LYMPHADENOPATHY WITH ELEVATED IGG4 PLASMA CELLS IN CHILDHOOD: A POSSIBLE ASSOCIATION WITH MALIGNANCIES OR IMMUNE MEDIATED DISEASES
(Abstract release date: 05/19/16)
EHA Library. Boaro M. 06/09/16; 134756; PB1856
Dr. Maria Paola Boaro
Contributions
Contributions
Abstract
Abstract: PB1856
Type: Publication Only
Background
IgG4 related disease (RD) is a fibroinflammatory condition, identified in 2003, that can involve every organ and apparatus. The diagnostic criteria for IgG4 (RD) are based on three characteristics of the disease: organ enlargement or nodular lesions in different organs, increase of serum IgG4 and infiltration of IgG4 plasma cells. The most frequent disease manifestations are autoimmune pancreatitis, sialadenitis, dacryoadenitis and retro-peritoneal fibrosis; IgG4 related lymphadenopathy is rare. Differential diagnosis of IgG4 RD are malignancies and autoinflammatory diseases. It is usually diagnosed in middle and senile aged men; only few pediatric cases in literature are described, and the organs involved are orbits, lungs, mediastinum and abdomen. Lymphadenopathy of IgG4 (RD) is often the first manifestation of the disease, that could progress and involve any organ; a disease localized only in lymph nodes is rare and poorely characterized. No pediatric cases of IgG4 related lymphadenopathy or association with malignancies or other immune-mediated disorder are described in literature.
Aims
To describe for the first time two pediatric cases of lymph node histological pattern of IgG4 (RD) with association with lymphoma and immune-mediated disease.
Methods
Data of the two pediatric patients were retrospectively reviewed. The specimens for the histological analysis were centrally reviewed.
Results
Data of two patients with a histological pattern of IgG4 lymphadenopathy were analyzed. Both of them had a history of persistent lymphoadenopathy. Patient #1, a 11 years old male, with normal serum IgG4 concentration, had a first cervical lymph node biopsy with the evidence of progressive transformation of germinal centers and a high number of IgG4 plasma cells; two months later, for increasing of lymph nodes dimension, he had a second biopsy and the diagnosis of nodular lymphocyte predominant Hodgkin Lymphoma (HL) was made. He was treated with three cycles of prednison, vinblastine and cyclophosphamide, obtaining a complete remission (follow-up: 18 months). Patient #2, a 18 years old male, with a personal history of asthma and atopic dermatitis, had a persistent neck lymphadenopathy after 8 years from an infectious mononucleosis. He had high serum IgG4 concentration. Histology showed follicular hyperplasia and increased IgG4 plasma cells in germinal centers. Following analysis evidenced elevated (10%) double negative TCR alpha-beta positive T cells, suggesting the diagnosis of Autoimmune Lymphoproliferative Syndrome (ALPS). Fas-mediated apoptosis assay is currently ongoing.
Conclusion
We described for the first time 2 pediatric cases with with histological lymph node characteristics usually found in IgG4 (RD); in particular in one patient this finding was associated with a nodular lymphocyte predominant HL, in another patient with clinical and hematological features suggestive of ALPS. The significance of this histological finding in adult patients with isolated lymphadenopathy is still unclear and rare correlations with lymphomas and with autoimmune diseases are reported. Our data suggest that also in childhood this pattern is not specific for IgG4 RD but may be present in different malignant and non malignant disorders. A careful throughout examination and follow-up should be carried out in patients affected by IgG4 RD or with lymph node biopsy showing the IgG4 features, to monitor the possible development of lymphoma and other immune mediated diseases.
Session topic: E-poster
Keyword(s): Autoimmune disease, Immunoglobulin, Lymphoma
Type: Publication Only
Background
IgG4 related disease (RD) is a fibroinflammatory condition, identified in 2003, that can involve every organ and apparatus. The diagnostic criteria for IgG4 (RD) are based on three characteristics of the disease: organ enlargement or nodular lesions in different organs, increase of serum IgG4 and infiltration of IgG4 plasma cells. The most frequent disease manifestations are autoimmune pancreatitis, sialadenitis, dacryoadenitis and retro-peritoneal fibrosis; IgG4 related lymphadenopathy is rare. Differential diagnosis of IgG4 RD are malignancies and autoinflammatory diseases. It is usually diagnosed in middle and senile aged men; only few pediatric cases in literature are described, and the organs involved are orbits, lungs, mediastinum and abdomen. Lymphadenopathy of IgG4 (RD) is often the first manifestation of the disease, that could progress and involve any organ; a disease localized only in lymph nodes is rare and poorely characterized. No pediatric cases of IgG4 related lymphadenopathy or association with malignancies or other immune-mediated disorder are described in literature.
Aims
To describe for the first time two pediatric cases of lymph node histological pattern of IgG4 (RD) with association with lymphoma and immune-mediated disease.
Methods
Data of the two pediatric patients were retrospectively reviewed. The specimens for the histological analysis were centrally reviewed.
Results
Data of two patients with a histological pattern of IgG4 lymphadenopathy were analyzed. Both of them had a history of persistent lymphoadenopathy. Patient #1, a 11 years old male, with normal serum IgG4 concentration, had a first cervical lymph node biopsy with the evidence of progressive transformation of germinal centers and a high number of IgG4 plasma cells; two months later, for increasing of lymph nodes dimension, he had a second biopsy and the diagnosis of nodular lymphocyte predominant Hodgkin Lymphoma (HL) was made. He was treated with three cycles of prednison, vinblastine and cyclophosphamide, obtaining a complete remission (follow-up: 18 months). Patient #2, a 18 years old male, with a personal history of asthma and atopic dermatitis, had a persistent neck lymphadenopathy after 8 years from an infectious mononucleosis. He had high serum IgG4 concentration. Histology showed follicular hyperplasia and increased IgG4 plasma cells in germinal centers. Following analysis evidenced elevated (10%) double negative TCR alpha-beta positive T cells, suggesting the diagnosis of Autoimmune Lymphoproliferative Syndrome (ALPS). Fas-mediated apoptosis assay is currently ongoing.
Conclusion
We described for the first time 2 pediatric cases with with histological lymph node characteristics usually found in IgG4 (RD); in particular in one patient this finding was associated with a nodular lymphocyte predominant HL, in another patient with clinical and hematological features suggestive of ALPS. The significance of this histological finding in adult patients with isolated lymphadenopathy is still unclear and rare correlations with lymphomas and with autoimmune diseases are reported. Our data suggest that also in childhood this pattern is not specific for IgG4 RD but may be present in different malignant and non malignant disorders. A careful throughout examination and follow-up should be carried out in patients affected by IgG4 RD or with lymph node biopsy showing the IgG4 features, to monitor the possible development of lymphoma and other immune mediated diseases.
Session topic: E-poster
Keyword(s): Autoimmune disease, Immunoglobulin, Lymphoma
Abstract: PB1856
Type: Publication Only
Background
IgG4 related disease (RD) is a fibroinflammatory condition, identified in 2003, that can involve every organ and apparatus. The diagnostic criteria for IgG4 (RD) are based on three characteristics of the disease: organ enlargement or nodular lesions in different organs, increase of serum IgG4 and infiltration of IgG4 plasma cells. The most frequent disease manifestations are autoimmune pancreatitis, sialadenitis, dacryoadenitis and retro-peritoneal fibrosis; IgG4 related lymphadenopathy is rare. Differential diagnosis of IgG4 RD are malignancies and autoinflammatory diseases. It is usually diagnosed in middle and senile aged men; only few pediatric cases in literature are described, and the organs involved are orbits, lungs, mediastinum and abdomen. Lymphadenopathy of IgG4 (RD) is often the first manifestation of the disease, that could progress and involve any organ; a disease localized only in lymph nodes is rare and poorely characterized. No pediatric cases of IgG4 related lymphadenopathy or association with malignancies or other immune-mediated disorder are described in literature.
Aims
To describe for the first time two pediatric cases of lymph node histological pattern of IgG4 (RD) with association with lymphoma and immune-mediated disease.
Methods
Data of the two pediatric patients were retrospectively reviewed. The specimens for the histological analysis were centrally reviewed.
Results
Data of two patients with a histological pattern of IgG4 lymphadenopathy were analyzed. Both of them had a history of persistent lymphoadenopathy. Patient #1, a 11 years old male, with normal serum IgG4 concentration, had a first cervical lymph node biopsy with the evidence of progressive transformation of germinal centers and a high number of IgG4 plasma cells; two months later, for increasing of lymph nodes dimension, he had a second biopsy and the diagnosis of nodular lymphocyte predominant Hodgkin Lymphoma (HL) was made. He was treated with three cycles of prednison, vinblastine and cyclophosphamide, obtaining a complete remission (follow-up: 18 months). Patient #2, a 18 years old male, with a personal history of asthma and atopic dermatitis, had a persistent neck lymphadenopathy after 8 years from an infectious mononucleosis. He had high serum IgG4 concentration. Histology showed follicular hyperplasia and increased IgG4 plasma cells in germinal centers. Following analysis evidenced elevated (10%) double negative TCR alpha-beta positive T cells, suggesting the diagnosis of Autoimmune Lymphoproliferative Syndrome (ALPS). Fas-mediated apoptosis assay is currently ongoing.
Conclusion
We described for the first time 2 pediatric cases with with histological lymph node characteristics usually found in IgG4 (RD); in particular in one patient this finding was associated with a nodular lymphocyte predominant HL, in another patient with clinical and hematological features suggestive of ALPS. The significance of this histological finding in adult patients with isolated lymphadenopathy is still unclear and rare correlations with lymphomas and with autoimmune diseases are reported. Our data suggest that also in childhood this pattern is not specific for IgG4 RD but may be present in different malignant and non malignant disorders. A careful throughout examination and follow-up should be carried out in patients affected by IgG4 RD or with lymph node biopsy showing the IgG4 features, to monitor the possible development of lymphoma and other immune mediated diseases.
Session topic: E-poster
Keyword(s): Autoimmune disease, Immunoglobulin, Lymphoma
Type: Publication Only
Background
IgG4 related disease (RD) is a fibroinflammatory condition, identified in 2003, that can involve every organ and apparatus. The diagnostic criteria for IgG4 (RD) are based on three characteristics of the disease: organ enlargement or nodular lesions in different organs, increase of serum IgG4 and infiltration of IgG4 plasma cells. The most frequent disease manifestations are autoimmune pancreatitis, sialadenitis, dacryoadenitis and retro-peritoneal fibrosis; IgG4 related lymphadenopathy is rare. Differential diagnosis of IgG4 RD are malignancies and autoinflammatory diseases. It is usually diagnosed in middle and senile aged men; only few pediatric cases in literature are described, and the organs involved are orbits, lungs, mediastinum and abdomen. Lymphadenopathy of IgG4 (RD) is often the first manifestation of the disease, that could progress and involve any organ; a disease localized only in lymph nodes is rare and poorely characterized. No pediatric cases of IgG4 related lymphadenopathy or association with malignancies or other immune-mediated disorder are described in literature.
Aims
To describe for the first time two pediatric cases of lymph node histological pattern of IgG4 (RD) with association with lymphoma and immune-mediated disease.
Methods
Data of the two pediatric patients were retrospectively reviewed. The specimens for the histological analysis were centrally reviewed.
Results
Data of two patients with a histological pattern of IgG4 lymphadenopathy were analyzed. Both of them had a history of persistent lymphoadenopathy. Patient #1, a 11 years old male, with normal serum IgG4 concentration, had a first cervical lymph node biopsy with the evidence of progressive transformation of germinal centers and a high number of IgG4 plasma cells; two months later, for increasing of lymph nodes dimension, he had a second biopsy and the diagnosis of nodular lymphocyte predominant Hodgkin Lymphoma (HL) was made. He was treated with three cycles of prednison, vinblastine and cyclophosphamide, obtaining a complete remission (follow-up: 18 months). Patient #2, a 18 years old male, with a personal history of asthma and atopic dermatitis, had a persistent neck lymphadenopathy after 8 years from an infectious mononucleosis. He had high serum IgG4 concentration. Histology showed follicular hyperplasia and increased IgG4 plasma cells in germinal centers. Following analysis evidenced elevated (10%) double negative TCR alpha-beta positive T cells, suggesting the diagnosis of Autoimmune Lymphoproliferative Syndrome (ALPS). Fas-mediated apoptosis assay is currently ongoing.
Conclusion
We described for the first time 2 pediatric cases with with histological lymph node characteristics usually found in IgG4 (RD); in particular in one patient this finding was associated with a nodular lymphocyte predominant HL, in another patient with clinical and hematological features suggestive of ALPS. The significance of this histological finding in adult patients with isolated lymphadenopathy is still unclear and rare correlations with lymphomas and with autoimmune diseases are reported. Our data suggest that also in childhood this pattern is not specific for IgG4 RD but may be present in different malignant and non malignant disorders. A careful throughout examination and follow-up should be carried out in patients affected by IgG4 RD or with lymph node biopsy showing the IgG4 features, to monitor the possible development of lymphoma and other immune mediated diseases.
Session topic: E-poster
Keyword(s): Autoimmune disease, Immunoglobulin, Lymphoma
{{ help_message }}
{{filter}}