Clinical and Laboratory Findings in 13 Patients With POEMS Syndrome
Cases . | Age/Sex . | Gammopathy . | Systemic Signs Other Than POEMS . | Tissues . | β-globin DNA . | HHV-8 DNA Sequences . | |||||
---|---|---|---|---|---|---|---|---|---|---|---|
Level (log5)* . | OD (450 nm) by DEIA† . | N-PCR 1 . | N-PCR 2 . | N-PCR 3 . | No. of Positive N-PCR (%)‡ . | Evidence of HHV-8 in Tissue1-153 . | |||||
POEMS syndrome with MCD | |||||||||||
POEMS-1 | 38/M | λ | Weight loss | Lymph node 1-155 | 4 | 0.53 | 2/2 | 2/2 | 2/2 | 100 | Present |
Anasarca | Spleen | 3 | 0.13 | 2/2 | 1/2 | 1/2 | 67 | Present | |||
Thrombocytosis | Bone marrow | 4 | 0.75 | 2/3 | 2/3 | 2/3 | 67 | Present | |||
Myeloma | 4 | 0.69 | 1/1 | 1/1 | 1/1 | 100 | Present | ||||
POEMS-2 | 68/M | IgA/λ | Weight loss | Lymph node | 5 | 1.05 | 2/2 | 2/2 | 2/2 | 100 | Present |
Anasarca | Spleen | 2 | 0.21 | 2/2 | 0/2 | 0/2 | 33 | Possible | |||
Thrombocytosis | Bone marrow | 4 | 0.87 | 2/2 | 2/2 | 1/2 | 100 | Present | |||
POEMS-3 | 46/F | IgA/λ | Anasarca | Lymph node | 3 | 0.56 | 4/4 | 2/4 | 2/4 | 67 | Present |
Thrombocytosis | Spleen | 2 | 0.09 | 2/2 | 2/2 | 2/2 | 100 | Present | |||
Myeloma | 4 | 0.45 | 2/2 | 2/2 | 2/2 | 100 | Present | ||||
POEMS-4 | 53/M | IgG/λ | Thrombocytosis | Lymph node 1-155 | 4 | 0.36 | 3/3 | 3/3 | 3/3 | 100 | Present |
Myeloma | 4 | 0.50 | 2/5 | 1/5 | 1/5 | 27 | Present | ||||
POEMS-5 | 38/M | IgG/λ | Weight loss | Lymph node | 5 | 0.81 | 1/1 | 1/1 | 1/1 | 100 | Present |
Edema | |||||||||||
Thrombocytosis | |||||||||||
POEMS-6 | 55/F | IgA/λ | Weight loss | Lymph node | 4 | 0.68 | 0/1 | 0/1 | 0/1 | 0 | Absent |
Anasarca | |||||||||||
POEMS-7 | 67/F | λ | Papillary edema | PBMC | 8 | 2.50 | 1/1 | 1/1 | 1/1 | 100 | Present |
POEMS syndrome without MCD | |||||||||||
POEMS-8 | 54/M | λ | Anasarca | Lymph node | 5 | 0.98 | 0/2 | 0/2 | 0/2 | 0 | Absent |
Myeloma | 4 | 0.46 | 0/2 | 0/2 | 0/2 | 0 | Absent | ||||
POEMS-9 | 44/M | IgA/λ | Weight loss | Lymph node | 4 | 0.35 | 0/1 | 0/1 | 0/1 | 0 | Absent |
Edema | Bone marrow | 4 | 0.59 | 0/1 | 0/1 | 0/1 | 0 | Absent | |||
Thrombocytosis | Myeloma | 4 | 0.93 | 0/1 | 0/1 | 0/1 | 0 | Absent | |||
POEMS-10 | 64/M | IgG/λ | No | Bone marrow | 4 | 0.78 | 0/1 | 0/1 | 0/1 | 0 | Absent |
POEMS-111-154 | 38/F | IgA/λ | Weight loss | Bone marrow | 4 | 0.67 | 1/2 | 2/2 | 1/2 | 67 | Present |
POEMS-12 | 58/M | IgA/λ | Weight loss | Bone marrow | 5 | 0.88 | 0/1 | 0/1 | 0/1 | 0 | Absent |
IgG/κ | Thrombocytosis | ||||||||||
POEMS-13 | 82/M | IgA/λ | Edema | PBMC | 8 | 2.50 | 0/2 | 0/2 | 0/2 | 0 | Absent |
Thrombocytosis |
Cases . | Age/Sex . | Gammopathy . | Systemic Signs Other Than POEMS . | Tissues . | β-globin DNA . | HHV-8 DNA Sequences . | |||||
---|---|---|---|---|---|---|---|---|---|---|---|
Level (log5)* . | OD (450 nm) by DEIA† . | N-PCR 1 . | N-PCR 2 . | N-PCR 3 . | No. of Positive N-PCR (%)‡ . | Evidence of HHV-8 in Tissue1-153 . | |||||
POEMS syndrome with MCD | |||||||||||
POEMS-1 | 38/M | λ | Weight loss | Lymph node 1-155 | 4 | 0.53 | 2/2 | 2/2 | 2/2 | 100 | Present |
Anasarca | Spleen | 3 | 0.13 | 2/2 | 1/2 | 1/2 | 67 | Present | |||
Thrombocytosis | Bone marrow | 4 | 0.75 | 2/3 | 2/3 | 2/3 | 67 | Present | |||
Myeloma | 4 | 0.69 | 1/1 | 1/1 | 1/1 | 100 | Present | ||||
POEMS-2 | 68/M | IgA/λ | Weight loss | Lymph node | 5 | 1.05 | 2/2 | 2/2 | 2/2 | 100 | Present |
Anasarca | Spleen | 2 | 0.21 | 2/2 | 0/2 | 0/2 | 33 | Possible | |||
Thrombocytosis | Bone marrow | 4 | 0.87 | 2/2 | 2/2 | 1/2 | 100 | Present | |||
POEMS-3 | 46/F | IgA/λ | Anasarca | Lymph node | 3 | 0.56 | 4/4 | 2/4 | 2/4 | 67 | Present |
Thrombocytosis | Spleen | 2 | 0.09 | 2/2 | 2/2 | 2/2 | 100 | Present | |||
Myeloma | 4 | 0.45 | 2/2 | 2/2 | 2/2 | 100 | Present | ||||
POEMS-4 | 53/M | IgG/λ | Thrombocytosis | Lymph node 1-155 | 4 | 0.36 | 3/3 | 3/3 | 3/3 | 100 | Present |
Myeloma | 4 | 0.50 | 2/5 | 1/5 | 1/5 | 27 | Present | ||||
POEMS-5 | 38/M | IgG/λ | Weight loss | Lymph node | 5 | 0.81 | 1/1 | 1/1 | 1/1 | 100 | Present |
Edema | |||||||||||
Thrombocytosis | |||||||||||
POEMS-6 | 55/F | IgA/λ | Weight loss | Lymph node | 4 | 0.68 | 0/1 | 0/1 | 0/1 | 0 | Absent |
Anasarca | |||||||||||
POEMS-7 | 67/F | λ | Papillary edema | PBMC | 8 | 2.50 | 1/1 | 1/1 | 1/1 | 100 | Present |
POEMS syndrome without MCD | |||||||||||
POEMS-8 | 54/M | λ | Anasarca | Lymph node | 5 | 0.98 | 0/2 | 0/2 | 0/2 | 0 | Absent |
Myeloma | 4 | 0.46 | 0/2 | 0/2 | 0/2 | 0 | Absent | ||||
POEMS-9 | 44/M | IgA/λ | Weight loss | Lymph node | 4 | 0.35 | 0/1 | 0/1 | 0/1 | 0 | Absent |
Edema | Bone marrow | 4 | 0.59 | 0/1 | 0/1 | 0/1 | 0 | Absent | |||
Thrombocytosis | Myeloma | 4 | 0.93 | 0/1 | 0/1 | 0/1 | 0 | Absent | |||
POEMS-10 | 64/M | IgG/λ | No | Bone marrow | 4 | 0.78 | 0/1 | 0/1 | 0/1 | 0 | Absent |
POEMS-111-154 | 38/F | IgA/λ | Weight loss | Bone marrow | 4 | 0.67 | 1/2 | 2/2 | 1/2 | 67 | Present |
POEMS-12 | 58/M | IgA/λ | Weight loss | Bone marrow | 5 | 0.88 | 0/1 | 0/1 | 0/1 | 0 | Absent |
IgG/κ | Thrombocytosis | ||||||||||
POEMS-13 | 82/M | IgA/λ | Edema | PBMC | 8 | 2.50 | 0/2 | 0/2 | 0/2 | 0 | Absent |
Thrombocytosis |
DNA extract (1 μg) of one sample of each evaluated tissue was serially fivefold diluted and subjected to β-globin PCR; the level of amplifiable extracted DNA corresponded to the last dilution giving a positive β-globin PCR after migration of amplicons on agarose gel and visualization under UV.
The results show the level of DEIA hybridization with the RS06 probe of the amplicons obtained with the β-globin PCR amplification of the 5-5 dilution of extracted DNA; the cutoff of positivity was 0.21.
For each tissue, the percentage of positive detection corresponded to the ratio of positive results of the 3 N-PCR evaluations out of the number of all tested samples of the considered tissue.
HHV-8 DNA was considered as present in a tissue when the 3 N-PCR detections were found positive in at least one sample of the tissue, absent when the 3 N-PCR were found negative in all tissue samples, and possible in the other cases.
These lymph node samples were positive by the nonnested outer KS330233 PCR.
Black African woman.