Tonsillar granuloma associated with hypogammaglobulinemia

Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies. Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Laajala, Aleksi [verfasserIn] Kuismin, Outi [verfasserIn] Tastula, Mikko [verfasserIn] Tiitto, Leena [verfasserIn] Kauppila, Saila [verfasserIn] Salo, Anna [verfasserIn] Åström, Pirjo [verfasserIn] Nissinen, Antti [verfasserIn] Glumoff, Virpi [verfasserIn] Seppänen, Mikko R. J. [verfasserIn] Hautala, Timo [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2020

Schlagwörter:	Granuloma Hypogammaglobulinemia Rituximab Sarcoidosis

Übergeordnetes Werk:	Enthalten in: Allergy, asthma and clinical immunology - London : BioMed Central, 2005, 16(2020), 1 vom: 29. Mai
Übergeordnetes Werk:	volume:16 ; year:2020 ; number:1 ; day:29 ; month:05

Links:	Volltext

DOI / URN:	10.1186/s13223-020-00441-1

Katalog-ID:	SPR039869490

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520			\|a Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.
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allfields	10.1186/s13223-020-00441-1 doi (DE-627)SPR039869490 (SPR)s13223-020-00441-1-e DE-627 ger DE-627 rakwb eng 610 ASE Laajala, Aleksi verfasserin aut Tonsillar granuloma associated with hypogammaglobulinemia 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies. Granuloma (dpeaa)DE-He213 Hypogammaglobulinemia (dpeaa)DE-He213 Rituximab (dpeaa)DE-He213 Sarcoidosis (dpeaa)DE-He213 Kuismin, Outi verfasserin aut Tastula, Mikko verfasserin aut Tiitto, Leena verfasserin aut Kauppila, Saila verfasserin aut Salo, Anna verfasserin aut Åström, Pirjo verfasserin aut Nissinen, Antti verfasserin aut Glumoff, Virpi verfasserin aut Seppänen, Mikko R. J. verfasserin aut Hautala, Timo verfasserin aut Enthalten in Allergy, asthma and clinical immunology London : BioMed Central, 2005 16(2020), 1 vom: 29. Mai (DE-627)512662487 (DE-600)2236671-4 1710-1492 nnns volume:16 year:2020 number:1 day:29 month:05 https://dx.doi.org/10.1186/s13223-020-00441-1 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2020 1 29 05
spelling	10.1186/s13223-020-00441-1 doi (DE-627)SPR039869490 (SPR)s13223-020-00441-1-e DE-627 ger DE-627 rakwb eng 610 ASE Laajala, Aleksi verfasserin aut Tonsillar granuloma associated with hypogammaglobulinemia 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies. Granuloma (dpeaa)DE-He213 Hypogammaglobulinemia (dpeaa)DE-He213 Rituximab (dpeaa)DE-He213 Sarcoidosis (dpeaa)DE-He213 Kuismin, Outi verfasserin aut Tastula, Mikko verfasserin aut Tiitto, Leena verfasserin aut Kauppila, Saila verfasserin aut Salo, Anna verfasserin aut Åström, Pirjo verfasserin aut Nissinen, Antti verfasserin aut Glumoff, Virpi verfasserin aut Seppänen, Mikko R. J. verfasserin aut Hautala, Timo verfasserin aut Enthalten in Allergy, asthma and clinical immunology London : BioMed Central, 2005 16(2020), 1 vom: 29. Mai (DE-627)512662487 (DE-600)2236671-4 1710-1492 nnns volume:16 year:2020 number:1 day:29 month:05 https://dx.doi.org/10.1186/s13223-020-00441-1 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2020 1 29 05
allfields_unstemmed	10.1186/s13223-020-00441-1 doi (DE-627)SPR039869490 (SPR)s13223-020-00441-1-e DE-627 ger DE-627 rakwb eng 610 ASE Laajala, Aleksi verfasserin aut Tonsillar granuloma associated with hypogammaglobulinemia 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies. Granuloma (dpeaa)DE-He213 Hypogammaglobulinemia (dpeaa)DE-He213 Rituximab (dpeaa)DE-He213 Sarcoidosis (dpeaa)DE-He213 Kuismin, Outi verfasserin aut Tastula, Mikko verfasserin aut Tiitto, Leena verfasserin aut Kauppila, Saila verfasserin aut Salo, Anna verfasserin aut Åström, Pirjo verfasserin aut Nissinen, Antti verfasserin aut Glumoff, Virpi verfasserin aut Seppänen, Mikko R. J. verfasserin aut Hautala, Timo verfasserin aut Enthalten in Allergy, asthma and clinical immunology London : BioMed Central, 2005 16(2020), 1 vom: 29. Mai (DE-627)512662487 (DE-600)2236671-4 1710-1492 nnns volume:16 year:2020 number:1 day:29 month:05 https://dx.doi.org/10.1186/s13223-020-00441-1 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2020 1 29 05
allfieldsGer	10.1186/s13223-020-00441-1 doi (DE-627)SPR039869490 (SPR)s13223-020-00441-1-e DE-627 ger DE-627 rakwb eng 610 ASE Laajala, Aleksi verfasserin aut Tonsillar granuloma associated with hypogammaglobulinemia 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies. Granuloma (dpeaa)DE-He213 Hypogammaglobulinemia (dpeaa)DE-He213 Rituximab (dpeaa)DE-He213 Sarcoidosis (dpeaa)DE-He213 Kuismin, Outi verfasserin aut Tastula, Mikko verfasserin aut Tiitto, Leena verfasserin aut Kauppila, Saila verfasserin aut Salo, Anna verfasserin aut Åström, Pirjo verfasserin aut Nissinen, Antti verfasserin aut Glumoff, Virpi verfasserin aut Seppänen, Mikko R. J. verfasserin aut Hautala, Timo verfasserin aut Enthalten in Allergy, asthma and clinical immunology London : BioMed Central, 2005 16(2020), 1 vom: 29. Mai (DE-627)512662487 (DE-600)2236671-4 1710-1492 nnns volume:16 year:2020 number:1 day:29 month:05 https://dx.doi.org/10.1186/s13223-020-00441-1 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2020 1 29 05
allfieldsSound	10.1186/s13223-020-00441-1 doi (DE-627)SPR039869490 (SPR)s13223-020-00441-1-e DE-627 ger DE-627 rakwb eng 610 ASE Laajala, Aleksi verfasserin aut Tonsillar granuloma associated with hypogammaglobulinemia 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies. Granuloma (dpeaa)DE-He213 Hypogammaglobulinemia (dpeaa)DE-He213 Rituximab (dpeaa)DE-He213 Sarcoidosis (dpeaa)DE-He213 Kuismin, Outi verfasserin aut Tastula, Mikko verfasserin aut Tiitto, Leena verfasserin aut Kauppila, Saila verfasserin aut Salo, Anna verfasserin aut Åström, Pirjo verfasserin aut Nissinen, Antti verfasserin aut Glumoff, Virpi verfasserin aut Seppänen, Mikko R. J. verfasserin aut Hautala, Timo verfasserin aut Enthalten in Allergy, asthma and clinical immunology London : BioMed Central, 2005 16(2020), 1 vom: 29. Mai (DE-627)512662487 (DE-600)2236671-4 1710-1492 nnns volume:16 year:2020 number:1 day:29 month:05 https://dx.doi.org/10.1186/s13223-020-00441-1 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2020 1 29 05
language	English
source	Enthalten in Allergy, asthma and clinical immunology 16(2020), 1 vom: 29. Mai volume:16 year:2020 number:1 day:29 month:05
sourceStr	Enthalten in Allergy, asthma and clinical immunology 16(2020), 1 vom: 29. Mai volume:16 year:2020 number:1 day:29 month:05
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Granuloma Hypogammaglobulinemia Rituximab Sarcoidosis
dewey-raw	610
isfreeaccess_bool	true
container_title	Allergy, asthma and clinical immunology
authorswithroles_txt_mv	Laajala, Aleksi @@aut@@ Kuismin, Outi @@aut@@ Tastula, Mikko @@aut@@ Tiitto, Leena @@aut@@ Kauppila, Saila @@aut@@ Salo, Anna @@aut@@ Åström, Pirjo @@aut@@ Nissinen, Antti @@aut@@ Glumoff, Virpi @@aut@@ Seppänen, Mikko R. J. @@aut@@ Hautala, Timo @@aut@@
publishDateDaySort_date	2020-05-29T00:00:00Z
hierarchy_top_id	512662487
dewey-sort	3610
id	SPR039869490
language_de	englisch
fullrecord	<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR039869490</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230519172006.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">201007s2020 xx \|\|\|\|\|o 00\| \|\|eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1186/s13223-020-00441-1</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR039869490</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)s13223-020-00441-1-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="082" ind1="0" ind2="4"><subfield code="a">610</subfield><subfield code="q">ASE</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Laajala, Aleksi</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Tonsillar granuloma associated with hypogammaglobulinemia</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2020</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. 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author	Laajala, Aleksi
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topic_title	610 ASE Tonsillar granuloma associated with hypogammaglobulinemia Granuloma (dpeaa)DE-He213 Hypogammaglobulinemia (dpeaa)DE-He213 Rituximab (dpeaa)DE-He213 Sarcoidosis (dpeaa)DE-He213
topic	ddc 610 misc Granuloma misc Hypogammaglobulinemia misc Rituximab misc Sarcoidosis
topic_unstemmed	ddc 610 misc Granuloma misc Hypogammaglobulinemia misc Rituximab misc Sarcoidosis
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title	Tonsillar granuloma associated with hypogammaglobulinemia
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title_full	Tonsillar granuloma associated with hypogammaglobulinemia
author_sort	Laajala, Aleksi
journal	Allergy, asthma and clinical immunology
journalStr	Allergy, asthma and clinical immunology
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author_browse	Laajala, Aleksi Kuismin, Outi Tastula, Mikko Tiitto, Leena Kauppila, Saila Salo, Anna Åström, Pirjo Nissinen, Antti Glumoff, Virpi Seppänen, Mikko R. J. Hautala, Timo
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author2-role	verfasserin
title_sort	tonsillar granuloma associated with hypogammaglobulinemia
title_auth	Tonsillar granuloma associated with hypogammaglobulinemia
abstract	Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.
abstractGer	Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.
abstract_unstemmed	Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.
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title_short	Tonsillar granuloma associated with hypogammaglobulinemia
url	https://dx.doi.org/10.1186/s13223-020-00441-1
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author2	Kuismin, Outi Tastula, Mikko Tiitto, Leena Kauppila, Saila Salo, Anna Åström, Pirjo Nissinen, Antti Glumoff, Virpi Seppänen, Mikko R. J. Hautala, Timo
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Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of $ CD27^{+} $ memory B cells (30.3%) and $ IgD^{−} %$ IgM^{−} %$ CD27^{+} $ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated $ CD21^{low} $ B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with $ Pneumovax^{®} $. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. 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Tonsillar granuloma associated with hypogammaglobulinemia

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