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Article ; Online: Evans syndrome: pathology and genomic hubris.

Rao, V Koneti

2022 Volume 139, Issue 3, Page(s) 312–313

MeSH term(s)	Anemia, Hemolytic, Autoimmune/genetics ; Genomics ; Humans ; Thrombocytopenia/genetics
Language	English
Publishing date	2022-01-20
Publishing country	United States
Document type	Editorial ; Comment
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood.2021013636
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Article ; Online: Spleen is cool.

Rao, V Koneti

Pediatric hematology and oncology

2019 Volume 36, Issue 6, Page(s) 327–329

MeSH term(s)	Humans ; Spleen
Language	English
Publishing date	2019-09-18
Publishing country	England
Document type	Journal Article
ZDB-ID	632914-7
ISSN	1521-0669 ; 0888-0018
ISSN (online)	1521-0669
ISSN	0888-0018
DOI	10.1080/08880018.2019.1660744
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Article ; Online: The Role of Bone Marrow Evaluation in Clinical Allergy and Immunology Practice: When and Why.

Boggs, Nathan A / Rao, V Koneti

The journal of allergy and clinical immunology. In practice

2020 Volume 8, Issue 10, Page(s) 3356–3362

Abstract: Allergists and immunologists rely on other specialists for higher risk procedures such as biopsies of the lung or gastrointestinal tract. However, we perform and interpret a handful of procedures ourselves. Training programs have historically required ... ...

Abstract	Allergists and immunologists rely on other specialists for higher risk procedures such as biopsies of the lung or gastrointestinal tract. However, we perform and interpret a handful of procedures ourselves. Training programs have historically required competency for prescribing immunoglobulin infusions, patch testing, rhino laryngoscopy, lung function testing, and provocation testing for airway hyperreactivity even though other specialists often perform them. Bone marrow aspirations and biopsies are not included in fellowship training assessments despite a significant number of marrow evaluations being requested by allergists and immunologists. For example, nearly 1 marrow assessment per month has been requested over 2 years for patients in the Allergy Immunology Clinic at Walter Reed National Military Medical Center. Marrow assessments are often required for diagnosis, monitoring, and treatment-related toxicities. Interpretive and procedural competency would benefit the field given the range of diseases in clinical immunology practice that require marrow assessment. We have generated a comprehensive list of the major conditions that might require bone marrow assessments in any Allergy and Immunology practice. We then summarize the specific tests that must be ordered and show how to determine sample quality. Finally, some providers may desire procedural competency and for those individuals we discuss tips for the procedure.
MeSH term(s)	Allergy and Immunology ; Bone Marrow ; Bone Marrow Examination ; Humans ; Hypersensitivity/diagnosis ; Patch Tests ; Specialization
Language	English
Publishing date	2020-06-09
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Intramural
ZDB-ID	2843237-X
ISSN	2213-2201 ; 2213-2198
ISSN (online)	2213-2201
ISSN	2213-2198
DOI	10.1016/j.jaip.2020.05.049
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Article ; Online: Serendipity in splendid isolation: rapamycin.

Rao, V Koneti

Blood

2016 Volume 127, Issue 1, Page(s) 5–6

Abstract: In this issue of Blood, Bride et al report results of the first prospective multi-institutional trial of a long-term single-agent therapy for refractory cytopenias using rapamycin in 30 patients and show remarkable efficacy in children with autoimmune ... ...

Abstract	In this issue of Blood, Bride et al report results of the first prospective multi-institutional trial of a long-term single-agent therapy for refractory cytopenias using rapamycin in 30 patients and show remarkable efficacy in children with autoimmune lymphoproliferative syndrome (ALPS).
MeSH term(s)	Autoimmune Diseases/drug therapy ; Drug Resistance, Neoplasm/drug effects ; Female ; Hematologic Diseases/drug therapy ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Neoplasm Recurrence, Local/drug therapy ; Salvage Therapy ; Sirolimus/therapeutic use
Chemical Substances	Immunosuppressive Agents ; Sirolimus (W36ZG6FT64)
Language	English
Publishing date	2016-01-06
Publishing country	United States
Document type	Journal Article ; Comment
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood-2015-11-679829
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Article ; Online: PI3Kδ Pathway Dysregulation and Unique Features of Its Inhibition by Leniolisib in Activated PI3Kδ Syndrome and Beyond.

Cant, Andrew J / Chandra, Anita / Munro, Ewen / Rao, V Koneti / Lucas, Carrie L

The journal of allergy and clinical immunology. In practice

2023 Volume 12, Issue 1, Page(s) 69–78

Abstract: The phosphoinositide 3-kinase (PI3K) pathway regulates diverse cellular processes, with finely tuned PI3Kδ activity being crucial for immune cell development and function. Genetic hyperactivation of PI3Kδ causes the inborn error of immunity activated ... ...

Abstract	The phosphoinositide 3-kinase (PI3K) pathway regulates diverse cellular processes, with finely tuned PI3Kδ activity being crucial for immune cell development and function. Genetic hyperactivation of PI3Kδ causes the inborn error of immunity activated phosphoinositide 3-kinase δ syndrome (APDS). Several PI3Kδ inhibitors have been investigated as treatment options for APDS, but only leniolisib has shown both efficacy and tolerability. In contrast, severe immune-mediated adverse events such as colitis, neutropenia, and hepatotoxicity have been observed with other PI3Kδ inhibitors, particularly those indicated for hematological malignancies. We propose that leniolisib is distinguished from other PI3Kδ inhibitors due to its structure, specific inhibitory properties selectively targeting the δ isoform without overinhibition of the δ or γ isoforms, and the precise match between APDS mechanism of disease and drug mechanism of action.
MeSH term(s)	Humans ; Phosphatidylinositol 3-Kinases ; Class I Phosphatidylinositol 3-Kinases/genetics ; Pyrimidines ; Phosphatidylinositol 3-Kinase
Chemical Substances	leniolisib ; Phosphatidylinositol 3-Kinases (EC 2.7.1.-) ; Class I Phosphatidylinositol 3-Kinases (EC 2.7.1.137) ; Pyrimidines ; Phosphatidylinositol 3-Kinase (EC 2.7.1.137)
Language	English
Publishing date	2023-09-28
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.2023.09.016
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Article: Approaches to Managing Autoimmune Cytopenias in Novel Immunological Disorders with Genetic Underpinnings Like Autoimmune Lymphoproliferative Syndrome.

Rao, V Koneti

Frontiers in pediatrics

2015 Volume 3, Page(s) 65

Abstract: Autoimmune lymphoproliferative syndrome (ALPS) is a rare disorder of apoptosis. It is frequently caused by mutations in FAS (TNFRSF6) gene. Unlike most of the self-limiting autoimmune cytopenias sporadically seen in childhood, multi lineage cytopenias ... ...

Abstract	Autoimmune lymphoproliferative syndrome (ALPS) is a rare disorder of apoptosis. It is frequently caused by mutations in FAS (TNFRSF6) gene. Unlike most of the self-limiting autoimmune cytopenias sporadically seen in childhood, multi lineage cytopenias due to ALPS are often refractory, as their inherited genetic defect is not going to go away. Historically, more ALPS patients have died due to overwhelming sepsis following splenectomy to manage their chronic cytopenias than due to any other cause, including malignancies. Hence, current recommendations underscore the importance of avoiding splenectomy in ALPS, by long-term use of corticosteroid-sparing immunosuppressive agents like mycophenolate mofetil and sirolimus. Paradigms learnt from managing ALPS patients in recent years is highlighted here and can be extrapolated to manage refractory cytopenias in patients with as yet undetermined genetic bases for their ailments. It is also desirable to develop international registries for children with rare and complex immune problems associated with chronic multilineage cytopenias in order to elucidate their natural history and long-term comorbidities due to the disease and its treatments.
Language	English
Publishing date	2015-07-21
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2015.00065
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Article ; Online: Autoimmune lymphoproliferative immunodeficiencies (ALPIDs): A proposed approach to redefining ALPS and other lymphoproliferative immune disorders.

Magerus, Aude / Rensing-Ehl, Anne / Rao, V Koneti / Teachey, David T / Rieux-Laucat, Frederic / Ehl, Stephan

The Journal of allergy and clinical immunology

2023 Volume 153, Issue 1, Page(s) 67–76

Abstract: Chronic nonmalignant lymphoproliferation and autoimmune cytopenia are relevant manifestations of immunohematologic diseases of childhood. Their diagnostic classification is challenging but important for therapy. Autoimmune lymphoproliferative syndrome ( ... ...

Abstract	Chronic nonmalignant lymphoproliferation and autoimmune cytopenia are relevant manifestations of immunohematologic diseases of childhood. Their diagnostic classification is challenging but important for therapy. Autoimmune lymphoproliferative syndrome (ALPS) is a genetically defined inborn error of immunity combining these manifestations, but it can explain only a small proportion of cases. Diagnostic categories such as ALPS-like disease, common variable immunodeficiency, or Evans syndrome have therefore been used. Advances in genetics and increasing availablity of targeted therapies call for more therapy-oriented disease classification. Moreover, recent discoveries in the (re)analysis of genetic conditions affecting FAS signaling ask for a more precise definition of ALPS. In this review, we propose the term autoimmune lymphoproliferative immunodeficiencies for a disease phenotype that is enriched for patients with genetic diseases for which targeted therapies are available. For patients without a current molecular diagnosis, this term defines a subgroup of immune dysregulatory disorders for further studies. Within the concept of autoimmune lymphoproliferative immunodeficiencies, we propose a revision of the ALPS classification, restricting use of this term to conditions with clear evidence of perturbation of FAS signaling and resulting specific biologic and clinical consequences. This proposed approach to redefining ALPS and other lymphoproliferative conditions provides a framework for disease classification and diagnosis that is relevant for the many specialists confronted with these diseases.
MeSH term(s)	Humans ; Autoimmune Lymphoproliferative Syndrome/diagnosis ; Autoimmune Lymphoproliferative Syndrome/genetics ; Autoimmune Lymphoproliferative Syndrome/therapy ; Anemia, Hemolytic, Autoimmune ; Common Variable Immunodeficiency ; Immune System Diseases ; Phenotype ; fas Receptor/genetics ; Lymphoproliferative Disorders/diagnosis ; Lymphoproliferative Disorders/genetics ; Lymphoproliferative Disorders/therapy ; Autoimmune Diseases
Chemical Substances	fas Receptor
Language	English
Publishing date	2023-11-17
Publishing country	United States
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Extramural
ZDB-ID	121011-7
ISSN	1097-6825 ; 1085-8725 ; 0091-6749
ISSN (online)	1097-6825 ; 1085-8725
ISSN	0091-6749
DOI	10.1016/j.jaci.2023.11.004
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Article ; Online: ITP: hematology's Cosette from Les Misérables.

Rao, V Koneti

Blood

2012 Volume 121, Issue 11, Page(s) 1928–1930

Abstract: In this issue of Blood, Gudbrandsdottir et al from Denmark report that in the largest multicenter cohort to date comprising newly diagnosed adults with primary immune thrombocytopenia (ITP), addition of rituximab (RTX) to high-dose dexamethasone (DEX) as ...

Abstract	In this issue of Blood, Gudbrandsdottir et al from Denmark report that in the largest multicenter cohort to date comprising newly diagnosed adults with primary immune thrombocytopenia (ITP), addition of rituximab (RTX) to high-dose dexamethasone (DEX) as first-line therapy yields higher sustained response rates.
MeSH term(s)	Antibodies, Monoclonal, Murine-Derived/administration & dosage ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Dexamethasone/administration & dosage ; Dexamethasone/therapeutic use ; Female ; Humans ; Male ; Purpura, Thrombocytopenic, Idiopathic/drug therapy ; Rituximab
Chemical Substances	Antibodies, Monoclonal, Murine-Derived ; Rituximab (4F4X42SYQ6) ; Dexamethasone (7S5I7G3JQL)
Language	English
Publishing date	2012-01-01
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Intramural ; Comment
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood-2013-01-477729
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Article ; Online: Primary immunodeficiencies: novel genes and unusual presentations.

Notarangelo, Luigi D / Uzel, Gulbu / Rao, V Koneti

Hematology. American Society of Hematology. Education Program

2019 Volume 2019, Issue 1, Page(s) 443–448

Abstract: Recent advances in genomics have greatly expanded the spectrum of primary immune deficiencies (PIDs). Along with the identification of pathogenic variants in novel genes, distinct phenotypes have been associated with different variants in the same gene. ... ...

Abstract	Recent advances in genomics have greatly expanded the spectrum of primary immune deficiencies (PIDs). Along with the identification of pathogenic variants in novel genes, distinct phenotypes have been associated with different variants in the same gene. Although PIDs have been historically defined based on increased susceptibility to infections, immune dysregulation has emerged as a frequent and in some cases, predominant phenotype. Autoimmune cytopenias with onset in childhood, lasting longer than 12 months, and affecting multiple lineages should raise the suspicion of a possible PID with monogenic origin. Characterization of the various molecular and cellular mechanisms responsible for these unusual manifestations of PIDs, although at times resource intensive, may allow for targeted intervention in many of them.
MeSH term(s)	Child ; Humans ; Male ; Primary Immunodeficiency Diseases/genetics ; Primary Immunodeficiency Diseases/metabolism ; Primary Immunodeficiency Diseases/therapy
Language	English
Publishing date	2019-12-06
Publishing country	United States
Document type	Case Reports ; Journal Article ; Research Support, N.I.H., Intramural ; Review
ZDB-ID	2084287-9
ISSN	1520-4383 ; 1520-4391
ISSN (online)	1520-4383
ISSN	1520-4391
DOI	10.1182/hematology.2019000051
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Article ; Online: Long-Term Treatment With Selective PI3Kδ Inhibitor Leniolisib in Adults With Activated PI3Kδ Syndrome.

Rao, V Koneti / Kulm, Elaine M / Grossman, Jennifer K / Buchbinder, David K / Chong, Hey / Bradt, Jason / Webster, Sharon / Šedivá, Anna / Dalm, Virgil A / Uzel, Gulbu

Blood advances

2024

Abstract: Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity that manifests as immune deficiency and dysregulation; symptoms include frequent infections and lymphoproliferation. In our dose-finding and phase 3 placebo- ... ...

Abstract	Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity that manifests as immune deficiency and dysregulation; symptoms include frequent infections and lymphoproliferation. In our dose-finding and phase 3 placebo-controlled trials, treatment with the selective PI3Kδ inhibitor leniolisib reduced lymphoproliferation and normalized lymphocyte subsets. Here, we present 6 years of follow-up from the 6 adult patients in the original dose-finding trial receiving leniolisib. We used data from the ongoing open-label extension study, which was supplemented at later time points by investigators, including health-related quality of life assessed through a clinician-reported questionnaire. We observed improvements in health-related quality of life: 5/6 patients experienced an increase in physical capabilities and socialization and a decrease in prescribed medications. Immune subsets improved in all patients: mean transitional B-cell levels decreased from 38.17% to 2.47% and the CD4:CD8 T-cell ratio normalized to 1.11. Manifestations seen before and within the first year of leniolisib exposure, such as infections and gastrointestinal conditions, attenuated following year 2 with few new conditions emerging out to year 6. Thrombocytopenia or lymphopenia remained present in half of patients at year 6. Of 83 adverse events through year 5, 90.36% were grade 1; none were grade 4-5 nor deemed leniolisib-related. Collectively, we saw an enhancement in health-related quality of life as well as durable changes in lymphocyte subsets and clinical manifestations, further supporting the use of leniolisib as a long-term therapeutic option for the treatment of APDS. (Funded by Novartis Pharmaceuticals Corporation and Pharming Group NV; ClinicalTrials.gov identifier: NCT02859727.).
Language	English
Publishing date	2024-04-09
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.2023011000
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