Publications by authors named "Genevieve M Crane"

34 Publications

Educational Case: Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies and Potential Role of the Spleen.

Acad Pathol 2021 Jan-Dec;8:23742895211001312. Epub 2021 Mar 30.

Robert J. Tomsich Department of Pathology and Laboratory Medicine, Cleveland Clinic Foundation, OH, USA.

http://journals.sagepub.com/doi/10.1177/2374289517715040..
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http://dx.doi.org/10.1177/23742895211001312DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8013907PMC
March 2021

The role of EBV in hematolymphoid proliferations: Emerging concepts relevant to diagnosis and treatment.

Histopathology 2021 Apr 7. Epub 2021 Apr 7.

Robert J. Tomsich Pathology and Laboratory Medicine Institute, Department of Laboratory Medicine, Cleveland Clinic, Cleveland, OH, USA.

Epstein-Barr virus (EBV) is a ubiquitous gammaherpesvirus with >90% of the adult population worldwide harboring latent infection. A small subset of those infected develop EBV-associated neoplasms including a range of lymphoproliferative disorders (LPD). The diagnostic distinction of these entities appears increasingly relevant as our understanding of EBV-host interactions and mechanisms of EBV-driven lymphomagenesis improves. EBV may lower the mutational threshold for malignant transformation, create potential vulnerabilities related to viral alteration of cell metabolism and allow for improved immune targeting. However, these tumors may escape immune surveillance by affecting their immune microenvironment, limiting viral gene expression or potential loss of the viral episome. Methods to manipulate the latency state of the virus to enhance immunogenicity are emerging as well as the potential to detect so-called "hit and run" cases where EBV has been lost. Finally, measurement of EBV DNA remains an important biomarker for screening and monitoring of LPD. Methods to distinguish EBV DNA derived from virions during lytic activation from latent, methylated EBV DNA present in EBV-associated neoplasms may broaden the utility of this testing, particularly in patients with compromised immune function. We will highlight some of these emerging areas relevant to the diagnosis and treatment of EBV-associated LPD with potential applicability to other EBV-associated neoplasms.
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http://dx.doi.org/10.1111/his.14379DOI Listing
April 2021

The Network That Never Sleeps.

Lab Med 2021 Mar 15. Epub 2021 Mar 15.

Department of Pathology and Laboratory Medicine, Loyola University Health System, Maywood, Illinois.

This review describes how Twitter is currently used by laboratory professionals for education, research, and networking. This platform has a global audience. It enables users to post information publicly, easily, rapidly, and free of charge. The absence of hierarchies enables interactions that may not be feasible offline. Laboratory professionals teach thousands of people using text, images, polls, and videos. Academic discussion flourishes without paywalls. Published research is shared faster than ever before, articles are discussed in online journal clubs, and research collaborations are facilitated. Pathologists network globally and make new friends within and beyond their specialty. Pathology departments and residency programs showcase trainees and faculty and celebrate graduations. As users in one time zone go to bed, others who are just waking up begin to read and tweet, creating a 24/7/365 live global online conference. We encourage others to plug into the power of Twitter, the network that never sleeps.
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http://dx.doi.org/10.1093/labmed/lmaa113DOI Listing
March 2021

A mechanosensitive peri-arteriolar niche for osteogenesis and lymphopoiesis.

Nature 2021 Mar 24;591(7850):438-444. Epub 2021 Feb 24.

Children's Research Institute and the Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Stromal cells in adult bone marrow that express leptin receptor (LEPR) are a critical source of growth factors, including stem cell factor (SCF), for the maintenance of haematopoietic stem cells and early restricted progenitors. LEPR cells are heterogeneous, including skeletal stem cells and osteogenic and adipogenic progenitors, although few markers have been available to distinguish these subsets or to compare their functions. Here we show that expression of an osteogenic growth factor, osteolectin, distinguishes peri-arteriolar LEPR cells poised to undergo osteogenesis from peri-sinusoidal LEPR cells poised to undergo adipogenesis (but retaining osteogenic potential). Peri-arteriolar LEPRosteolectin cells are rapidly dividing, short-lived osteogenic progenitors that increase in number after fracture and are depleted during ageing. Deletion of Scf from adult osteolectin cells did not affect the maintenance of haematopoietic stem cells or most restricted progenitors but depleted common lymphoid progenitors, impairing lymphopoiesis, bacterial clearance, and survival after acute bacterial infection. Peri-arteriolar osteolectin cell maintenance required mechanical stimulation. Voluntary running increased, whereas hindlimb unloading decreased, the frequencies of peri-arteriolar osteolectin cells and common lymphoid progenitors. Deletion of the mechanosensitive ion channel PIEZO1 from osteolectin cells depleted osteolectin cells and common lymphoid progenitors. These results show that a peri-arteriolar niche for osteogenesis and lymphopoiesis in bone marrow is maintained by mechanical stimulation and depleted during ageing.
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http://dx.doi.org/10.1038/s41586-021-03298-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7979521PMC
March 2021

Next-Generation Scholarship: Rebranding Hematopathology Using Twitter: The MD Anderson Experience.

Mod Pathol 2021 05 20;34(5):854-861. Epub 2020 Nov 20.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Hematopathologists are witnessing very exciting times, as a new era of unsurpassed technological advances is unfolding exponentially, enhancing our understanding of diseases at the genomic and molecular levels. In the evolving field of precision medicine, our contributions as hematopathologists to medical practice are of paramount importance. Social media platforms such as Twitter have helped facilitate and enrich our professional  interactions and collaborations with others in our field and in other medical disciplines leading to a more holistic approach to patient care. These platforms also have created a novel means for instantaneous dissemination of new findings and recent publications, and are proving to be increasingly useful tools that can be harnessed to expand our knowledge and amplify our presence in the medical community. In this Editorial, we share our experience as hematopathologists with Twitter, and how we leveraged this platform to boost scholarly activities within and beyond our subspecialty, and as a powerful medium for worldwide dissemination of educational material and to promote our remote teaching activities during the COVID-19 pandemic.
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http://dx.doi.org/10.1038/s41379-020-00715-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7678590PMC
May 2021

Social Media for Hematopathologists: Medical Practice Reinvented-#Hemepath.

Curr Hematol Malig Rep 2020 10 30;15(5):383-390. Epub 2020 Oct 30.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.

Purpose Of Review: Social media engagement by medical professionals with varied background subspecialties has steadily gained popularity in recent years. As a heavily visual discipline, pathology has been able to leverage social media platforms for trainee education, curbside and official consultations, interdisciplinary communication, and interactions among medical professionals and patient education. The pathology community has been at the forefront of using social media as an educational forum, and the hematopathology community has emerged as one of the strongest and most influential presences on these online platforms. In this review, we perform an in-depth analysis of various Twitter metrics to demonstrate key trends in the usage of social media as it pertains to hematopathology using the hashtag #Hemepath and we describe specific details on how hematopathologists have managed to take advantage of Twitter in furthering our mission of advancing medical education and disseminating knowledge using these innovative virtual educational experiences.

Recent Findings: The hematopathology community has a great degree of enthusiasm among residents, fellows, and faculty in sharing educational material using case-based examples, participating in group-based online activities, introducing new publications by article authors or readership, and disseminating educational "pearls" from medical conferences, using hashtags and digital images that otherwise would not be readily available to many around the globe. This practice is helping reshape the structure of our field and is providing opportunities to optimize the educational experience by enhancing the instant exposure to cutting-edge information and expert opinions, among other valuable features. The hematopathology community has leveraged social media platforms for disseminating educational material and strengthening interdisciplinary interactions and is a "poster child" for a medical subspecialty that has thrived and flourished by more broadly adopting virtual educational platforms. We hope that this review will provide details on how social media platforms can be used by others in the medical field to achieve similar goals.
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http://dx.doi.org/10.1007/s11899-020-00600-6DOI Listing
October 2020

Spleen: Development, anatomy and reactive lymphoid proliferations.

Semin Diagn Pathol 2021 Mar 16;38(2):112-124. Epub 2020 Jun 16.

Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA. Electronic address:

The unique architecture of the spleen enables it to play a key role in the interactions between the circulatory, reticuloendothelial and immune systems. Response to circulating antigens in the setting of infection, autoimmune disease or other conditions may result in a range of benign lymphoid proliferations. Moreover, patients with underlying immune deficiency may also show abnormal lymphoid proliferations within the spleen. This review will highlight the histologic, immunophenotypic and clinical features of reactive lymphoid proliferations to aid in their recognition and provide a context for understanding their development in relation to normal splenic structure and function.
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http://dx.doi.org/10.1053/j.semdp.2020.06.003DOI Listing
March 2021

Immunodeficiency-Related Lymphoid Proliferations: New Insights With Relevance to Practice.

Curr Hematol Malig Rep 2020 08;15(4):360-371

Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA.

Purpose Of Review: Our understanding of risk factors and mechanisms underlying immunosuppression-related lymphoproliferative disorders continues to evolve. An increasing number of patients are living with altered immune status due to HIV, solid organ or hematopoietic stem cell transplant, treatment of autoimmune disease, or advanced age. This review covers advances in understanding, emerging trends, and revisions to diagnostic guidelines.

Recent Findings: The tumor microenvironment, including interactions between the host immune system and tumor cells, is of increasing interest in the setting of immunosuppression. While some forms of lymphoproliferative disease are associated with unique risk factors, common mechanisms are also emerging. Indolent forms, such as Epstein-Barr virus positive mucocutaneous ulcer, are important to recognize. As methods to modulate the immune system evolve, more data are needed to understand and minimize lymphoproliferative disease risk. A better understanding of individual risk factors and common mechanisms underlying immunosuppression-related lymphoproliferations will ultimately enable improved prevention and treatment of these disorders.
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http://dx.doi.org/10.1007/s11899-020-00594-1DOI Listing
August 2020

Ofatumumab for post-transplant lymphoproliferative disorder.

Hematol Oncol Stem Cell Ther 2020 May 11. Epub 2020 May 11.

Department of Medical Oncology, Division of Hematological Malignancies, Sidney Kimmel Cancer Center, Philadelphia, PA, USA.

Posttransplant lymphoproliferative disorder (PTLD) includes a range of abnormal lymphoid proliferation following solid organ or allogeneic hematopoietic stem cell transplantation (HSCT), often associated with Epstein-Barr virus (EBV) infection. Treatment generally incudes rituximab, a chimeric monoclonal antibody directed against CD20. Here we present a 56-year-old woman with EBV-associated PTLD following allogeneic HSCT who was intolerant of rituximab. The patient was instead treated with ofatumumab, a fully human monoclonal antibody directed against CD20, with significant response in EBV viral load and lymphadenopathy. Ofatumumab could represent an important treatment option for patients unable to tolerate rituximab.
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http://dx.doi.org/10.1016/j.hemonc.2020.04.004DOI Listing
May 2020

Infantile Myelofibrosis and Myeloproliferation with CDC42 Dysfunction.

J Clin Immunol 2020 05 17;40(4):554-566. Epub 2020 Apr 17.

Division of Hematology/Oncology, Boston Children's Hospital and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

Studies of genetic blood disorders have advanced our understanding of the intrinsic regulation of hematopoiesis. However, such genetic studies have only yielded limited insights into how interactions between hematopoietic cells and their microenvironment are regulated. Here, we describe two affected siblings with infantile myelofibrosis and myeloproliferation that share a common de novo mutation in the Rho GTPase CDC42 (Chr1:22417990:C>T, p.R186C) due to paternal germline mosaicism. Functional studies using human cells and flies demonstrate that this CDC42 mutant has altered activity and thereby disrupts interactions between hematopoietic progenitors and key tissue microenvironmental factors. These findings suggest that further investigation of this and other related disorders may provide insights into how hematopoietic cell-microenvironment interactions play a role in human health and can be disrupted in disease. In addition, we suggest that deregulation of CDC42 may underlie more common blood disorders, such as primary myelofibrosis.
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http://dx.doi.org/10.1007/s10875-020-00778-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253386PMC
May 2020

Peripheral eosinophilia in primary immunodeficiencies of actin dysregulation: A case series of Wiskott-Aldrich syndrome, CARMIL2 and DOCK8 deficiency and review of the literature.

Ann Diagn Pathol 2019 Dec 5;43:151413. Epub 2019 Oct 5.

Department of Pathology & Laboratory Medicine, New York-Presbyterian Hospital, Weill Cornell Medical College, 520 East 70th St., New York, NY 10021, United States of America.

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http://dx.doi.org/10.1016/j.anndiagpath.2019.151413DOI Listing
December 2019

Integrin alpha11 is an Osteolectin receptor and is required for the maintenance of adult skeletal bone mass.

Elife 2019 01 11;8. Epub 2019 Jan 11.

Children's Research Institute, University of Texas Southwestern Medical Center, Dallas, United States.

We previously discovered a new osteogenic growth factor that is required to maintain adult skeletal bone mass, Osteolectin/Clec11a. Osteolectin acts on Leptin Receptor (LepR) skeletal stem cells and other osteogenic progenitors in bone marrow to promote their differentiation into osteoblasts. Here we identify a receptor for Osteolectin, integrin α11, which is expressed by LepR cells and osteoblasts. α11β1 integrin binds Osteolectin with nanomolar affinity and is required for the osteogenic response to Osteolectin. Deletion of (which encodes α11) from mouse and human bone marrow stromal cells impaired osteogenic differentiation and blocked their response to Osteolectin. Like deficient mice, mice appeared grossly normal but exhibited reduced osteogenesis and accelerated bone loss during adulthood. Osteolectin binding to α11β1 promoted Wnt pathway activation, which was necessary for the osteogenic response to Osteolectin. This reveals a new mechanism for maintenance of adult bone mass: Wnt pathway activation by Osteolectin/α11β1 signaling.
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http://dx.doi.org/10.7554/eLife.42274DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349404PMC
January 2019

Social Media in Pathology: Continuing a Tradition of Dialogue and Education.

Arch Pathol Lab Med 2018 08;142(8):889-890

7   Departments of Pathology and Dermatology, University of Arkansas for Medical Sciences, Little Rock.

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http://dx.doi.org/10.5858/arpa.2018-0057-LEDOI Listing
August 2018

Expanding the Spectrum of EBV-positive Marginal Zone Lymphomas: A Lesion Associated With Diverse Immunodeficiency Settings.

Am J Surg Pathol 2018 10;42(10):1306-1316

Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda.

Traditionally low-grade B-cell lymphomas have been excluded from the category of monomorphic posttransplant lymphoproliferative disorders. However, recent reports identified Epstein-Barr virus-positive (EBV) extranodal marginal zone lymphomas (MZL), almost exclusively seen in the posttransplant setting. Some reported cases responded to reduced immunosuppression, suggesting that they should be considered as a form of posttransplant lymphoproliferative disorders. We identified 10 cases of EBV MZL, 9 in extranodal sites and 1 presenting in lymph node. Two cases arose following solid organ transplantation, but other settings included iatrogenic immunosuppression for rheumatoid arthritis (2); prior chemotherapy (2); congenital immune deficiency (1); and increased age (3), as the only potential cause of immune dysfunction. There were 4 males and 6 females; age range 18 to 86. The atypical plasmacytoid and/or monocytoid B cells were positive for EBV in all cases, with either latency I or II in all cases tested. Monotypic light chain expression was shown in all with 6 cases positive for IgG, and 2 for IgM, undetermined in 2. Clonal immunoglobulin gene rearrangement was positive in all cases with successful amplification. MYD88 L265P was wild type in the 6 cases tested. We show that EBV MZLs can arise in a variety of clinical settings, and are most often extranodal. Treatment varied, but most patients had clinically indolent disease with response to reduction of immune suppression, or immunochemotherapy.
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http://dx.doi.org/10.1097/PAS.0000000000001113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6133753PMC
October 2018

A 17-Year-Old Boy With Right Face Palsy, Left Leg Weakness, and Lytic Skull-Bone Lesions.

J Pediatric Infect Dis Soc 2018 Dec;7(4):350-354

Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Human T-cell lymphotropic virus (HTLV), an infection that is endemic in certain parts of Asia, Africa, and South America, has been associated with malignancy and neurological deficits. Here, we describe a pediatric patient with chronic HTLV-I infection who developed complications associated with HTLV-I (ie, adult T-cell leukemia/lymphoma and HTLV-I-associated myelopathy/tropical spastic paraparesis). To our knowledge, this presentation in a child has never been described. The patient underwent a bone marrow transplant and, at the time of this writing, was in remission. This case report highlights the fact that HTLV-related complications, previously expected to occur after decades of infection, also can occur in pediatric patients, particularly those who acquired HTLV-I perinatally.
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http://dx.doi.org/10.1093/jpids/pix101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276027PMC
December 2018

Ascorbate regulates haematopoietic stem cell function and leukaemogenesis.

Nature 2017 09 21;549(7673):476-481. Epub 2017 Aug 21.

Children's Research Institute and the Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA.

Stem-cell fate can be influenced by metabolite levels in culture, but it is not known whether physiological variations in metabolite levels in normal tissues regulate stem-cell function in vivo. Here we describe a metabolomics method for the analysis of rare cell populations isolated directly from tissues and use it to compare mouse haematopoietic stem cells (HSCs) to restricted haematopoietic progenitors. Each haematopoietic cell type had a distinct metabolic signature. Human and mouse HSCs had unusually high levels of ascorbate, which decreased with differentiation. Systemic ascorbate depletion in mice increased HSC frequency and function, in part by reducing the function of Tet2, a dioxygenase tumour suppressor. Ascorbate depletion cooperated with Flt3 internal tandem duplication (Flt3) leukaemic mutations to accelerate leukaemogenesis, through cell-autonomous and possibly non-cell-autonomous mechanisms, in a manner that was reversed by dietary ascorbate. Ascorbate acted cell-autonomously to negatively regulate HSC function and myelopoiesis through Tet2-dependent and Tet2-independent mechanisms. Ascorbate therefore accumulates within HSCs to promote Tet activity in vivo, limiting HSC frequency and suppressing leukaemogenesis.
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http://dx.doi.org/10.1038/nature23876DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5910063PMC
September 2017

Adult haematopoietic stem cell niches.

Nat Rev Immunol 2017 Sep 12;17(9):573-590. Epub 2017 Jun 12.

Children's Research Institute, University of Texas Southwestern Medical Center.

Stem cell niches are specialized microenvironments that promote the maintenance of stem cells and regulate their function. Recent advances have improved our understanding of the niches that maintain adult haematopoietic stem cells (HSCs). These advances include new markers for HSCs and niche cells, systematic analyses of the expression patterns of niche factors, genetic tools for functionally identifying niche cells in vivo, and improved imaging techniques. Together, they have shown that HSC niches are perivascular in the bone marrow and spleen. Endothelial cells and mesenchymal stromal cells secrete factors that promote HSC maintenance in these niches, but other cell types also directly or indirectly regulate HSC niches.
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http://dx.doi.org/10.1038/nri.2017.53DOI Listing
September 2017

Tumor-Infiltrating Macrophages in Post-Transplant, Relapsed Classical Hodgkin Lymphoma Are Donor-Derived.

PLoS One 2016;11(9):e0163559. Epub 2016 Sep 29.

Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland, United States of America.

Tumor-associated inflammatory cells in classical Hodgkin lymphoma (CHL) typically outnumber the neoplastic Hodgkin/Reed-Sternberg (H/RS) cells. The composition of the inflammatory infiltrate, particularly the fraction of macrophages, has been associated with clinical behavior. Emerging work from animal models demonstrates that most tissue macrophages are maintained by a process of self-renewal under physiologic circumstances and certain inflammatory states, but the contribution from circulating monocytes may be increased in some disease states. This raises the question of the source of macrophages involved in human disease, particularly that of CHL. Patients with relapsed CHL following allogeneic bone marrow transplant (BMT) provide a unique opportunity to begin to address this issue. We identified 4 such patients in our archives. Through molecular chimerism and/or XY FISH studies, we demonstrated the DNA content in the post-BMT recurrent CHL was predominantly donor-derived, while the H/RS cells were derived from the patient. Where possible to evaluate, the cellular composition of the inflammatory infiltrate, including the percentage of macrophages, was similar to that of the original tumor. Our findings suggest that the H/RS cells themselves define the inflammatory environment. In addition, our results demonstrate that tumor-associated macrophages in CHL are predominantly derived from circulating monocytes rather than resident tissue macrophages. Given the association between tumor microenvironment and disease progression, a better understanding of macrophage recruitment to CHL may open new strategies for therapeutic intervention.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0163559PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5042490PMC
September 2016

An Uncommon Cause of Obstructive Jaundice: An Infrequent Neoplasm.

Am J Med 2017 Feb 13;130(2):e43-e45. Epub 2016 Sep 13.

Thayer Firm, Osler Medical Service, Johns Hopkins Hospital, Baltimore, Md; Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md. Electronic address:

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http://dx.doi.org/10.1016/j.amjmed.2016.08.027DOI Listing
February 2017

Pathology Image-Sharing on Social Media: Recommendations for Protecting Privacy While Motivating Education.

AMA J Ethics 2016 Aug 1;18(8):817-25. Epub 2016 Aug 1.

Assistant professor of pathology and dermatology at the University of Arkansas for Medical Sciences in Little Rock, Arkansas, and the chair and founding member of social media subcommittees for both the United States and Canadian Academy of Pathology and the American Society of Dermatopathology and is a deputy editor-in-chief of the Archives of Pathology & Laboratory Medicine.

There is a rising interest in the use of social media by pathologists. However, the use of pathology images on social media has been debated, particularly gross examination, autopsy, and dermatologic condition photographs. The immediacy of the interactions, increased interest from patients and patient groups, and fewer barriers to public discussion raise additional considerations to ensure patient privacy is protected. Yet these very features all add to the power of social media for educating other physicians and the nonmedical public about disease and for creating better understanding of the important role of pathologists in patient care. The professional and societal benefits are overwhelmingly positive, and we believe the potential for harm is minimal provided common sense and routine patient privacy principles are utilized. We lay out ethical and practical guidelines for pathologists who use social media professionally.
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http://dx.doi.org/10.1001/journalofethics.2016.18.8.stas1-1608DOI Listing
August 2016

Double Jeopardy: Autoimmune Myelofibrosis with Pyoderma Gangrenosum.

Am J Med 2017 Jan 9;130(1):e1-e4. Epub 2016 Jul 9.

Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md. Electronic address:

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http://dx.doi.org/10.1016/j.amjmed.2016.06.016DOI Listing
January 2017

Epidural spinal compression as an initial presentation of Hodgkin lymphoma.

J Clin Neurosci 2016 Apr 23;26:166-8. Epub 2015 Dec 23.

Department of Neurosurgery, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Meyer 5-185, Baltimore, MD 21287, USA.

Classical Hodgkin lymphoma (CHL) commonly arises in lymph nodes and initial presentation with extranodal disease is rare. We report a patient who presented with progressively worsening back pain, lower extremity weakness and numbness concerning for a myelopathic process of uncertain etiology. MRI revealed an epidural soft tissue mass with cord displacement, for which she underwent resection. Histological analysis of the surgical specimen demonstrated CHL. Further investigation revealed an anterior mediastinal mass, consistent with spread from a more typical location.
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http://dx.doi.org/10.1016/j.jocn.2015.10.026DOI Listing
April 2016

Epithelial-myoepithelial carcinoma metastasis to the thoracic spine.

J Clin Neurosci 2016 Feb 21;24:143-6. Epub 2015 Oct 21.

Department of Neurosurgery, The Johns Hopkins University, 600 North Wolfe Street, Meyer 5-185, Baltimore, MD 21287, USA. Electronic address:

Epithelial-myoepithelial carcinoma (EMC) is a very rare salivary gland malignancy accounting for less than 1% of salivary gland tumors, and classically arises from the parotid gland in females. Spinal cord compression caused by EMC metastasized from the parotid gland has only been described once in the literature to our knowledge. We report the first case of a patient with parotid EMC spinal metastasis undergoing a gross total resection with instrumented fusion. This case illustrates that an en bloc resection with a planned transgression through the spinal canal may be a reasonable option for EMC metastasized to the spine.
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http://dx.doi.org/10.1016/j.jocn.2015.07.014DOI Listing
February 2016

Hematolymphoid lesions of the sinonasal tract.

Semin Diagn Pathol 2016 Mar 9;33(2):71-80. Epub 2015 Sep 9.

Department of Pathology, The Johns Hopkins Medical Institutions, Weinberg 2242, 401 N Broadway, Baltimore, Maryland 21231-2410. Electronic address:

Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent T-lymphoblastic proliferations; and Rosai-Dorfman disease. Differentiating aggressive lymphomas from non-hematopoietic neoplasms such as poorly differentiated squamous cell carcinoma, olfactory neuroblastoma, or sinonasal undifferentiated carcinoma may pose diagnostic challenges. In addition, the necrosis, vascular damage, and inflammatory infiltrates that are associated with some hematolymphoid disorders can result in misdiagnosis as infectious, autoimmune, or inflammatory conditions. Here, we review hematolymphoid disorders involving the sinonasal tract including their key clinical and histopathologic features.
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http://dx.doi.org/10.1053/j.semdp.2015.09.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4752909PMC
March 2016

Primary CNS lymphoproliferative disease, mycophenolate and calcineurin inhibitor usage.

Oncotarget 2015 Oct;6(32):33849-66

Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.

Immunosuppression for solid organ transplantation increases lymphoproliferative disease risk. While central nervous system (CNS) involvement is more rare, we noticed an increase in primary CNS (PCNS) disease. To investigate a potential association with the immunosuppressive regimen we identified all post-transplant lymphoproliferative disease (PTLD) cases diagnosed over a 28-year period at our institution (174 total, 29 PCNS) and all similar cases recorded in a United Network for Organ Sharing-Organ Procurement and Transplant Network (UNOS-OPTN) datafile. While no PCNS cases were diagnosed at our institution between 1986 and 1997, they comprised 37% of PTLD cases diagnosed from 2011-2014. PCNS disease was more often associated with renal vs. other organ transplant, Epstein-Barr virus, large B-cell morphology and mycophenolate mofetil (MMF) as compared to PTLD that did not involve the CNS. Calcineurin inhibitors were protective against PCNS disease when given alone or in combination with MMF. A multivariate analysis of a larger UNOS-OPTN dataset confirmed these findings, where both MMF and lack of calcineurin inhibitor usage were independently associated with risk for development of PCNS PTLD. These findings have significant implications for the transplant community, particularly given the introduction of new regimens lacking calcineurin inhibitors. Further investigation into these associations is warranted.
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http://dx.doi.org/10.18632/oncotarget.5292DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4741807PMC
October 2015

Cytomorphologic and Flow Cytometric Analysis of Cerebrospinal Fluid with T-Cell Lymphoma Involvement: A Retrospective Study of Rare Cases.

Acta Cytol 2015 29;59(4):325-31. Epub 2015 Aug 29.

Divisions of Cytopathology and Hematopathology, Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Md., USA.

Objective: To compare the efficacy of conventional cytomorphologic analysis and flow cytometry (FC) in the diagnosis of T-cell lymphoma in the cerebrospinal fluid (CSF).

Study Design: We performed a retrospective review of CSF samples from 2002 to 2012 that showed involvement of a T-cell lymphoma, either by cytomorphologic analysis and/or FC. Patients' demographics, clinical history and follow-up were assessed.

Results: Thirty-nine CSF samples were identified from 9 patients. A definitive diagnosis of T-cell lymphoma involvement was made by cytomorphologic analysis and FC in 6 (15.4%) and 39 (100.0%) specimens, respectively. In specimens with definitive cytopathologic diagnoses, the cytomorphologic features included increased cellularity, a monotonous lymphoid population and large, atypical lymphoid cells. Considering cytomorphologic features only, 9 specimens demonstrated atypical lymphocytes not fulfilling the criteria for malignancy, and 24 specimens were negative for malignancy.

Conclusions: CSF with T-cell lymphoma involvement may yield paucicellular or acellular specimens depending on the volume of the CSF, the time interval between specimen collection and specimen processing and the application of preservative to CSF. The rate of detection of T-cell lymphoma in the CSF by FC is unequivocally higher than by cytomorphologic analysis. Careful attention to clinical history is crucial, as FC testing may be tailored to evaluate for T-cell lymphoproliferative disorders in limited samples.
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http://dx.doi.org/10.1159/000439106DOI Listing
January 2016

Langerhans cell histiocytosis of the cervical spine.

Spine J 2016 Jan 11;16(1):e11-2. Epub 2015 Aug 11.

Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.

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http://dx.doi.org/10.1016/j.spinee.2015.08.010DOI Listing
January 2016

Metastatic extrapulmonary small cell carcinoma to the cerebellopontine angle: a case report and review of the literature.

Case Rep Oncol Med 2015 25;2015:847058. Epub 2015 Feb 25.

Department of Neurosurgery, The Johns Hopkins Hospital, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.

Extrapulmonary small cell carcinomas (EPSCC) are rare malignancies with poor patient prognoses. We present the case of a 63-year-old male who underwent surgical resection of a poorly differentiated small cell carcinoma, likely from a small intestinal primary tumor that metastasized to the cerebellopontine angle (CPA). A 63-year-old male presented with mild left facial paralysis, hearing loss, and balance instability. MRI revealed a 15 mm mass in the left CPA involving the internal auditory canal consistent with a vestibular schwannoma. Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm. The patient underwent a suboccipital craniectomy and the mass was grossly different visually and in consistency from a standard vestibular schwannoma. The final pathology revealed a poorly differentiated small cell carcinoma. Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease. The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms. Surgical resection and radiotherapy are potential treatment options to improve survival in patients diagnosed with NET brain metastases. We present the first documented case of skull base metastasis of a poorly differentiated small cell carcinoma involving the CPA.
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http://dx.doi.org/10.1155/2015/847058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4355812PMC
March 2015

Myocardial calcifications: pathophysiology, etiologies, differential diagnoses, and imaging findings.

J Cardiovasc Comput Tomogr 2015 Jan-Feb;9(1):58-67. Epub 2014 Oct 22.

Department of Radiology, The Johns Hopkins Hospital, Baltimore, MD, 601 N. Caroline St., Room 4214, Baltimore, MD 21287, USA. Electronic address:

Myocardial calcifications are not uncommonly encountered by the cardiac imager and may have a range of imaging appearances, from focal calcific deposits to diffuse myocardial involvement. A number of pathological processes can both cause and result from myocardial calcification; therefore, accurate identification and characterization are important. This pictorial essay will review the mechanisms, etiologies, imaging features, and differential diagnoses of myocardial calcification with imaging examples.
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http://dx.doi.org/10.1016/j.jcct.2014.10.004DOI Listing
November 2015