Contributions
Abstract: PB1699
Type: Publication Only
Session title: Myeloma and other monoclonal gammopathies - Clinical
Background
POEMS syndrome (POEMS) is a rare paraneoplastic syndrome of an underlying plasma cell disorder (PCD). Castleman disease (CD),itself,can also cause symptoms or signs of polyneuropathy,volume overload,cytokine release,but only those with peripheral neuropathy and a plasma cell clone can be classified as classical POEMS.
Aims
Both classical POEMS syndrome with CD and castleman disease variant of POEMS syndrome(CD-POEMS),literature lacks a standardized treatment approach.
Methods
Here we present a case who is diagnosed with CD-POEMS and successfully treated with a combination of Daratumumab, Lenalidomide and Dexamethasone (DRd).
Results
A 54-yr-old man had admitted to our department of neurology with weakness of both arms and legs. Three years ago,patient developed tingling on legs and ataxia. He needed assistance for walking. The neurological examination showed bilateral upper and lower limb muscle weakness especially dominant on distal parts of the extremities on admission. There was an evident atrophy on distal part of the extremities. Stocking-glove pattern for loss of vibration and pinprick sensation was found. Romberg sign was present. The rest of the neurological examination was normal. Electrodiagnostic study showed that sensory nerve action potential,compound muscle action potential is diminished on bilateral upper and lower extremities. To exclude a possible accompanying plasma cell disorder,serum protein electrophoresis,serum immunofixation electrophoresis, serum free kappa and lambda light chains were odered and revealed no monoclonal protein. But further urine immunofixation electrophoresis revealed a lambda monoclonal protein in urine. Then serum VEGF and IL6 levels were ordered. At the same time because of a previous CT scan showing multiple mediastinal masses, a PET scan was ordered to document any FDG avid masses. Serum VEGF level was 531 pg/ml (<96 pg/ml) and serum IL-6 level was measured as 24.75 pg/ml (<7 pg/ml) PET scan revealed axillary, paraaortic and aorta-caval masses with a biggest dimension of 5x4 cm and a maximum SUVmax of 3.5. Excisional biopsy of the most FDG avid mass was reported as hyalin vascular type Castleman Disease. He also suffered from coronary artery disease and a chronic subdural hematoma, which are now regarded as the vascular complications of PCD. The patient was diagnosed as CD-POEMS and a combination of Daratumumab – Lenalidomide – Dexamethasone was initiated with a protocol of weekly Daratumumab 16 mg/m2 for first 2 cycles, then once in every two weeks for 4 cycles and then once in a month with the same dose, Lenalidomide 25 mg po 21/28 and dexamethasone 20 mgs per day D1,2 D8,9 D15,16 /28. The aforementioned triple combination was well tolerated. After the 1st cycle of the treatment a VEGF response was achieved, and serum VEGF level was declined to 27.2 pg/ml. Also the serum IL-6 level was decreased and measured as 6.31 pg/ml. The patient was able to stand up and walk with the help of two walking sticks. The PET scan revealed a complete response regarding the Castleman component of the disease. At the third cycle of DRd combination he also underwent a successful revascularization of coronary arteries for the treatment of coronary artery disease. He is now on the 6th Cycle of the treatment with an ongoing and even deepening hematological and neurological response and able to walk without any support.
Conclusion
This case should serve as a proof of consept among the efficacy and safety of Daratumumab-Lenalidomide-Dexamethasone combination in the treatment of castleman variant of POEMS syndrome.
Keyword(s): Monoclonal gammopathy, Treatment
Abstract: PB1699
Type: Publication Only
Session title: Myeloma and other monoclonal gammopathies - Clinical
Background
POEMS syndrome (POEMS) is a rare paraneoplastic syndrome of an underlying plasma cell disorder (PCD). Castleman disease (CD),itself,can also cause symptoms or signs of polyneuropathy,volume overload,cytokine release,but only those with peripheral neuropathy and a plasma cell clone can be classified as classical POEMS.
Aims
Both classical POEMS syndrome with CD and castleman disease variant of POEMS syndrome(CD-POEMS),literature lacks a standardized treatment approach.
Methods
Here we present a case who is diagnosed with CD-POEMS and successfully treated with a combination of Daratumumab, Lenalidomide and Dexamethasone (DRd).
Results
A 54-yr-old man had admitted to our department of neurology with weakness of both arms and legs. Three years ago,patient developed tingling on legs and ataxia. He needed assistance for walking. The neurological examination showed bilateral upper and lower limb muscle weakness especially dominant on distal parts of the extremities on admission. There was an evident atrophy on distal part of the extremities. Stocking-glove pattern for loss of vibration and pinprick sensation was found. Romberg sign was present. The rest of the neurological examination was normal. Electrodiagnostic study showed that sensory nerve action potential,compound muscle action potential is diminished on bilateral upper and lower extremities. To exclude a possible accompanying plasma cell disorder,serum protein electrophoresis,serum immunofixation electrophoresis, serum free kappa and lambda light chains were odered and revealed no monoclonal protein. But further urine immunofixation electrophoresis revealed a lambda monoclonal protein in urine. Then serum VEGF and IL6 levels were ordered. At the same time because of a previous CT scan showing multiple mediastinal masses, a PET scan was ordered to document any FDG avid masses. Serum VEGF level was 531 pg/ml (<96 pg/ml) and serum IL-6 level was measured as 24.75 pg/ml (<7 pg/ml) PET scan revealed axillary, paraaortic and aorta-caval masses with a biggest dimension of 5x4 cm and a maximum SUVmax of 3.5. Excisional biopsy of the most FDG avid mass was reported as hyalin vascular type Castleman Disease. He also suffered from coronary artery disease and a chronic subdural hematoma, which are now regarded as the vascular complications of PCD. The patient was diagnosed as CD-POEMS and a combination of Daratumumab – Lenalidomide – Dexamethasone was initiated with a protocol of weekly Daratumumab 16 mg/m2 for first 2 cycles, then once in every two weeks for 4 cycles and then once in a month with the same dose, Lenalidomide 25 mg po 21/28 and dexamethasone 20 mgs per day D1,2 D8,9 D15,16 /28. The aforementioned triple combination was well tolerated. After the 1st cycle of the treatment a VEGF response was achieved, and serum VEGF level was declined to 27.2 pg/ml. Also the serum IL-6 level was decreased and measured as 6.31 pg/ml. The patient was able to stand up and walk with the help of two walking sticks. The PET scan revealed a complete response regarding the Castleman component of the disease. At the third cycle of DRd combination he also underwent a successful revascularization of coronary arteries for the treatment of coronary artery disease. He is now on the 6th Cycle of the treatment with an ongoing and even deepening hematological and neurological response and able to walk without any support.
Conclusion
This case should serve as a proof of consept among the efficacy and safety of Daratumumab-Lenalidomide-Dexamethasone combination in the treatment of castleman variant of POEMS syndrome.
Keyword(s): Monoclonal gammopathy, Treatment