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Inicio Medicina Clínica Secondary hypogammaglobulinemia in adults—A large retrospective cohort study
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Vol. 162. Issue 4.
Pages 170-178 (February 2024)
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Vol. 162. Issue 4.
Pages 170-178 (February 2024)
Original article
Secondary hypogammaglobulinemia in adults—A large retrospective cohort study
Hipogammaglobulinemia secundaria en adultos: estudio de cohorte retrospectiva
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Ernestina Angarolaa,
Corresponding author
, Verónica Andrea Peuchotb, Fernando Warleyc, Diana Inés Liberatored
a Internal Medicine Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
b Clinical Research Unit, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
c Hematology Section, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
d Immunology Section, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
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Tables (3)
Table 1. Clinical features in adults with secondary hypogammaglobulinemia (n=1012).
Table 2. Subgroup analysis according to most frequent SHG-etiological factors.
Table 3. Multivariate analysis of infections or death according to etiological factors.
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Additional material (1)
Abstract
Background and objective

IgG replacement therapy (IgG-RT) has radically changed the clinical evolution of primary immunodeficiencies, yet the information regarding secondary hypogammaglobulinemia (SHG) is insufficient or conflicting. We aim to describe clinical features, evolution and treatment of SHG patients in our center.

Methods

Dynamic retrospective cohort between January 2001 and July 2021 of adults with gamma globulin fraction <0.6g/dL in a serum protein electrophoresis and a coincident decrease of IgG levels – with a disease-related SHG or treatment that reduces serum immunoglobulins.

Results

We included 1012 patients with SHG with a median follow-up of 5 years (IQR 2–8). Hematological diseases were identified in 95% of the patients and 61% received drugs related to SHG. Sixty five percent had more than one etiological factor associated with SHG. Infectious diseases were present in 69% of the patients, 48% had respiratory infections and 17% had severe infections. There was statistical association between respiratory and severe infections with multiple myeloma (MM), lymphoma and rituximab. MGUS had less infections and death compared with other etiologies. IgG-RT was indicated in 18.7% of the patients and 4.6% received it for more than 6 months with variable intervals. Among the latter group, there was a significant reduction of all-type infections and respiratory infections with IgG-RT (p<0.001), and it was consistent with similar findings in lymphoma, MM and all IgG levels subgroups.

Conclusion

SHG was associated with more than one etiological factor and a high frequency of infections. IgG-RT indication was irregular yet still effective. It is relevant to consider IgG levels screening, monitoring and accurate indication of IgG-RT.

Keywords:
Secondary hypogammaglobulinemia
Hematologic neoplasms
Pneumonia
Transplantation
Immunosuppression
Abbreviations:
SHG
IgG-RT
MM
MGUS
CLL
SOT
HSCT
Resumen
Antecedentes y objetivos

La IgG sustitutiva ha cambiado radicalmente la evolución de las inmunodeficiencias primarias, mientras que la información sobre hipogammaglobulinemia secundaria (HGS) es insuficiente y discordante. El objetivo del estudio es describir las características clínicas, evolución y tratamiento de pacientes con HGS.

Métodos

Cohorte retrospectiva dinámica entre enero de 2001 y julio de 2021 de adultos con proteinograma y fracción de gammaglobulina <0,6g/dL y dosaje disminuido de IgG, con enfermedad o tratamiento que produzcan HGS.

Resultados

Se incluyó a 1.012 pacientes con HGS con una mediana de seguimiento de 5 años (IIC 2-8). El 95% tenía enfermedad hematológica y el 61% recibió fármacos asociados a HGS. El 65% tenía más de un factor etiológico asociado con HGS. El 69% presentó infecciones de cualquier tipo, el 48% infecciones respiratorias y el 17%, infecciones graves. Hubo asociación significativa entre infecciones respiratorias y graves entre los subgrupos de mieloma múltiple, linfoma y rituximab. Los pacientes con MGUS tuvieron menor frecuencia de infecciones y muerte comparados con otros factores etiológicos. El 18,7% de los pacientes recibió IgG sustitutiva y el 4,6% de forma crónica, con intervalos variables. Los últimos tuvieron disminución significativa de infecciones de cualquier tipo e infecciones respiratorias con IgG sustitutiva (p<0,001), que se mantuvo en los subgrupos con mieloma múltiple, linfoma y todos los niveles de IgG.

Conclusión

La HGS asoció más de un factor etiológico y alta frecuencia de infecciones. La indicación de IgG sustitutiva fue irregular, pero, aún así, efectiva. Se plantea considerar el dosaje de inmunoglobulinas, monitoreo y la adecuada indicación de IgG sustitutiva.

Palabras clave:
Hipogammaglobulinemia secundaria
Neoplasias hematológicas
Neumonía
Trasplante
Inmunosupresión

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