Mild hypogammaglobulinemia can be a serious condition

L.M.A. Janssen, P. Bassett, T. Macken, J. van Esch, J. Pruijt, A. Knoops, M. Sköld, A. Parker, J. de Vries, Esther de Vries

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Abstract

Background
Most patients with primary antibody deficiency (PAD) suffer from less well-described and understood forms of hypogammaglobulinemia (unclassified primary antibody deficiency, unPAD). Because of the moderately decreased immunoglobulin levels compared to CVID, unPAD is generally considered to be clinically mild and not very relevant.

Objective
To describe our cohort of—mainly—unPAD patients, and to analyze whether subgroups can be identified.

Methods
Data were prospectively collected (February-2012 to June-2016) as part of a standardized, 1-day Care Pathway for suspected primary immunodeficiency. The TNO-AZL Questionnaire for Health-Related Quality of Life (HRQoL) was part of the pre-first-visit intake procedure.

Results
Three hundred and twenty patients were referred to the Care Pathway. Data from 23/27 children and 99/113 adults who were diagnosed with PAD and gave informed consent were available for analysis. 89/99 adults had unPAD, the majority (74%) were female and 44% already showed bronchiectasis. HRQoL was significantly decreased in all domains, meaning that a lot of unPAD patients had to cope simultaneously with pain, negative feelings and impairments in cognition, home management tasks, sleep, social interaction, and work. The most prominently impaired HRQoL domain was vitality, indicating these patients feel extremely tired and worn out.

Conclusion
These results highlight the need for more attention to the potential patient burden of unPADs. A larger cohort is needed to increase our understanding of unPADs and to analyze whether distinct subgroups can be identified. For now, it is important for the clinician to acknowledge the existence of unPAD and be aware of its potential consequences, in order to timely and appropriately manage its effects and complications.
Original languageEnglish
Article number2384
Number of pages9
JournalFrontiers in Immunology
Volume9
DOIs
Publication statusPublished - 2018

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Agammaglobulinemia
Informed Consent

Keywords

  • CHILDREN
  • CLASSIFICATION
  • COMMON VARIABLE IMMUNODEFICIENCY
  • DISEASE
  • DISORDERS
  • IDENTIFICATION
  • IGA DEFICIENCY
  • PATIENT
  • QUALITY-OF-LIFE
  • RESPONSES
  • common variable immunodeficiency disorders
  • hypogammaglobulinemia
  • immunodeficiency
  • pneumococcal vaccination response
  • primary antibody deficiency
  • primary immunodeficiency
  • unclassified primary antibody deficiency

Cite this

Janssen, L. M. A., Bassett, P., Macken, T., van Esch, J., Pruijt, J., Knoops, A., ... de Vries, E. (2018). Mild hypogammaglobulinemia can be a serious condition. Frontiers in Immunology, 9, [2384]. https://doi.org/10.3389/fimmu.2018.02384
Janssen, L.M.A. ; Bassett, P. ; Macken, T. ; van Esch, J. ; Pruijt, J. ; Knoops, A. ; Sköld, M. ; Parker, A. ; de Vries, J. ; de Vries, Esther. / Mild hypogammaglobulinemia can be a serious condition. In: Frontiers in Immunology. 2018 ; Vol. 9.
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abstract = "BackgroundMost patients with primary antibody deficiency (PAD) suffer from less well-described and understood forms of hypogammaglobulinemia (unclassified primary antibody deficiency, unPAD). Because of the moderately decreased immunoglobulin levels compared to CVID, unPAD is generally considered to be clinically mild and not very relevant.ObjectiveTo describe our cohort of—mainly—unPAD patients, and to analyze whether subgroups can be identified.MethodsData were prospectively collected (February-2012 to June-2016) as part of a standardized, 1-day Care Pathway for suspected primary immunodeficiency. The TNO-AZL Questionnaire for Health-Related Quality of Life (HRQoL) was part of the pre-first-visit intake procedure.ResultsThree hundred and twenty patients were referred to the Care Pathway. Data from 23/27 children and 99/113 adults who were diagnosed with PAD and gave informed consent were available for analysis. 89/99 adults had unPAD, the majority (74{\%}) were female and 44{\%} already showed bronchiectasis. HRQoL was significantly decreased in all domains, meaning that a lot of unPAD patients had to cope simultaneously with pain, negative feelings and impairments in cognition, home management tasks, sleep, social interaction, and work. The most prominently impaired HRQoL domain was vitality, indicating these patients feel extremely tired and worn out.ConclusionThese results highlight the need for more attention to the potential patient burden of unPADs. A larger cohort is needed to increase our understanding of unPADs and to analyze whether distinct subgroups can be identified. For now, it is important for the clinician to acknowledge the existence of unPAD and be aware of its potential consequences, in order to timely and appropriately manage its effects and complications.",
keywords = "CHILDREN, CLASSIFICATION, COMMON VARIABLE IMMUNODEFICIENCY, DISEASE, DISORDERS, IDENTIFICATION, IGA DEFICIENCY, PATIENT, QUALITY-OF-LIFE, RESPONSES, common variable immunodeficiency disorders, hypogammaglobulinemia, immunodeficiency, pneumococcal vaccination response, primary antibody deficiency, primary immunodeficiency, unclassified primary antibody deficiency",
author = "L.M.A. Janssen and P. Bassett and T. Macken and {van Esch}, J. and J. Pruijt and A. Knoops and M. Sk{\"o}ld and A. Parker and {de Vries}, J. and {de Vries}, Esther",
year = "2018",
doi = "10.3389/fimmu.2018.02384",
language = "English",
volume = "9",
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Janssen, LMA, Bassett, P, Macken, T, van Esch, J, Pruijt, J, Knoops, A, Sköld, M, Parker, A, de Vries, J & de Vries, E 2018, 'Mild hypogammaglobulinemia can be a serious condition', Frontiers in Immunology, vol. 9, 2384. https://doi.org/10.3389/fimmu.2018.02384

Mild hypogammaglobulinemia can be a serious condition. / Janssen, L.M.A.; Bassett, P.; Macken, T.; van Esch, J.; Pruijt, J.; Knoops, A.; Sköld, M.; Parker, A.; de Vries, J.; de Vries, Esther.

In: Frontiers in Immunology, Vol. 9, 2384, 2018.

Research output: Contribution to journalArticleScientificpeer-review

TY - JOUR

T1 - Mild hypogammaglobulinemia can be a serious condition

AU - Janssen, L.M.A.

AU - Bassett, P.

AU - Macken, T.

AU - van Esch, J.

AU - Pruijt, J.

AU - Knoops, A.

AU - Sköld, M.

AU - Parker, A.

AU - de Vries, J.

AU - de Vries, Esther

PY - 2018

Y1 - 2018

N2 - BackgroundMost patients with primary antibody deficiency (PAD) suffer from less well-described and understood forms of hypogammaglobulinemia (unclassified primary antibody deficiency, unPAD). Because of the moderately decreased immunoglobulin levels compared to CVID, unPAD is generally considered to be clinically mild and not very relevant.ObjectiveTo describe our cohort of—mainly—unPAD patients, and to analyze whether subgroups can be identified.MethodsData were prospectively collected (February-2012 to June-2016) as part of a standardized, 1-day Care Pathway for suspected primary immunodeficiency. The TNO-AZL Questionnaire for Health-Related Quality of Life (HRQoL) was part of the pre-first-visit intake procedure.ResultsThree hundred and twenty patients were referred to the Care Pathway. Data from 23/27 children and 99/113 adults who were diagnosed with PAD and gave informed consent were available for analysis. 89/99 adults had unPAD, the majority (74%) were female and 44% already showed bronchiectasis. HRQoL was significantly decreased in all domains, meaning that a lot of unPAD patients had to cope simultaneously with pain, negative feelings and impairments in cognition, home management tasks, sleep, social interaction, and work. The most prominently impaired HRQoL domain was vitality, indicating these patients feel extremely tired and worn out.ConclusionThese results highlight the need for more attention to the potential patient burden of unPADs. A larger cohort is needed to increase our understanding of unPADs and to analyze whether distinct subgroups can be identified. For now, it is important for the clinician to acknowledge the existence of unPAD and be aware of its potential consequences, in order to timely and appropriately manage its effects and complications.

AB - BackgroundMost patients with primary antibody deficiency (PAD) suffer from less well-described and understood forms of hypogammaglobulinemia (unclassified primary antibody deficiency, unPAD). Because of the moderately decreased immunoglobulin levels compared to CVID, unPAD is generally considered to be clinically mild and not very relevant.ObjectiveTo describe our cohort of—mainly—unPAD patients, and to analyze whether subgroups can be identified.MethodsData were prospectively collected (February-2012 to June-2016) as part of a standardized, 1-day Care Pathway for suspected primary immunodeficiency. The TNO-AZL Questionnaire for Health-Related Quality of Life (HRQoL) was part of the pre-first-visit intake procedure.ResultsThree hundred and twenty patients were referred to the Care Pathway. Data from 23/27 children and 99/113 adults who were diagnosed with PAD and gave informed consent were available for analysis. 89/99 adults had unPAD, the majority (74%) were female and 44% already showed bronchiectasis. HRQoL was significantly decreased in all domains, meaning that a lot of unPAD patients had to cope simultaneously with pain, negative feelings and impairments in cognition, home management tasks, sleep, social interaction, and work. The most prominently impaired HRQoL domain was vitality, indicating these patients feel extremely tired and worn out.ConclusionThese results highlight the need for more attention to the potential patient burden of unPADs. A larger cohort is needed to increase our understanding of unPADs and to analyze whether distinct subgroups can be identified. For now, it is important for the clinician to acknowledge the existence of unPAD and be aware of its potential consequences, in order to timely and appropriately manage its effects and complications.

KW - CHILDREN

KW - CLASSIFICATION

KW - COMMON VARIABLE IMMUNODEFICIENCY

KW - DISEASE

KW - DISORDERS

KW - IDENTIFICATION

KW - IGA DEFICIENCY

KW - PATIENT

KW - QUALITY-OF-LIFE

KW - RESPONSES

KW - common variable immunodeficiency disorders

KW - hypogammaglobulinemia

KW - immunodeficiency

KW - pneumococcal vaccination response

KW - primary antibody deficiency

KW - primary immunodeficiency

KW - unclassified primary antibody deficiency

U2 - 10.3389/fimmu.2018.02384

DO - 10.3389/fimmu.2018.02384

M3 - Article

VL - 9

JO - Frontiers in Immunology

JF - Frontiers in Immunology

SN - 1664-3224

M1 - 2384

ER -

Janssen LMA, Bassett P, Macken T, van Esch J, Pruijt J, Knoops A et al. Mild hypogammaglobulinemia can be a serious condition. Frontiers in Immunology. 2018;9. 2384. https://doi.org/10.3389/fimmu.2018.02384