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The net result of STAT3 inhibition by dominant negative mutations is skewing toward TH2 differentiation and the resultant clinical triad.46 Case 284 described a 7-year-old girl with a STAT3 mutation who had the classic features of eczema, recurrent skin infections, a markedly elevated IgE level, and eosinophilia. Blood 2022; 140 (11): 11931199. Reactivation of herpesviruses, especially human herpesvirus 6, has been implicated in DRESS pathogenesis.10. Idiopathic HE/HES, by definition, lack features of a clonal abnormality. The workshop received a single unusual case of tryptase-positive AML with inv(16)(p13.1q22) (case 43; Image 12C and Image 12D). T Both were male patients, 63 and 71 years of age, respectively, and both had pancytopenia, absolute eosinophilia, and circulating blasts. This case emphasizes the importance of a highly sensitive flow cytometric analysis in suspected cases with marked eosinophilia but no conspicuous blasts in PB. Case 288 was classified as CMML-1. 2003 - present. Society for Hematopathology ND Clinical manifestations are highly variable yet frequently include severe infections (including viral, disseminated nontuberculous mycobacterial and invasive fungal infections), pulmonary dysfunction, sensorineural hearing loss, multiple cutaneous and genital warts, panniculitis, venous thrombosis, lymphedema, and hypothyroidism.79 Patients have a strong propensity to develop MDS or AML, which may in some cases be the initial manifestation of the disease. CEL, NOS is a clinically aggressive MPN with eosinophilia as the dominant feature. The laboratory also performs a wide range of cytochemical stains. The BM biopsy specimens in IHES were normocellular (predominantly) to slightly hypercellular BM with intact trilineage hematopoiesis and various percentages of increased eosinophils. Society for Hematopathology | LinkedIn Scientific hematopathology societies, in collaboration with hematology societies, should be primarily responsible for establishing a standing International Working Group, which would in turn collaborate with the World Health Organization (WHO)/International Agency for Research on Cancer (IARC) to realize and disseminate the classification. Patients with DOCK8 deficiency are at high risk for developing malignancies, including B- and T-cell lymphomas and squamous cell carcinoma.58 Case 256 highlighted this cancer predisposition, as the patient developed an ALK1-negative anaplastic large cell lymphoma 4 years after her initial diagnosis. Box 557 She had a history of L-tryptophan ingestion, and this eosinophilia-myalgia syndrome is now known to be induced by ingestion of this supplement, specifically toxins formed as contaminants during its synthesis.9, Cases of Idiopathic Hypereosinophilic Syndrome and Reactive and Secondary Eosinophilia. Boscaro E, Urbino I, Catania FM, Arrigo G, Secreto C, Olivi M, D'Ardia S, Frairia C, Giai V, Freilone R, Ferrero D, Audisio E, Cerrano M. Cancers (Basel). The meeting venue will be in the Houston Theater District (Downtown). Finally, three of the cases submitted for the conference (cases 74, 12, and 140) were examples of eosinophilia associated with myeloid neoplasms that arose from a germline cancer predisposition, including two affecting adults. The clinical and laboratory features of these cases (56 and 65) are summarized in Table 9. International Consensus Classification of Myeloid Neoplasms and Acute SH Members logging in for the first time should use the "Forget your Password" option to retrieve your temporary password via email. SA Corresponding author: Katalin Kelemen, MD, PhD; Search for other works by this author on: Department of Pathology, Karolinska University Hospital and Institute, Department of Pathology, Texas Tech University Health Sciences Center, Department of Laboratory Medicine, Cleveland Clinic, Department of Pathology and Laboratory Medicine, Childrens Hospital of Philadelphia, Department of Pathology, University of Utah School of Medicine, Institute of Pathology, University of Munich (LMU), Institute of Pathology and Neuropathology, Eberhard Karls University of Tbingen and Comprehensive Cancer Center, Tbingen University Hospital, Department of Hematopathology, University of Texas MD Anderson Cancer Center, British Committee for Standards in Haematology, Guideline for the investigation and management of eosinophilia, The diagnostic work-up of hypereosinophilia, Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes, Pathogenesis, classification, and therapy of eosinophilia and eosinophil disorders, The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia, Association of the eosinophilia-myalgia syndrome with the ingestion of tryptophan, Drug reaction with eosinophilia and systemic symptoms (DRESS): an interplay among drugs, viruses, and immune system, Eosinophilia in rheumatologic/vascular disorders. Malcovati In a study of patients with MPNs, genomic characterization allowed the identification of distinct genomic subgroups, which provides a classification based on driver mutations.31 Subsequently, the combined use of clinical and genomic data enabled the creation of prognostic models capable of generating personally tailored predictions of clinical outcomes.31 Genomic profiling has led to further dissection of DLBCL, with recently proposed discrete recognizable entities associated with variable prognoses.32,33 The algorithm for the diagnostic work-up of large B-cell lymphoma within the IC classification of mature lymphoid neoplasms integrates morphology, immunophenotyping, clinical features, and genomic analyses. Thus, a universal digital portal (accessed through an internet browser or mobile app) will likely be instrumental for the routine diagnostic workup and management of hematologic neoplasms in the future. Third, genomic profiling can allow the categorization of morphologically defined neoplasms into distinct genomic subgroups. In these cases, often cryptic by conventional karyotype and FISH studies, genetic testing such as RNA sequencing is recommended to exclude the possibility of a specific gene fusion. The International Agency for Research on Cancer (IARC) was created in France in 1965 as a specialized cancer agency of the WHO. Through data sharing, the creation of large comprehensive patient data sets will allow the use of methods of inference, including statistical analyses and machine learning models, aimed at further identifying distinct disease subgroups. , Handa H, Yokohama A, et al. CEL, NOS is distinguished from HES by the presence of a clonal cytogenetic or molecular abnormality or increased blast cells (>2% in the PB or >5% in the BM but <20% blasts in either compartment). Alsultan Ryan S. Robetorye, M.D., Ph.D. - Mayo Clinic SA , Shaver AC, Kovach AE, et al. , Al-Helale M, Alhissi S, et al. Translocations of 5q32 (PDGFRB) or 8p11.23 (FGFR1) are usually accompanied by an abnormal karyotype on cytogenetic evaluation.7 Rarely, PDGFRB rearrangements are cytogenetically cryptic and can be detected by RT-PCR or RNA sequencing analysis.8 The PCM1-JAK2 fusion was recently added to this World Health Organization (WHO) category as a provisional entity. Similar to patients with hyper-IgE syndrome, patients with WAS typically have eczema, are highly susceptible to recurrent infections, and have an increased risk of autoimmunity and malignancies.60 Patients with WAS typically have petechiae due to microthrombocytopenia since platelet production, function, and clearance are altered secondary to biallelic WASP mutations.61,62 Since WASP is on the X chromosome, evaluation for WAS should be undertaken in male infants with eczema or recurrent infections in association with thrombocytopenia.63, Two cases of WAS were submitted to the conference (cases 19 and 138A). National Library of Medicine Email: [emailprotected], 2023 European Association for Haematopathology, Recording: Young EA4HP General Meeting | Monday 12 June 2023, Young EA4HP Members invited to General Meeting on 12 June 2023, 10% discount on the Early Bird registration fee for the 4th International Conference on Lymphocyte Engineering | 12-14 September 2023 Munich, Germany, 2023 SH/EA4HP Workshop from November 9th to 11th, EuroClonality WORKSHOP: Clonality assessment in Pathology | March 4 6 2024, Case Submission Open JW2024 | April 13 14 Hefei, China 2024, 22nd Meeting of the European Association of Haematopathology. The contribution of prognostic factors to socio-demographic inequalities in breast cancer survival in Victoria, Australia. Dear Colleagues and Friends, On behalf of the Society of Hematopathology (SH) and European Association for Hematopathology (EAHP), I would like to invite you to Houston, Texas, to attend the 2023 SH/EAHP Workshop from November 9 to 11. Two of these cases were examples of therapy-related MDS/MPN (TR-MDS/MPN); the third case (110) is a TR-MN that is hard to classify in view of the history of intermittent cytopenias with spontaneous normalization antedating the development of the eosinophilia and basophilia, better left as a TR-MN not further classifiable. bExamples would include but are not limited to eosinophilic gastrointestinal disorders (eg, eosinophilic esophagitis), eosinophilic pulmonary disorders (eg, eosinophilic pneumonia), and eosinophilic dermatologic conditions (broad spectrum). FISH analysis of the PB demonstrated 89% of the leukocytes to contain a PML/RARA fusion. Abbondanzo Additional causes of eosinophilia, including infections and myeloid growth factor administration, should also be considered potential etiologies for the relative eosinophilia. A negative screen for the aforementioned abnormalities should prompt a consideration of the diagnosis of chronic eosinophilic leukemia (CEL), not otherwise specified (NOS), when there is cytogenetic, molecular, and morphologic evidence of a myeloid malignancy that has a predominant eosinophilia but cannot be classified as a specific myeloid neoplasm otherwise. Both cases broaden the differential diagnosis of myeloid neoplasms presenting with eosinophilia to include rare cases of JMML. "Virtual Curriculum" is a series of lectures designed to provide hematopathology educational resources to pathology trainees affected by the COVID-19 pandemic. Posting of new educational offerings will be announced from our . The remaining drug-related cases further illustrate the diversity of drugs that can lead to eosinophilia. Clipboard, Search History, and several other advanced features are temporarily unavailable. Despite the higher frequency of eosinophilic germline disorders within the pediatric population, they still remain quite uncommon and likely account for less than 10% of eosinophilia cases in children.42 Many of the inborn causes of eosinophilia are associated with primary immunodeficiencies that can involve T cells, B cells, natural killer cells, or phagocytic cells on their own or in combination. Both of these cases have been described elsewhere.81,82. We look forward to meeting you in person to sharing knowledge and discussing the latest advances in the diagnosis and classification of T- and NK-cell lymphomas. DA Worldwide representation in this IWG is paramount to ensure that future proposals have applicability in regions where resources may not be equitable. Eosinophilia is commonly reactive. Society for Hematopathology An additional two cases that were submitted to the conference had signs of a primary immunodeficiency, but an underlying genetic abnormality was not identified. However, a single mutation, especially if involving the genes DNMT3A, TET2, and ASXL1 in a morphologically normal BM, most likely represents CHIP, whereas mutations involving TP53, EZH2, SEPBP1, STAT5B, CSF3R, NRAS, KRAS, and more than one mutation, particularly if of higher allele frequency (VAF >10%), in association with an abnormal finding in BM and in the correct clinical context, could be used to provide clonality confirmation in establishing a diagnosis of CEL, NOS.21. Thus, morphologic findings may precede cytogenetic and molecular abnormalities in CEL, NOS and warrant clinical follow-up. DRESS usually manifests 2 to 6 weeks after drug exposure, which was nicely illustrated by cases 262, 160, and 229. Molecular Genetic Pathology Fellowship - University of New Mexico MA The laboratory evaluation should begin with a routine chromosome and fluorescence in situ hybridization (FISH) analysis for myeloid disorders that are defined by specific cytogenetic abnormalities. The eosinophilia in these cases is somewhat unique, as a significant association between the involved genetic loci and eosinophilia has not been established for any germline alterations within this subclassification of cases. Certifications American Board of Pathology, Hematology American Board of Pathology, Anatomic and Clinic . , Espinal-Witter R, Geyer JT, et al. The current International Consensus classification, with its strong morphology component, will represent a basis for refinement. 2023 Jan;98(1):166-179. doi: 10.1002/ajh.26751. University of Wisconsin Hospitals and Clinics, Madison, WI, Hematopathology. , Orazi A, Hasserjian R, et al. The rarity of eosinophilia in properly diagnosed cases of MDS is well known. Hertzman Nakayama BM and PB demonstrated a proliferation of several myeloid lineages, including monocytes, eosinophils, basophils, and mast cells. T Within the subcategory of primary immunodeficiencies with associated eosinophilia, three of the cases are representative of autosomal-dominant (case 284) and autosomal-recessive (cases 90 and 256) hyper-IgE syndrome and provide a framework to consider a number of the other submitted primary immunodeficiency cases. BM histology (C) showed features of myelodysplastic syndrome/myeloproliferative neoplasm with increased number of megakaryocytes with clustering. Molecular testing helps to better subclassify myeloid neoplasms with eosinophilia and to identify patients for targeted treatments. This case is an example of a tryptase-positive AML, a finding that correlates well with the mastocytosis-like clinical presentation. , Nandakumar V, Jiang XX, et al. Therefore, after considering the clinical information, morphologic findings, genes involved, number of mutations, and allele frequency, the panel felt the information was insufficient to establish a diagnosis of CEL, NOS.21, While the number of publications on NGS in IHES is limited, the study by Wang et al14,24 reported that patients with IHES who had clonality detected by NGS showed clinical features more akin to CEL, NOS, in contrast to patients with IHES who lacked evidence of molecular clonality. On the other hand, the presence of aberrant basophilic eosinophilic granules is not unique for AML with inv(16), and in our workshop series, it was observed in a case of AML with biallelic mutation of CEBPA (case 281) and in a case of early T-cell precursor acute lymphoblastic leukemia (case 158). 2009 Jul 30;114(5):937-51. doi: 10.1182/blood-2009-03-209262. Wang et al21 have reported frequent dysmegakaryopoiesis and other dysplastic features in CEL, NOS and suggested that, similar to other myeloid neoplasms, BM morphology should be one of the major criteria to distinguish CEL, NOS from IHES. In the presence of persistent HE, a diagnosis of CEL, NOS may be preferable in most instances. Hematopathology Faculty Member - The University of Arkansas for Medical Sciences Department of Pathology. L Schober Clinical, Laboratory, Cytogenetic, and Molecular Findings of Myeloproliferative Neoplasm, Myelodysplastic Syndrome, Myelodysplastic/Myeloproliferative Neoplasm, and Therapy-Related Myeloid Neoplasm Cases. The World Health Organization classification of neoplastic diseases of the haematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee Meeting, Airlie House, Virginia, November 1997. Careers. Matsushima The IWG would optimally create a Web-based portal, which could be readily accessed via computer or mobile device worldwide. This workshop brought together a unique set of diverse entities for study and discussion. Case 71 was of a virus-associated HE, specifically parvovirus B19. Both cases describe male infants with eczema and petechiae. Case 213: Chronic eosinophilic pneumonia. Strasser B, Grnberger M, Steindl R, Heibl S, Thaler J, Haushofer A. Mol Clin Oncol. Scattered eosinophils can be seen percolating through the myocardium (H&E, 1,000). Society for Hematopathology We are an academic practice based at TriCore Reference Laboratories, where we perform and interpret all testing necessary to support a contemporary hematopathology serviceincluding 10-color flow cytometry, comprehensive cytogenetic and molecular offerings, and in-house next-generation sequencing for myeloid and other neoplasms. L The SH is a diverse community of international physicians, scientists, diagnostic hematopathologists and surgical pathologists from a variety of backgrounds including community practice and academia, as well as researchers with interests in hematology/hematopathology. Non-SH Members should click the "Create New Account" button below to open a SH Online account. As delineated below, several key themes and concepts emerged from these case presentations. Therefore, eosinophilia can be driven by several other mechanisms in B-ALL, some of which may not be known yet. Finally, an interactive feature allowing ongoing exchange between the IWG and the scientific community will be of fundamental importance to solicit feedback and enable refinement on a continual basis.
society for hematopathology