able to tolerate PO Labs: No routine labs indicated Age 2-12mo: >50 No concerns for pathogen with increased virulence Influenza PCR during influenza season Age 1-5yo: >40 (ex. CA-MRSA) Blood cultures if not fully immunized OR fails to Age >5yo: >20 Family able to carefully observe child at home, comply improve/worsens after initiation of antibiotics Dyspnea with therapy plan, and attend follow up appointments Urinary antigen detection testing is not Retractions recommended in children; false-positive tests are common. Grunting If patient does not meet outpatient management criteria Radiography: No routine CXR indicated Nasal flaring refer to inpatient pneumonia guideline for initial workup Apnea and testing. AP and lateral CXR if fails initial antibiotic therapy Altered mental status AP and lateral CXR 4-6 weeks after diagnosis if Pulse oximetry <90% on room air recurrent pneumonia involving the same lobe Treatment Selection Suspected Viral Pneumonia Most Common Pathogens: Influenza A & B, Adenovirus, Respiratory Syncytial Virus, Parainfluenza No antimicrobial therapy is necessary. Most common in <5yo If influenza positive, see influenza guidelines for treatment algorithm. Suspected Bacterial -
Characterisation and Outcomes of ARDS Secondary to Pneumonia in Patients with and Without SARS-Cov-2: a Single-Centre Experience
BMJ Open Resp Res: first published as 10.1136/bmjresp-2020-000731 on 30 November 2020. Downloaded from Critical care Characterisation and outcomes of ARDS secondary to pneumonia in patients with and without SARS- CoV-2: a single- centre experience Rahul Y Mahida ,1 Minesh Chotalia,1,2 Joseph Alderman,1,2 Chhaya Patel,3 Amber Hayden,4 Ruchi Desai,4 Emily Beesley,4 Louise E Crowley,1 Marina Soltan,1 Mansoor Bangash,1,2 Dhruv Parekh ,1,2 Jaimin Patel,1,2 David R Thickett1 To cite: Mahida RY, ABSTRACT Key messages Chotalia M, Alderman J, Introduction Acute respiratory distress syndrome et al. Characterisation and (ARDS) is the major cause of mortality in patients with This is the first UK study comparing the clinical outcomes of ARDS secondary SARS- CoV-2 pneumonia. It appears that development of ► characteristics and outcomes of acute respiratory to pneumonia in patients with ‘cytokine storm’ in patients with SARS- CoV-2 pneumonia and without SARS- CoV-2: distress syndrome (ARDS) secondary to pneumonia precipitates progression to ARDS. However, severity a single- centre experience. in patients with and without SARS- CoV-2. scores on admission do not predict severity or mortality BMJ Open Resp Res in patients with SARS- CoV-2 pneumonia. Our objective ► Are patients with SARS- CoV-2 ARDS clinically dis- 2020;7:e000731. doi:10.1136/ tinct to other patients with ARDS, therefore, requiring bmjresp-2020-000731 was to determine whether patients with SARS- CoV-2 ARDS are clinically distinct, therefore requiring alternative alternative management strategies? ► Additional material is management strategies, compared with other patients ► While the clinical syndromes of ARDS secondary to published online only. -
Lung Histopathological Findings in Fatal Pandemic Influenza a (H1N1)
Med Intensiva. 2012;36(1):24---31 www.elsevier.es/medintensiva ORIGINAL Lung histopathological findings in fatal pandemic influenza A (H1N1) a,b a,b b,c d a,b N. Nin , C. Sánchez-Rodríguez , L.S. Ver , P. Cardinal , A. Ferruelo , e d f g h a,b L. Soto , A. Deicas , N. Campos , O. Rocha , D.H. Ceraso , M. El-Assar , b,c a,b a,b a,b,∗ J. Ortín , P. Fernández-Segoviano , A. Esteban , J.A. Lorente a Hospital Universitario de Getafe, Madrid, Spain b CIBER de Enfermedades Respiratorias, Instituto de Salud Carlos III, Madrid, Spain c Centro Nacional de Biotecnología, CSIC, Madrid, Spain d Sanatorio Casmu, Montevideo, Uruguay e Instituto Nacional de Tórax, Santiago de Chile, Chile f Hospital Regional de Salto, Salto, Uruguay g Sanatorio GREMEDA, Artigas, Uruguay h Hospital Juan A. Fernández, Buenos Aires, Argentina Received 18 July 2011; accepted 19 October 2011 Available online 10 December 2011 KEYWORDS Abstract H1N1; Objective: To describe the lung pathological changes in influenza A (H1N1) viral pneumonia. ARDS; We studied morphological changes, nitro-oxidative stress and the presence of viral proteins in lung tissue. Lung pathology; Mechanical Methods and patients: Light microscopy was used to examine lung tissue from 6 fatal cases ventilation of pandemic influenza A (H1N1) viral pneumonia. Fluorescence for oxidized dihydroethy- dium, nitrotyrosine, inducible NO synthase (NOS2) and human influenza A nucleoprotein (NP) (for analysis under confocal microscopy) was also studied in lung tissue specimens. Results: Age ranged from 15 to 50 years. Three patients were women, and 5 had preexisting medical conditions.