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  1. Article ; Online: Diagnosis and therapeutic decision-making for the neutropenic patient.

    Connelly, James A / Walkovich, Kelly

    Hematology. American Society of Hematology. Education Program

    2022  Volume 2021, Issue 1, Page(s) 492–503

    Abstract: Determining the cause of a low neutrophil count in a pediatric or adult patient is essential for the hematologist's clinical decision-making. Fundamental to this diagnostic process is establishing the presence or lack of a mature neutrophil storage pool, ...

    Abstract Determining the cause of a low neutrophil count in a pediatric or adult patient is essential for the hematologist's clinical decision-making. Fundamental to this diagnostic process is establishing the presence or lack of a mature neutrophil storage pool, as absence places the patient at higher risk for infection and the need for supportive care measures. Many diagnostic tests, eg, a peripheral blood smear and bone marrow biopsy, remain important tools, but greater understanding of the diversity of neutropenic disorders has added new emphasis on evaluating for immune disorders and genetic testing. In this article, a structure is provided to assess patients based on the mechanism of neutropenia and to prioritize testing based on patient age and hypothesized pathophysiology. Common medical quandaries including fever management, need for growth factor support, risk of malignant transformation, and curative options in congenital neutropenia are reviewed to guide medical decision-making in neutropenic patients.
    Language English
    Publishing date 2022-01-05
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2084287-9
    ISSN 1520-4383 ; 1520-4391
    ISSN (online) 1520-4383
    ISSN 1520-4391
    DOI 10.1182/hematology.2021000284
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Understanding neutropenia secondary to intrinsic or iatrogenic immune dysregulation.

    Walkovich, Kelly / Connelly, James A

    Hematology. American Society of Hematology. Education Program

    2022  Volume 2021, Issue 1, Page(s) 504–513

    Abstract: As a key member of the innate and adaptive immune response, neutrophils provide insights into the hematopoietic and inflammatory manifestations of inborn errors of immunity (IEI) and the consequences of immunotherapy. The facile recognition of IEI ... ...

    Abstract As a key member of the innate and adaptive immune response, neutrophils provide insights into the hematopoietic and inflammatory manifestations of inborn errors of immunity (IEI) and the consequences of immunotherapy. The facile recognition of IEI presenting with neutropenia provides an avenue for hematologists to facilitate early diagnosis and expedite biologically rationale care. Moreover, enhancing the understanding of the molecular mechanisms driving neutropenia in IEI-decreased bone marrow reserves, diminished egress from the bone marrow, and decreased survival-offers an opportunity to further dissect the pathophysiology driving neutropenia secondary to iatrogenic immune dysregulation, eg, immune checkpoint inhibitors and chimeric antigen receptor T-cell therapy.
    Language English
    Publishing date 2022-01-05
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2084287-9
    ISSN 1520-4383 ; 1520-4391
    ISSN (online) 1520-4383
    ISSN 1520-4391
    DOI 10.1182/hematology.2021000285
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  3. Article ; Online: A Sherlock Approach to a Kindred With a Variable Immunohematologic Phenotype.

    Walkovich, Kelly / Grunebaum, Eyal

    The journal of allergy and clinical immunology. In practice

    2022  Volume 10, Issue 7, Page(s) 1714–1722

    Abstract: Given the ubiquity of leukopenia and sinopulmonary infections in childhood, differentiating patients with inborn errors of immunity (IEI) from otherwise healthy patients can be challenging. The diagnostic complexity is further exacerbated in disorders ... ...

    Abstract Given the ubiquity of leukopenia and sinopulmonary infections in childhood, differentiating patients with inborn errors of immunity (IEI) from otherwise healthy patients can be challenging. The diagnostic complexity is further exacerbated in disorders with wide phenotypic variability such as warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome. However, using a Sherlock approach with careful attention to details in the patient's medical history and physical examination coupled with a comprehensive family history can heighten the index of suspicion for underlying IEI. Subsequent iterative and deductive reasoning incorporating results from laboratory interrogation, response (or lack thereof) to standard therapy, and emergence of new symptoms can further aid in a timely diagnosis of IEI. Herein, we detail a WHIM syndrome kindred with marked phenotype variability, identified after the presentation of a child with intermittent neutropenia and sinopulmonary infections. The complexity of this kindred highlights the utility of an interspecialty, collaborative Sherlock approach to diagnosis, and care. In addition, the genetic underpinnings, diagnostic approaches, clinical features, supportive care options, and management of WHIM syndrome are reviewed.
    MeSH term(s) Agammaglobulinemia/diagnosis ; Agammaglobulinemia/genetics ; Biological Variation, Population ; Humans ; Immunologic Deficiency Syndromes/diagnosis ; Immunologic Deficiency Syndromes/genetics ; Immunologic Deficiency Syndromes/therapy ; Neutropenia/diagnosis ; Phenotype ; Primary Immunodeficiency Diseases ; Receptors, CXCR4/genetics ; Warts
    Chemical Substances Receptors, CXCR4
    Language English
    Publishing date 2022-04-22
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2022.04.004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Understanding the Spectrum of Immune Dysregulation Manifestations in Autoimmune Lymphoproliferative Syndrome and Autoimmune Lymphoproliferative Syndrome-like Disorders.

    Failing, Christopher / Blase, Jennifer R / Walkovich, Kelly

    Rheumatic diseases clinics of North America

    2023  Volume 49, Issue 4, Page(s) 841–860

    Abstract: As a disorder of immune dysregulation, autoimmune lymphoproliferative syndrome (ALPS) stems from pathogenic variants in the first apoptosis signal-mediated apoptosis (Fas) and Fas-ligand pathway that result in elevations of CD3+ TCRαβ+ CD4- CD8- T cells ... ...

    Abstract As a disorder of immune dysregulation, autoimmune lymphoproliferative syndrome (ALPS) stems from pathogenic variants in the first apoptosis signal-mediated apoptosis (Fas) and Fas-ligand pathway that result in elevations of CD3+ TCRαβ+ CD4- CD8- T cells along with chronic lymphoproliferation, a heightened risk for malignancy, and importantly for the rheumatologist, increased risk of autoimmunity. While immune cytopenias are the most encountered autoimmune phenomena, there is increasing appreciation for ocular, musculoskeletal, pulmonary and renal inflammatory manifestations similar to more common rheumatology diseases. Additionally, ALPS-like conditions that share similar clinical features and opportunities for targeted therapy are increasingly recognized via genetic testing, highlighting the need for rheumatologists to be facile in the recognition and diagnosis of this spectrum of disorders. This review will focus on clinical and laboratory features of both ALPS and ALPS-like disorders with the intent to provide a framework for rheumatologists to understand the pathophysiologic drivers and discriminate between diagnoses.
    MeSH term(s) Humans ; Autoimmune Lymphoproliferative Syndrome/diagnosis ; Autoimmune Lymphoproliferative Syndrome/genetics ; fas Receptor/genetics ; Autoimmunity ; Neoplasms ; Autoimmune Diseases
    Chemical Substances fas Receptor
    Language English
    Publishing date 2023-08-23
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 92118-x
    ISSN 1558-3163 ; 0889-857X
    ISSN (online) 1558-3163
    ISSN 0889-857X
    DOI 10.1016/j.rdc.2023.07.001
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  5. Article ; Online: Heterozygous TCF3-related disease presenting as X-linked agammaglobulinemia mimicry in a male toddler with B-cell aplasia, agammaglobulinemia, and severe neutropenia.

    Bou-Maroun, Laura M / Walkovich, Kelly J / Frazier, Lauren / Hannibal, Mark / Michniacki, Thomas F

    Pediatric blood & cancer

    2024  Volume 71, Issue 6, Page(s) e30947

    Language English
    Publishing date 2024-03-10
    Publishing country United States
    Document type Letter
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.30947
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  6. Article ; Online: Case Report: Use of Obinutuzumab as an Alternative Monoclonal Anti-CD20 Antibody in a Patient With Refractory Immune Thrombocytopenia Complicated by Rituximab-Induced Serum Sickness and Anti-Rituximab Antibodies.

    Blase, Jennifer R / Frame, David / Michniacki, Thomas F / Walkovich, Kelly

    Frontiers in immunology

    2022  Volume 13, Page(s) 863177

    Abstract: Management of refractory immune thrombocytopenia frequently involves rituximab, a chimeric anti-CD20 monoclonal antibody, to target B cells and induce remission in most patients. However, neutralizing antibodies to rituximab that nullify therapeutic ... ...

    Abstract Management of refractory immune thrombocytopenia frequently involves rituximab, a chimeric anti-CD20 monoclonal antibody, to target B cells and induce remission in most patients. However, neutralizing antibodies to rituximab that nullify therapeutic response and may lead to serum sickness have been rarely reported. Here, we present a case of a young adult woman with Evans syndrome treated with rituximab, complicated by the development of serum sickness, acute respiratory distress syndrome, and platelet refractoriness presumed secondary to neutralizing antibodies to rituximab. She was successfully treated with the humanized anti-CD20 monoclonal antibody, obinutuzumab, with subsequent symptom resolution. Additionally, a review of 10 previously published cases of serum-sickness associated with the use of rituximab for idiopathic thrombocytopenic purpura (ITP) is summarized. This case highlights that recognition of more subtle or rare symptoms of rituximab-induced serum sickness is important to facilitate rapid intervention.
    MeSH term(s) Antibodies, Monoclonal, Humanized ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Antibodies, Neutralizing/therapeutic use ; Female ; Humans ; Purpura, Thrombocytopenic, Idiopathic/chemically induced ; Purpura, Thrombocytopenic, Idiopathic/diagnosis ; Purpura, Thrombocytopenic, Idiopathic/drug therapy ; Rituximab/adverse effects ; Serum Sickness/chemically induced ; Serum Sickness/diagnosis
    Chemical Substances Antibodies, Monoclonal, Humanized ; Antibodies, Monoclonal, Murine-Derived ; Antibodies, Neutralizing ; Rituximab (4F4X42SYQ6) ; obinutuzumab (O43472U9X8)
    Language English
    Publishing date 2022-04-19
    Publishing country Switzerland
    Document type Case Reports ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2022.863177
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  7. Article ; Online: Congenital Neutropenia and Rare Functional Phagocyte Disorders in Children.

    Walkovich, Kelly / Connelly, James A

    Hematology/oncology clinics of North America

    2019  Volume 33, Issue 3, Page(s) 533–551

    Abstract: Both profound neutropenia and functional phagocyte disorders render patients susceptible to recurrent, unusual, and/or life-threatening infections. Many disorders also have nonhematologic manifestations and a substantial risk of leukemogenesis. Diagnosis ...

    Abstract Both profound neutropenia and functional phagocyte disorders render patients susceptible to recurrent, unusual, and/or life-threatening infections. Many disorders also have nonhematologic manifestations and a substantial risk of leukemogenesis. Diagnosis relies on clinical suspicion and interrogation of the complete blood count with differential/bone marrow examination coupled with immunologic and genetic analyses. Treatment of the quantitative neutrophil disorders depends on granulocyte colony-stimulating factor, whereas management of functional phagocyte disease is reliant on antimicrobials and/or targeted therapies. Hematopoietic stem cell transplant remains the only curative option for most disorders but is not used on a routine basis.
    MeSH term(s) Child ; Congenital Bone Marrow Failure Syndromes/genetics ; Congenital Bone Marrow Failure Syndromes/immunology ; Congenital Bone Marrow Failure Syndromes/therapy ; Granulocyte Colony-Stimulating Factor/therapeutic use ; Hematopoietic Stem Cell Transplantation/methods ; Humans ; Leukemia/genetics ; Leukemia/immunology ; Leukemia/therapy ; Myelodysplastic Syndromes/genetics ; Myelodysplastic Syndromes/immunology ; Myelodysplastic Syndromes/therapy ; Neutropenia/congenital ; Neutropenia/genetics ; Neutropenia/immunology ; Neutropenia/therapy ; Neutrophils/immunology ; Neutrophils/metabolism ; Phagocytes/immunology ; Phagocytes/metabolism ; Rare Diseases/genetics ; Rare Diseases/immunology ; Rare Diseases/therapy
    Chemical Substances Granulocyte Colony-Stimulating Factor (143011-72-7)
    Language English
    Publishing date 2019-04-01
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 93115-9
    ISSN 1558-1977 ; 0889-8588
    ISSN (online) 1558-1977
    ISSN 0889-8588
    DOI 10.1016/j.hoc.2019.01.004
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  8. Article ; Online: Adjustments to pharmacologic therapies for hemophagocytic lymphohistiocytosis while on extracorporeal support.

    Weyand, Angela C / Barbaro, Ryan P / Walkovich, Kelly J / Frame, David G

    Pediatric blood & cancer

    2021  Volume 68, Issue 6, Page(s) e29007

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is an immune dysregulatory syndrome characterized by severe inflammation and end-organ damage. Due to significant organ dysfunction, patients often require extracorporeal membrane oxygenation (ECMO) and continuous ...

    Abstract Hemophagocytic lymphohistiocytosis (HLH) is an immune dysregulatory syndrome characterized by severe inflammation and end-organ damage. Due to significant organ dysfunction, patients often require extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT). In this report, we describe consideration for adjusting treatment in the context of extracorporeal organ support. We describe agents commonly used and dosing adjustments made in light of extracorporeal organ support. We report six cases that illustrate the feasibility of initiating standard HLH therapies in patients requiring these modalities.
    MeSH term(s) Anti-Inflammatory Agents/therapeutic use ; Child, Preschool ; Continuous Renal Replacement Therapy ; Dexamethasone/therapeutic use ; Etoposide/therapeutic use ; Extracorporeal Membrane Oxygenation ; Humans ; Infant ; Infant, Newborn ; Intensive Care Units, Pediatric ; Lymphohistiocytosis, Hemophagocytic/drug therapy ; Retrospective Studies
    Chemical Substances Anti-Inflammatory Agents ; Etoposide (6PLQ3CP4P3) ; Dexamethasone (7S5I7G3JQL)
    Language English
    Publishing date 2021-03-10
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.29007
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    ab Jg. 2022: Lesesaal (EG)

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  9. Article ; Online: Absolute neutrophil count nadir in healthy pediatric patients with the Duffy-null phenotype.

    Merz, Lauren E / Li, Shih-Hon / Ney, Gina / Michniacki, Thomas F / Hannibal, Mark C / Walkovich, Kelly J

    Blood advances

    2023  Volume 7, Issue 15, Page(s) 4182–4185

    MeSH term(s) Leukocyte Count ; Neutrophils ; Phenotype ; Humans ; Child
    Language English
    Publishing date 2023-06-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2915908-8
    ISSN 2473-9537 ; 2473-9529
    ISSN (online) 2473-9537
    ISSN 2473-9529
    DOI 10.1182/bloodadvances.2023010368
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  10. Article ; Online: Expanding clinical spectrum of female X-linked lymphoproliferative syndrome 2.

    Suryaprakash, Shruthi / El-Baba, Mohammad / Walkovich, Kelly J / Savaşan, Süreyya

    Pediatric blood & cancer

    2020  Volume 68, Issue 2, Page(s) e28592

    MeSH term(s) Adolescent ; Agammaglobulinemia/diagnosis ; Agammaglobulinemia/pathology ; Antibodies, Viral/blood ; B-Lymphocytes/cytology ; B-Lymphocytes/immunology ; Epstein-Barr Virus Infections/diagnosis ; Female ; Genetic Diseases, X-Linked/diagnosis ; Genetic Diseases, X-Linked/genetics ; Genetic Diseases, X-Linked/virology ; Herpesvirus 4, Human/immunology ; Herpesvirus 4, Human/isolation & purification ; Humans ; Lymphocyte Count ; Lymphoproliferative Disorders/diagnosis ; Lymphoproliferative Disorders/genetics ; Lymphoproliferative Disorders/virology ; X-Linked Inhibitor of Apoptosis Protein/genetics
    Chemical Substances Antibodies, Viral ; X-Linked Inhibitor of Apoptosis Protein ; XIAP protein, human
    Language English
    Publishing date 2020-07-19
    Publishing country United States
    Document type Case Reports ; Letter
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.28592
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