Session Type: ePosters
Session Title: ePosters
Authors(s): M. Torres (1), M. Santos (1), C. Saunders (2), S. Pereira (2), S. Lino (1), D. Seixas (1), E. Leal (1), C. Cruz (1), A. Dias (1), H. Pinheiro (1), A. Caeiro (1), A. Garrote (1), A. Pinto (1), J. Ramirez (1), S. Betkova (1), J. Ribeiro (1), D. Povoas (1), O. Cardoso (1), M. Manata (1), F. Maltez (1)
Authors Affiliations(s): (1) Infectious Diseases Department oh Hospital de Curry Cabral, Portugal, (2) Haematology Department oh Hospital de Curry Cabral, Portugal
Background:
Human herpesvirus 8 (HHV-8) is responsible for a spectrum of lymphoproliferative disorders including multicentric Castleman disease (MCD). This is a rare disorder usually presenting with generalized lymphadenopathy, splenomegaly and constitutional symptoms. The incidence of HHV-8-associated MCD in human immunodeficiency virus (HIV) infected patients appears to be increasing since the introduction of the antiretroviral therapy (ART). In these patients, coexistent Kaposi Sarcoma (KS) is frequent and worsens the prognosis. Treatment with rituximab and the advent of effective ART have improved the outcome.
We present a retrospective analysis of five HHV-8-associated MCD patients diagnosed for the last 10 years.
Case:
Four patients were male and the median age was 36 years old [31-65]. Three patients were diagnosed simultaneously with HIV infection. Regarding risk factors for HHV-8-associated MCD, four were non-caucasian, four had more than 33 years old, four had no previous exposure to ART and two had nadir CD4 count >200/microliter. All patients presented with lymphadenopathies and anemia, four patients with fever and two with constitutional symptoms and hepatosplenomegaly. One patient had concomitant KS with visceral involvement and was treated with a combination of doxorubicin and rituximab with good outcome; two of the three patients treated with rituximab had favourable response to treatment. Median follow-up period of these three patients was 16 months [6-30], with no signs of disease recurrence. Two patients died: one before starting treatment and the other after treatment, with an infectious complication.
Discussion:HHV-8-associated MCD has been known for its poor prognosis, with mortality rates as high as 70-85% and a median survival of 8-14 months. However, recent studies have shown a significant improvement in the outcomes of these patients, attributable to early diagnosis and treatment with rituximab. Hence, especially in an era of effective ART, this rare diagnosis should be considered in symptomatic patients with HIV, if risk factors and haematological alterations with concurrent fever, lymphadenopathies and hepatosplenomegaly are evident.
Keyword(s): Multicentric Castleman Disease, Kaposi Sarcoma, Human Immunodeficiency virusSession Type: ePosters
Session Title: ePosters
Authors(s): M. Torres (1), M. Santos (1), C. Saunders (2), S. Pereira (2), S. Lino (1), D. Seixas (1), E. Leal (1), C. Cruz (1), A. Dias (1), H. Pinheiro (1), A. Caeiro (1), A. Garrote (1), A. Pinto (1), J. Ramirez (1), S. Betkova (1), J. Ribeiro (1), D. Povoas (1), O. Cardoso (1), M. Manata (1), F. Maltez (1)
Authors Affiliations(s): (1) Infectious Diseases Department oh Hospital de Curry Cabral, Portugal, (2) Haematology Department oh Hospital de Curry Cabral, Portugal
Background:
Human herpesvirus 8 (HHV-8) is responsible for a spectrum of lymphoproliferative disorders including multicentric Castleman disease (MCD). This is a rare disorder usually presenting with generalized lymphadenopathy, splenomegaly and constitutional symptoms. The incidence of HHV-8-associated MCD in human immunodeficiency virus (HIV) infected patients appears to be increasing since the introduction of the antiretroviral therapy (ART). In these patients, coexistent Kaposi Sarcoma (KS) is frequent and worsens the prognosis. Treatment with rituximab and the advent of effective ART have improved the outcome.
We present a retrospective analysis of five HHV-8-associated MCD patients diagnosed for the last 10 years.
Case:
Four patients were male and the median age was 36 years old [31-65]. Three patients were diagnosed simultaneously with HIV infection. Regarding risk factors for HHV-8-associated MCD, four were non-caucasian, four had more than 33 years old, four had no previous exposure to ART and two had nadir CD4 count >200/microliter. All patients presented with lymphadenopathies and anemia, four patients with fever and two with constitutional symptoms and hepatosplenomegaly. One patient had concomitant KS with visceral involvement and was treated with a combination of doxorubicin and rituximab with good outcome; two of the three patients treated with rituximab had favourable response to treatment. Median follow-up period of these three patients was 16 months [6-30], with no signs of disease recurrence. Two patients died: one before starting treatment and the other after treatment, with an infectious complication.
Discussion:HHV-8-associated MCD has been known for its poor prognosis, with mortality rates as high as 70-85% and a median survival of 8-14 months. However, recent studies have shown a significant improvement in the outcomes of these patients, attributable to early diagnosis and treatment with rituximab. Hence, especially in an era of effective ART, this rare diagnosis should be considered in symptomatic patients with HIV, if risk factors and haematological alterations with concurrent fever, lymphadenopathies and hepatosplenomegaly are evident.
Keyword(s): Multicentric Castleman Disease, Kaposi Sarcoma, Human Immunodeficiency virus
Session Type: ePosters
Session Title: ePosters
Authors(s): M. Torres (1), M. Santos (1), C. Saunders (2), S. Pereira (2), S. Lino (1), D. Seixas (1), E. Leal (1), C. Cruz (1), A. Dias (1), H. Pinheiro (1), A. Caeiro (1), A. Garrote (1), A. Pinto (1), J. Ramirez (1), S. Betkova (1), J. Ribeiro (1), D. Povoas (1), O. Cardoso (1), M. Manata (1), F. Maltez (1)
Authors Affiliations(s): (1) Infectious Diseases Department oh Hospital de Curry Cabral, Portugal, (2) Haematology Department oh Hospital de Curry Cabral, Portugal
Background:
Human herpesvirus 8 (HHV-8) is responsible for a spectrum of lymphoproliferative disorders including multicentric Castleman disease (MCD). This is a rare disorder usually presenting with generalized lymphadenopathy, splenomegaly and constitutional symptoms. The incidence of HHV-8-associated MCD in human immunodeficiency virus (HIV) infected patients appears to be increasing since the introduction of the antiretroviral therapy (ART). In these patients, coexistent Kaposi Sarcoma (KS) is frequent and worsens the prognosis. Treatment with rituximab and the advent of effective ART have improved the outcome.
We present a retrospective analysis of five HHV-8-associated MCD patients diagnosed for the last 10 years.
Case:
Four patients were male and the median age was 36 years old [31-65]. Three patients were diagnosed simultaneously with HIV infection. Regarding risk factors for HHV-8-associated MCD, four were non-caucasian, four had more than 33 years old, four had no previous exposure to ART and two had nadir CD4 count >200/microliter. All patients presented with lymphadenopathies and anemia, four patients with fever and two with constitutional symptoms and hepatosplenomegaly. One patient had concomitant KS with visceral involvement and was treated with a combination of doxorubicin and rituximab with good outcome; two of the three patients treated with rituximab had favourable response to treatment. Median follow-up period of these three patients was 16 months [6-30], with no signs of disease recurrence. Two patients died: one before starting treatment and the other after treatment, with an infectious complication.
Discussion:HHV-8-associated MCD has been known for its poor prognosis, with mortality rates as high as 70-85% and a median survival of 8-14 months. However, recent studies have shown a significant improvement in the outcomes of these patients, attributable to early diagnosis and treatment with rituximab. Hence, especially in an era of effective ART, this rare diagnosis should be considered in symptomatic patients with HIV, if risk factors and haematological alterations with concurrent fever, lymphadenopathies and hepatosplenomegaly are evident.
Keyword(s): Multicentric Castleman Disease, Kaposi Sarcoma, Human Immunodeficiency virusSession Type: ePosters
Session Title: ePosters
Authors(s): M. Torres (1), M. Santos (1), C. Saunders (2), S. Pereira (2), S. Lino (1), D. Seixas (1), E. Leal (1), C. Cruz (1), A. Dias (1), H. Pinheiro (1), A. Caeiro (1), A. Garrote (1), A. Pinto (1), J. Ramirez (1), S. Betkova (1), J. Ribeiro (1), D. Povoas (1), O. Cardoso (1), M. Manata (1), F. Maltez (1)
Authors Affiliations(s): (1) Infectious Diseases Department oh Hospital de Curry Cabral, Portugal, (2) Haematology Department oh Hospital de Curry Cabral, Portugal
Background:
Human herpesvirus 8 (HHV-8) is responsible for a spectrum of lymphoproliferative disorders including multicentric Castleman disease (MCD). This is a rare disorder usually presenting with generalized lymphadenopathy, splenomegaly and constitutional symptoms. The incidence of HHV-8-associated MCD in human immunodeficiency virus (HIV) infected patients appears to be increasing since the introduction of the antiretroviral therapy (ART). In these patients, coexistent Kaposi Sarcoma (KS) is frequent and worsens the prognosis. Treatment with rituximab and the advent of effective ART have improved the outcome.
We present a retrospective analysis of five HHV-8-associated MCD patients diagnosed for the last 10 years.
Case:
Four patients were male and the median age was 36 years old [31-65]. Three patients were diagnosed simultaneously with HIV infection. Regarding risk factors for HHV-8-associated MCD, four were non-caucasian, four had more than 33 years old, four had no previous exposure to ART and two had nadir CD4 count >200/microliter. All patients presented with lymphadenopathies and anemia, four patients with fever and two with constitutional symptoms and hepatosplenomegaly. One patient had concomitant KS with visceral involvement and was treated with a combination of doxorubicin and rituximab with good outcome; two of the three patients treated with rituximab had favourable response to treatment. Median follow-up period of these three patients was 16 months [6-30], with no signs of disease recurrence. Two patients died: one before starting treatment and the other after treatment, with an infectious complication.
Discussion:HHV-8-associated MCD has been known for its poor prognosis, with mortality rates as high as 70-85% and a median survival of 8-14 months. However, recent studies have shown a significant improvement in the outcomes of these patients, attributable to early diagnosis and treatment with rituximab. Hence, especially in an era of effective ART, this rare diagnosis should be considered in symptomatic patients with HIV, if risk factors and haematological alterations with concurrent fever, lymphadenopathies and hepatosplenomegaly are evident.
Keyword(s): Multicentric Castleman Disease, Kaposi Sarcoma, Human Immunodeficiency virus