RAPID EFFICACY OF LENALIDOMIDE PLUS DEXAMETHASONE FOR POEMS SYNDROME: ASSESSMENT OF FOUR PATIENT?S CLINICAL OUTCOME
(Abstract release date: 05/21/15)
EHA Library. Besisik S. 06/12/15; 102897; PB1897
Disclosure(s): Istanbul University, Istanbul Medical FacultyInternal Medicine, Hematology
Sevgi Besisik
Contributions
Contributions
Abstract
Abstract: PB1897
Type: Publication Only
Background
The POEMS syndrome (coined to refer to polyneuropathy; PNP), organomegaly, endocrinopathy, M protein, and skin changes) is a rare disorder and characterized by elevated vascular endothelial growth factor (VEGF) levels. The treatment for POEMS syndrome is mostly derived from regimens that have shown efficacy in other plasma cell dyscrasia, such as lenalidomide which reduces proinflammaotory and proangiogenic cytokines.
Aims
We report the efficay of a combination of lenalidomide and dexamethasone (Ld) in four patients with POEMS syndrome.
Methods
Four patients (M : F, 3 : 1)(age ranf-ge: 37 - 56 years) had been diagnosed as having POEMS syndrome. Serum VEGF level elevation could be documented only in one patient. The common main complain were fatique and weight loss which defined the reason that Internal Medicine speciaist were the first physicians. Two of the patients had severe PNP which inhibits normal daily activity and led to need assistance for walking. Two patients had severe extravascular volume overload as pleural effusion and ascite. As endocrinopathy, all of them had hypogonatorophic hypogonadism and hypocortisolemia among hypothyroidism and mild diabetes. Hyperpotasemia, mild increased serum creatinine level and hyponatremia were noted as biochemical changes. Only two patients had hyperpigmentation and acanthosis nigrigans. Treatment was started with lenalidomide (25mg, days 1-21) and dexamethasone (40mg weekly) following approvement for reimbursement. All patients received venous thrombosis prophylaxis.
Results
Massive ascite and also pleural effusion dramaticaly diminished and disappeared with the time. All of the patients put on weight and biochemistry was normalized. With intensive physical and occupational therapy, there was also steady improvement of the pateint's motor function. Two patients were agree to collect autologous peripheral blood stem cell harvesting but decide for delayed transplantation. One patient had needed radiotherapy for the pain related with bone lesions. The patient's serum free light chain ratio became normalized but M-protein was still detectable with immunfixatipn.
Summary
Our experience suggest that Ld therapy is not only realtively safe but also a promising option for POEMS syndrome.
Session topic: Publication Only
Type: Publication Only
Background
The POEMS syndrome (coined to refer to polyneuropathy; PNP), organomegaly, endocrinopathy, M protein, and skin changes) is a rare disorder and characterized by elevated vascular endothelial growth factor (VEGF) levels. The treatment for POEMS syndrome is mostly derived from regimens that have shown efficacy in other plasma cell dyscrasia, such as lenalidomide which reduces proinflammaotory and proangiogenic cytokines.
Aims
We report the efficay of a combination of lenalidomide and dexamethasone (Ld) in four patients with POEMS syndrome.
Methods
Four patients (M : F, 3 : 1)(age ranf-ge: 37 - 56 years) had been diagnosed as having POEMS syndrome. Serum VEGF level elevation could be documented only in one patient. The common main complain were fatique and weight loss which defined the reason that Internal Medicine speciaist were the first physicians. Two of the patients had severe PNP which inhibits normal daily activity and led to need assistance for walking. Two patients had severe extravascular volume overload as pleural effusion and ascite. As endocrinopathy, all of them had hypogonatorophic hypogonadism and hypocortisolemia among hypothyroidism and mild diabetes. Hyperpotasemia, mild increased serum creatinine level and hyponatremia were noted as biochemical changes. Only two patients had hyperpigmentation and acanthosis nigrigans. Treatment was started with lenalidomide (25mg, days 1-21) and dexamethasone (40mg weekly) following approvement for reimbursement. All patients received venous thrombosis prophylaxis.
Results
Massive ascite and also pleural effusion dramaticaly diminished and disappeared with the time. All of the patients put on weight and biochemistry was normalized. With intensive physical and occupational therapy, there was also steady improvement of the pateint's motor function. Two patients were agree to collect autologous peripheral blood stem cell harvesting but decide for delayed transplantation. One patient had needed radiotherapy for the pain related with bone lesions. The patient's serum free light chain ratio became normalized but M-protein was still detectable with immunfixatipn.
Summary
Our experience suggest that Ld therapy is not only realtively safe but also a promising option for POEMS syndrome.
Session topic: Publication Only
Abstract: PB1897
Type: Publication Only
Background
The POEMS syndrome (coined to refer to polyneuropathy; PNP), organomegaly, endocrinopathy, M protein, and skin changes) is a rare disorder and characterized by elevated vascular endothelial growth factor (VEGF) levels. The treatment for POEMS syndrome is mostly derived from regimens that have shown efficacy in other plasma cell dyscrasia, such as lenalidomide which reduces proinflammaotory and proangiogenic cytokines.
Aims
We report the efficay of a combination of lenalidomide and dexamethasone (Ld) in four patients with POEMS syndrome.
Methods
Four patients (M : F, 3 : 1)(age ranf-ge: 37 - 56 years) had been diagnosed as having POEMS syndrome. Serum VEGF level elevation could be documented only in one patient. The common main complain were fatique and weight loss which defined the reason that Internal Medicine speciaist were the first physicians. Two of the patients had severe PNP which inhibits normal daily activity and led to need assistance for walking. Two patients had severe extravascular volume overload as pleural effusion and ascite. As endocrinopathy, all of them had hypogonatorophic hypogonadism and hypocortisolemia among hypothyroidism and mild diabetes. Hyperpotasemia, mild increased serum creatinine level and hyponatremia were noted as biochemical changes. Only two patients had hyperpigmentation and acanthosis nigrigans. Treatment was started with lenalidomide (25mg, days 1-21) and dexamethasone (40mg weekly) following approvement for reimbursement. All patients received venous thrombosis prophylaxis.
Results
Massive ascite and also pleural effusion dramaticaly diminished and disappeared with the time. All of the patients put on weight and biochemistry was normalized. With intensive physical and occupational therapy, there was also steady improvement of the pateint's motor function. Two patients were agree to collect autologous peripheral blood stem cell harvesting but decide for delayed transplantation. One patient had needed radiotherapy for the pain related with bone lesions. The patient's serum free light chain ratio became normalized but M-protein was still detectable with immunfixatipn.
Summary
Our experience suggest that Ld therapy is not only realtively safe but also a promising option for POEMS syndrome.
Session topic: Publication Only
Type: Publication Only
Background
The POEMS syndrome (coined to refer to polyneuropathy; PNP), organomegaly, endocrinopathy, M protein, and skin changes) is a rare disorder and characterized by elevated vascular endothelial growth factor (VEGF) levels. The treatment for POEMS syndrome is mostly derived from regimens that have shown efficacy in other plasma cell dyscrasia, such as lenalidomide which reduces proinflammaotory and proangiogenic cytokines.
Aims
We report the efficay of a combination of lenalidomide and dexamethasone (Ld) in four patients with POEMS syndrome.
Methods
Four patients (M : F, 3 : 1)(age ranf-ge: 37 - 56 years) had been diagnosed as having POEMS syndrome. Serum VEGF level elevation could be documented only in one patient. The common main complain were fatique and weight loss which defined the reason that Internal Medicine speciaist were the first physicians. Two of the patients had severe PNP which inhibits normal daily activity and led to need assistance for walking. Two patients had severe extravascular volume overload as pleural effusion and ascite. As endocrinopathy, all of them had hypogonatorophic hypogonadism and hypocortisolemia among hypothyroidism and mild diabetes. Hyperpotasemia, mild increased serum creatinine level and hyponatremia were noted as biochemical changes. Only two patients had hyperpigmentation and acanthosis nigrigans. Treatment was started with lenalidomide (25mg, days 1-21) and dexamethasone (40mg weekly) following approvement for reimbursement. All patients received venous thrombosis prophylaxis.
Results
Massive ascite and also pleural effusion dramaticaly diminished and disappeared with the time. All of the patients put on weight and biochemistry was normalized. With intensive physical and occupational therapy, there was also steady improvement of the pateint's motor function. Two patients were agree to collect autologous peripheral blood stem cell harvesting but decide for delayed transplantation. One patient had needed radiotherapy for the pain related with bone lesions. The patient's serum free light chain ratio became normalized but M-protein was still detectable with immunfixatipn.
Summary
Our experience suggest that Ld therapy is not only realtively safe but also a promising option for POEMS syndrome.
Session topic: Publication Only
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