Choroba castlemana
Rokowania, prognozy i postęp choroby
Choroba Castlemana to rzadkie zaburzenie limfoproliferacyjne o zróżnicowanym rokowaniu zależnym od podtypu. Jednoogniskowa choroba Castlemana (UCD) cechuje się doskonałym rokowaniem, z 10-letnim wskaźnikiem przeżycia po całkowitej resekcji przekraczającym 95% oraz 5-letnim wskaźnikiem przeżycia na poziomie 93,6%. W przypadku niecałkowitej resekcji, przeżycie po 20 miesiącach od radioterapii wynosi około 82%. UCD zwykle nie wpływa na oczekiwaną długość życia, jednak należy monitorować ryzyko rozwoju pęcherzycy paraneoplastycznej (PNP), które występuje u ≤5% pacjentów i jest potencjalnie śmiertelne. Występowanie transformacji UCD w wieloogniskową chorobę Castlemana (MCD) HHV-8-negatywną nie zostało nigdy odnotowane.
Prognozy w chorobie Castlemana
Choroba Castlemana stanowi rzadkie zaburzenie limfoproliferacyjne, którego rokowanie różni się znacząco w zależności od podtypu. Perspektywy dla pacjentów mogą być bardzo zróżnicowane – od doskonałego rokowania w postaci jednoogniskowej, do znacznie gorszego w przypadku postaci wieloogniskowej.12
Rokowanie w jednoogniskowej chorobie Castlemana (UCD)
Jednoogniskowa choroba Castlemana (UCD) charakteryzuje się doskonałym rokowaniem. Wskaźnik 10-letniego przeżycia po całkowitej resekcji przekracza 95%. W przypadkach, gdy całkowita resekcja nie jest możliwa, wskaźnik przeżycia po 20 miesiącach od radioterapii wynosi około 82%. Generalnie, niecałkowita resekcja wiąże się z gorszymi wynikami leczenia.3 Średnia długość przeżycia po diagnozie UCD przekracza 10 lat, a oczekiwana długość życia zwykle nie ulega zmianie w wyniku choroby.4 Pięcioletni wskaźnik przeżycia dla pacjentów z UCD wynosi 93,6%.5
Dla większości osób z UCD perspektywy są dobre. Usunięcie zajętego węzła chłonnego często wystarcza do wyleczenia. Przy odpowiednim leczeniu, UCD zwykle nie wpływa na oczekiwaną długość życia.6
Potencjalne powikłania UCD
Rozwój chłoniaka i/lub pęcherzycy paraneoplastycznej (PNP) to dwa rzadkie schorzenia współistniejące, które mogą być śmiertelne dla pacjentów z UCD. Chociaż ryzyko rozwoju PNP jest niskie (≤5%), jest to najbardziej śmiertelne potencjalne powikłanie i powinno być oceniane u wszystkich pacjentów z UCD w momencie diagnozy oraz po niej, aby zapewnić szybkie rozpoczęcie leczenia, jeśli wystąpi. Leczenie PNP często obejmuje chemioterapię i leki immunosupresyjne.7
Warto podkreślić, że nigdy nie odnotowano przypadków UCD, które przekształciłyby się w wieloogniskową chorobę Castlemana (MCD) HHV-8-negatywną.8
Rokowanie w wieloogniskowej chorobie Castlemana (MCD)
Wieloogniskowa choroba Castlemana (MCD) ma znacznie gorsze rokowanie niż UCD. Pięcioletni wskaźnik przeżycia dla pacjentów z MCD wynosi około 51,2%.9 Wyniki dla pacjentów z MCD różnią się w zależności od etiologii – HHV-8-związana lub idiopatyczna (iMCD).10
Rokowanie w MCD związanej z HHV-8
MCD związana z HHV-8 historycznie wiązała się ze złym rokowaniem, przy czym większość pacjentów umierała w ciągu 2 lat od diagnozy. Jednakże, pacjenci mają dobre rokowanie, gdy są leczeni rytuksymabem, z ponad 94% wskaźnikiem przeżycia 2-letniego. U pacjentów, którzy nie reagują na sam rytuksymab, można dodać chemioterapeutyki, takie jak doksorubicyna i etopozyd, w celu kontrolowania choroby.11
Rokowanie w idiopatycznej MCD (iMCD)
Rokowanie dla pacjentów z idiopatyczną MCD (iMCD) jest gorsze niż dla pacjentów z MCD związaną z HHV-8. W badaniach przeprowadzonych przed wprowadzeniem terapii anty-IL-6, około 65% pacjentów przeżywało 5 lat po diagnozie.12 Badanie z 2021 roku wykazało 75% wskaźnik 5-letniego przeżycia całkowitego wśród pacjentów z iMCD.1314
Ogólne rokowanie w iMCD jest niekorzystne: około 23-45% pacjentów umiera w ciągu 5 lat od diagnozy, a około 60% umiera w ciągu 10 lat.15 Jednak istnieje uzasadniona nadzieja, że wskaźniki przeżycia są obecnie znacznie lepsze dzięki nowym przełomowym metodom leczenia (takim jak blokada IL-6).1617
Czynniki prognostyczne w MCD
Analiza wieloczynnikowa przy użyciu modelu regresji Coxa ujawniła, że wiek, splenomegalia i poziom albuminy w surowicy przed leczeniem są niezależnymi czynnikami prognostycznymi dla całkowitego przeżycia w MCD.18 Starszy wiek (≥40 lat), splenomegalia i hipoalbuminemia były czynnikami ryzyka gorszego rokowania w MCD.1920
Model prognostyczny dla iMCD
Międzynarodowe Konsorcjum ds. Choroby Castlemana niedawno przedstawiło pierwszy model prognostyczny dla idiopatycznej wieloogniskowej choroby Castlemana (iMCD), korelujący z wynikiem klinicznym. Wartość predykcyjna tego indeksu (iMCD-IPI) została potwierdzona w dużej kohorcie walidacyjnej składającej się z 197 pacjentów z iMCD, zorganizowanej przez Międzynarodowe Konsorcjum ds. Choroby Castlemana. Proponowany nowy model jest cenny dla przewidywania wyniku klinicznego i wyboru optymalnych terapii przy użyciu parametrów klinicznych.21
Jakość życia i chorobowe obciążenie
Badanie rejestru ACCELERATE wykazało, że pacjenci z MCD HHV-8-negatywną (idiopatyczną) mogą doświadczać znacznego obciążenia chorobą i pogorszonej jakości życia. Ciężka zachorowalność związana z chorobą może obejmować ostrą niewydolność nerek, sepsę i zapalenie płuc, wśród innych komplikacji.22
Perspektywy dla osób z MCD zależą od typu MCD i jego nasilenia. U niektórych osób występują intensywne zaostrzenia objawów, które mogą zagrażać życiu. Inni mają bardziej przewlekłe objawy. Od 65% do 75% osób zdiagnozowanych z MCD żyje jeszcze pięć lat później. Dzięki przełomowym terapiom istnieje nadzieja, że perspektywy będą nadal się poprawiać.23
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Materiały źródłowe
- #1 Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5765290/
Castleman disease (CD) is a rare lymphoproliferative disorder. […] The 5-year overall survival (OS) of 185 CD cases was 80.3%. […] The outcome for UCD patients was better than MCD patients, with 5-year OS rates of 93.6% and 51.2%, respectively. […] In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. […] This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis. […] The log-rank test for OS showed a statistically significant difference between UCD and MCD patients (P < .001).
- #2 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
The prognosis of Castleman disease varies, depending on the subtype. […] Unicentric Castleman disease (UCD) carries an excellent prognosis. The reported 10-year survival rate after complete resection is more than 95%. […] In unresectable cases, the reported survival at 20 months after radiotherapy is 82%. […] Overall, incomplete resection is associated with poorer outcomes. […] Development of lymphoma and/or paraneoplastic pemphigus (PNP) are two rare comorbidities that can be deadly for UCD patients. […] Though the risk of developing (PNP) is low ( 5%), it is the most deadly potential complication and should be evaluated in all UCD patients at the time of diagnosis and following diagnosis to ensure that treatment is rapidly initiated if it presents. […] Treatment of PNP often includes chemotherapy and immunosuppressive drugs.
- #3 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
The prognosis of Castleman disease varies, depending on the subtype. […] Unicentric Castleman disease (UCD) carries an excellent prognosis. The reported 10-year survival rate after complete resection is more than 95%. […] In unresectable cases, the reported survival at 20 months after radiotherapy is 82%. […] Overall, incomplete resection is associated with poorer outcomes. […] Development of lymphoma and/or paraneoplastic pemphigus (PNP) are two rare comorbidities that can be deadly for UCD patients. […] Though the risk of developing (PNP) is low ( 5%), it is the most deadly potential complication and should be evaluated in all UCD patients at the time of diagnosis and following diagnosis to ensure that treatment is rapidly initiated if it presents. […] Treatment of PNP often includes chemotherapy and immunosuppressive drugs.
- #4 Prognosis – Castleman Disease Collaborative Network | CDCNhttps://cdcn.org/castleman-disease/prognosis/
The average length of survival after UCD diagnosis is greater than 10 years, and life expectancy is usually not changed by UCD. […] The 5-year overall survival rate of HIV negative (presumed HHV8-negative) MCD was 65% in a 2012 case series published prior to the approval of anti-IL-6 therapy. A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as anti-IL6 blockade).
- #5 Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5765290/
Castleman disease (CD) is a rare lymphoproliferative disorder. […] The 5-year overall survival (OS) of 185 CD cases was 80.3%. […] The outcome for UCD patients was better than MCD patients, with 5-year OS rates of 93.6% and 51.2%, respectively. […] In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. […] This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis. […] The log-rank test for OS showed a statistically significant difference between UCD and MCD patients (P < .001).
- #6 Castleman Disease: Types, Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/17920-castleman-disease
The prognosis (outlook) is good for most people with UCD. Removing the affected lymph node is often enough to cure it. With treatment, UCD doesn’t usually affect life expectancy. […] The outlook for people with MCD depends on the type of MCD and how serious it is. Some people have intense symptom flare-ups that can be life-threatening. Others have more chronic symptoms. Sixty-five percent to 75% of people diagnosed with MCD are still alive five years later. With breakthrough therapies, there’s hope that the outlook will continue to improve.
- #7 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
The prognosis of Castleman disease varies, depending on the subtype. […] Unicentric Castleman disease (UCD) carries an excellent prognosis. The reported 10-year survival rate after complete resection is more than 95%. […] In unresectable cases, the reported survival at 20 months after radiotherapy is 82%. […] Overall, incomplete resection is associated with poorer outcomes. […] Development of lymphoma and/or paraneoplastic pemphigus (PNP) are two rare comorbidities that can be deadly for UCD patients. […] Though the risk of developing (PNP) is low ( 5%), it is the most deadly potential complication and should be evaluated in all UCD patients at the time of diagnosis and following diagnosis to ensure that treatment is rapidly initiated if it presents. […] Treatment of PNP often includes chemotherapy and immunosuppressive drugs.
- #8 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #9 Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5765290/
Castleman disease (CD) is a rare lymphoproliferative disorder. […] The 5-year overall survival (OS) of 185 CD cases was 80.3%. […] The outcome for UCD patients was better than MCD patients, with 5-year OS rates of 93.6% and 51.2%, respectively. […] In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. […] This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis. […] The log-rank test for OS showed a statistically significant difference between UCD and MCD patients (P < .001).
- #10 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #11 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #12 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #13 Prognosis – Castleman Disease Collaborative Network | CDCNhttps://cdcn.org/castleman-disease/prognosis/
The average length of survival after UCD diagnosis is greater than 10 years, and life expectancy is usually not changed by UCD. […] The 5-year overall survival rate of HIV negative (presumed HHV8-negative) MCD was 65% in a 2012 case series published prior to the approval of anti-IL-6 therapy. A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as anti-IL6 blockade).
- #14 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #15 Predictive Model for Idiopathic Multicentric Castleman Disease Supporting Treatment Decisionshttps://pmc.ncbi.nlm.nih.gov/articles/PMC7794201/
The International Castleman Disease Consortium recently presented the first idiopathic multicentric Castleman disease prognostic model correlating with clinical outcome. […] The overall prognosis of iMCD is poor: approximately 23%45% patients die within 5years of diagnosis, and approximately 60% die within 10years. […] The predictive value of this iMCDIPI was confirmed in a large validation cohort of 197 patients with iMCD organized from The International Castleman Disease Consortium. […] The proposed novel model is valuable for predicting clinical outcome and selecting optimal therapies using clinical parameters.
- #16 Prognosis – Castleman Disease Collaborative Network | CDCNhttps://cdcn.org/castleman-disease/prognosis/
The average length of survival after UCD diagnosis is greater than 10 years, and life expectancy is usually not changed by UCD. […] The 5-year overall survival rate of HIV negative (presumed HHV8-negative) MCD was 65% in a 2012 case series published prior to the approval of anti-IL-6 therapy. A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as anti-IL6 blockade).
- #17 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #18 Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5765290/
Castleman disease (CD) is a rare lymphoproliferative disorder. […] The 5-year overall survival (OS) of 185 CD cases was 80.3%. […] The outcome for UCD patients was better than MCD patients, with 5-year OS rates of 93.6% and 51.2%, respectively. […] In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. […] This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis. […] The log-rank test for OS showed a statistically significant difference between UCD and MCD patients (P < .001).
- #19 Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5765290/
Castleman disease (CD) is a rare lymphoproliferative disorder. […] The 5-year overall survival (OS) of 185 CD cases was 80.3%. […] The outcome for UCD patients was better than MCD patients, with 5-year OS rates of 93.6% and 51.2%, respectively. […] In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. […] This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis. […] The log-rank test for OS showed a statistically significant difference between UCD and MCD patients (P < .001).
- #20 Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5765290/
Univariate analysis identified older age, splenomegaly, and hypoalbuminemia as independent prognostic factors of MCD patients. […] Multivariate analysis using a Cox proportional hazards regression model showed that age, splenomegaly, and serum albumin level were independent factors for OS in MCD patients.
- #21 Predictive Model for Idiopathic Multicentric Castleman Disease Supporting Treatment Decisionshttps://pmc.ncbi.nlm.nih.gov/articles/PMC7794201/
The International Castleman Disease Consortium recently presented the first idiopathic multicentric Castleman disease prognostic model correlating with clinical outcome. […] The overall prognosis of iMCD is poor: approximately 23%45% patients die within 5years of diagnosis, and approximately 60% die within 10years. […] The predictive value of this iMCDIPI was confirmed in a large validation cohort of 197 patients with iMCD organized from The International Castleman Disease Consortium. […] The proposed novel model is valuable for predicting clinical outcome and selecting optimal therapies using clinical parameters.
- #22 Castleman Disease: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/2219018-overview
No cases of UCD have ever been reported to develop into HHV-8negative MCD. […] HHV-8associated MCD was historically associated with a poor prognosis, with most patients dying within 2 years of diagnosis. […] However, patients have a good prognosis when treated with rituximab, with greater than 94% 2-year survival. […] In patients who do not respond to rituximab alone, chemotherapy agents such as doxorubicin and etoposide may be added to control the disease. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] In studies performed prior to the advent of antiIL-6 therapy, approximately 65% of patients survived for 5 years after diagnosis. […] A study in 2021 found a 75% 5-year overall survival rate among iMCD patients. […] There is reasonable hope that the survival rates are currently greatly improved due to the new breakthroughs in treatment (such as IL-6 blockade). […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.
- #23 Castleman Disease: Types, Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/17920-castleman-disease
The prognosis (outlook) is good for most people with UCD. Removing the affected lymph node is often enough to cure it. With treatment, UCD doesn’t usually affect life expectancy. […] The outlook for people with MCD depends on the type of MCD and how serious it is. Some people have intense symptom flare-ups that can be life-threatening. Others have more chronic symptoms. Sixty-five percent to 75% of people diagnosed with MCD are still alive five years later. With breakthrough therapies, there’s hope that the outlook will continue to improve.
Zobacz więcej
rytuksymab ostra niewydolność nerek zapalenie płuc niecałkowita resekcja etopozyd splenomegalia chemioterapeutyk wieloogniskowa choroba Castlemana pęcherzyca paraneoplastyczna idiopatyczna MCD terapia anty-IL-6 zaburzenie limfoproliferacyjne chemioterapia jednoogniskowa choroba Castlemana wskaźnik przeżycia choroba Castlemana radioterapia doksorubicyna MCD związana z HHV-8 lek immunosupresyjny całkowita resekcja sepsa poziom albuminy hipoalbuminemia