Table 1

Baseline characteristics

CharacteristicN%Median (range)
Age, y 38  50 (19-82) 
Gender, male 29 76  
Patients with prior therapies directed at CIDP or MPD, number patients treated 22 58  
Eastern Cooperative Oncology Group PS 2/3 12/11 31/29  
Polyneuropathy 38 100  
Monoclonal plasma cell disorder 38 100  
VEGF, pg/mL 19  375 (<32-3764) 
Castleman disease  
Organomegaly 22 58  
 Lymphadenopathy 16 42  
 Hepatomegaly 16  
 Splenomegaly 12 32  
 Endocrinopathy 33 87  
Serum immunoglobulin isotypes NA   
 IgG/IgA 27/7 71/18  
 κ/λ 2/32 5/84  
 Nonsecretory 11  
Bone marrow 38 100  
 Plasma cells, % NA  1 (1-20*
 Polyclonal/monoclonal 38/0* 100/0*  
Skin involvement 30 79  
Papilledema 13  
DLCO, % predicted 15  67 (34-108) 
RSVP, mm water 11  34 (20-62) 
Thrombocytosis or erythrocytosis 22 58  
Extravascular volume overload 31 82  
 Lower extremity edema 28 74  
 Pericardial effusion  
 Pleural effusion 13  
 Ascites 18  
Bone lesions per patient NA  1 (1-6) 
Bone lesions with sclerosis 35 92  
Number of bone lesions in total 66   
 Spine 19 29  
 Pelvis 31 47  
 Long bone  
 Rib, scapula, clavicle, or sternum 11 17  
CharacteristicN%Median (range)
Age, y 38  50 (19-82) 
Gender, male 29 76  
Patients with prior therapies directed at CIDP or MPD, number patients treated 22 58  
Eastern Cooperative Oncology Group PS 2/3 12/11 31/29  
Polyneuropathy 38 100  
Monoclonal plasma cell disorder 38 100  
VEGF, pg/mL 19  375 (<32-3764) 
Castleman disease  
Organomegaly 22 58  
 Lymphadenopathy 16 42  
 Hepatomegaly 16  
 Splenomegaly 12 32  
 Endocrinopathy 33 87  
Serum immunoglobulin isotypes NA   
 IgG/IgA 27/7 71/18  
 κ/λ 2/32 5/84  
 Nonsecretory 11  
Bone marrow 38 100  
 Plasma cells, % NA  1 (1-20*
 Polyclonal/monoclonal 38/0* 100/0*  
Skin involvement 30 79  
Papilledema 13  
DLCO, % predicted 15  67 (34-108) 
RSVP, mm water 11  34 (20-62) 
Thrombocytosis or erythrocytosis 22 58  
Extravascular volume overload 31 82  
 Lower extremity edema 28 74  
 Pericardial effusion  
 Pleural effusion 13  
 Ascites 18  
Bone lesions per patient NA  1 (1-6) 
Bone lesions with sclerosis 35 92  
Number of bone lesions in total 66   
 Spine 19 29  
 Pelvis 31 47  
 Long bone  
 Rib, scapula, clavicle, or sternum 11 17  

MPD, myeloproliferative disorder; RSVP, right ventricular systolic pressure; NA, not applicable/available.

*

There was 1 biclonal patient with iliac crest bone marrow containing clonal plasma cells not related to POEMS syndrome that instead related to concomitant smoldering multiple myeloma.

N refers to the number of lesions, not patients, and the percentage is calculated from total lesions.

Spine only includes cervical, thoracic, and lumbar regions.

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