Poems Syndrome - 2015 update

2015 update on POEMS syndrome

This is my summary of what we learned about POEMS syndrome in 2015. Please let me know if you have any question, notice any mistakes, or think other articles have interesting results! Contact me at harryide6@gmail.com.

--Harry A Ide, 24 January 2016

Brief summary

REVIEWS: Dispenzieri's new review is the best summary of what we know about POEMS.

SYMPTOMS: Subclinical dysautonomia may be more common in POEMS than we realized. The cause of POEMS neuropathy is still unknown, but two studies provide further evidence. And several studies suggest that VEGF is the cause of optic disc edema in POEMS.

DIAGNOSIS: Patients with high platelet counts are much more likely to have POEMS than CIDP, and axonal excitability may also help distinguish them. Further, several studies confirmed that CT is better than PET at detecting sclerotic (that is, more dense) bone lesions in POEMS.

PROGNOSIS: Achieving complete hematologic response matters more than what the treatment is. Patients with low albumin at diagnosis are more likely to progress or die after treatment (and they should therefore be treated early and aggressively). Serum VEGF at six months is a useful indicator of progression-free survival three years after treatment.

TREATMENT: Autologous cytokine-induced killer (CIK) cells may be a new treatment for POEMS. And the first prospective, double-blind, randomized, study of thalidomide as a treatment for POEMS is concluding. Finally, two studies from Mayo added more evidence that both neuropathy and lung function improve after an autologous stem cell transplant for POEMS. And PET scans are improved three months after treatment.


eye problems
distinguishing POEMS from CIDP
imaging skeletal lesions
a new treatment?
autologous stem cell transplant
PET after treatment
articles published (or accepted) in 2015
other cited articles


Dispenzieri 2015 is her latest review of POEMS, and is clearly the first place to go for information about POEMS.



Autonomic dysfunction hasn't been reported often in POEMS. But in a retrospective study of 60 POEMS patients who received an autologous stem cell transplant at Mayo, although only 2 patients (3%) had symptoms of dysautonomia (excluding erectile dysfunction) before the auto, 11 of the 20 tested patients (55%) had subclinical mild to moderate autonomic dysfunction (Karam 2015 p.1983). Autonomic symptoms improved in both symptomatic patients by the first visit after the auto (p.1984).


What we knew (or suspected)

The cause of POEMS neuropathy isn't well understood. On one theory, increased VEGF reduces the blood-nerve barrier, allowing neurotoxins (perhaps IL-12) to enter nerves and damage them (Kanai 2012). On another theory, increased VEGF results in nerve edema, which leads to nerve damage from compression and depolarization of the axon (Kanda 2013).

Two studies have reported nerve ultrasound results in POEMS patients. One examined nerve ultrasounds from eight POEMS patients, finding no general changes, but increased cross-sectional area at sites where nerves are often entrapped, without any correlation with symptoms (Lucchetta 2011). And nerve ultrasounds of one patient found generally thickened nerves in both upper limbs, with low systolic peaks in arterial blood flow (Yanik 2011).

And electron microscope studies of POEMS nerve biopsies often find a feature called 'uncompacted myelin lamellae' (see especially Vallat 2008).

What we learned

Two studies provide some additional evidence about how nerve damage occurs in POEMS.

Mitsuma 2015 looked at axonal excitability and nerve ultrasounds for 33 POEMS patients to gather more evidence about the cause of POEMS neuropathy. (See also the summary in Krishnan 2015.) They expected to find membrane depolarization, resulting from loss of blood flow to nerves due to compression from edema. But overall their findings did not support that theory, perhaps because when VEGF is chronically high (with the resulting edema) the body can adjust.

Hashimoto 2015 reported sural nerve biopsies from 33 POEMS patients (and 7 controls). They found that uncompacted myelin lamellae were (apparently) associated with axonal damage, and not with demyelination. (They suggest that abnormalities in Schmidt-Lanterman incisures may cause axonal degeneration and also uncompacted myelin lamellae.) They also found sodium and potassium channel abnormalities, even in neurons that appeared normal, which may help explain why neuropathy in POEMS patients who are treated early improves dramatically. (They contrast POEMS with anti-MAG-associated neuropathy, which shows poorer recovery after treatment, perhaps because neuropathy is caused initially by larger-scale defects in nerves.)

eye problems

Yokouchi 2015, 2015a, and 2015b provide evidence that increased levels of VEGF (vascular endothelial growth factor) causes eye problems in POEMS. In Yokouchi 2015, seven of the sixteen patients had bilateral optic disc edema, which is consistent with the report in Kaushik 2011 that 17 of 33 patients had optic disc edema. VEGF was correlated with subfoveal choroidal thickness but not foveal thickness (measured using enhanced depth imaging-optical coherence tomography), perhaps because higher levels of VEGF increased choroidal vascular permeability, but did not increase retinal edema because retinal blood vessels are smaller and have a smaller volume of flow than choroidal blood vessels. And in one patient, thalidomide reduced VEGF, choroidal thickness, and optic disc edema (Yokouchi 2015a). Finally, in 34 patients, VEGF and peripapillary retinal thickness were significantly correlated (Yokouchi 2015b).


distinguishing POEMS from CIDP

what we knew

POEMS and CIDP have overlapping symptoms. And CIDP is a common initial misdiagnosis for POEMS--in one study, 70% of the 51 POEMS patients met the EFNS/PNS criteria for definite CIDP (Nasu 2012).

Some differences have been reported. In particular, serum VEGF is higher in POEMS patients (Wang 2014), and CIDP primarily affects the ends of nerves (where the blood-nerve barrier is weakest), while POEMS also affects the middle (perhaps because increased VEGF in POEMS reduces the blood-nerve barrier) (Mauermann 2012; Nasu 2012--see those articles for more details about differences in EMG/NCS).

what we learned

POEMS patients are more much likely to have high platelet counts ("thrombocytosis") than CIDP patients. 53.7% of the 138 POEMS patients (using the Dispenzieri 2003 criteria) in Naddaf 2015 had thrombocytosis; only 1.5% of the 67 CIDP patients did. (The mean and median platelet counts were significantly higher, also. As the article notes, this fits with the increased risk of strokes and other problems caused by clotting, with Dupont 2009 finding a 5-year risk of 13.4%.)

Axonal excitability is also different in POEMS and CIDP. In particular, Mitsuma 2015 found that the strength-duration time constant and superexcitability are both increased in POEMS and reduced in CIDP, and conclude that whether axonal excitability can help distinguish POEMS from CIDP needs to be studied.

imaging skeletal lesions

what we knew about CT and PET

CT is better than PET at finding small lesions, but PET can be useful for follow-up on larger lesions, which have a lytic (less dense) core and sclerotic (more dense) rim (see especially Glazebrook 2014). In Glazebrook 2014, PET showed increased activity in only 15 of the 24 patients (62.5%), mostly in the lytic center, and less often in the sclerotic margin. No sclerotic lesions less than 6 mm in size showed greater activity; all greater than 2 cm in size showed greater activity.

what we learned

Two studies confirmed that CT is better at detecting POEMS sclerotic lesions than PET (Pan 2015, reporting ninety POEMS patients, and Shi 2015, reporting 22 patients). Another largely confirmed previous reports about the appearance of POEMS skeletal lesions on CT, and also reported that CT was better than ultrasound at detecting ascites, better at detecting pleural effusion than chest x-ray, and found enlarged liver, spleen, or lymph nodes in more patients than previous studies (Shi 2016, 24 patients, using the Dispenzieri 2003 criteria).

what we knew about MRI

Scattered individual case reports about use of MRI in POEMS have found low T1 and T2 signal intensity corresponding to sclerotic lesions, with some increased T2 signals, reflecting either lytic areas or edema (perhaps in a rim around the sclerotic lesion). (See Del Rio Prado 2015 (Spanish), Glazebrook 2014, Minarik 2012, Montoriol 2011, Chong 2006, Michel 2003, Furuzono 1993 (Japanese), Kim 1992, Okumura 1992 (postmortem), Brazis 1990 (which Chong 2006 p.693 suggests might actually be an invasive lesion from Castleman disease and not POEMS), some of which I know only from their abstracts.)

what we learned about MRI

Shi 2015 adds reports of MRIs in four POEMS patients, three of whom had bone lesions. In their study, MRI was as sensitive as CT, but was also able to distinguish metabolically active from inactive lesions. MRI showed decreased T2 in most sclerotic lesions (probably because they were relatively inactive, with no reactive edema), and increased T2 in mixed and lytic lesions (probably reflecting increased activity).


in general

what we knew

D'Souza 2012 found that POEMS patients were more likely to progress after an autologous stem cell transplant if

  • they had an IgG-λ m protein,
  • they had FDG-avid lesions on PET scan before the auto,
  • they did not achieve complete hematologic response after the auto (which required a negative bone marrow, and negative urine and serum immunofixation)
  • or they were 50 years or younger when they were transplanted

what we learned

Kourelis 2016 (accepted but not yet officially published) is a retrospective study of 262 patients who were treated at Mayo for POEMS between June 1974 and May 2014 (diagnosed by the Dispenzieri 2007 criteria) and for whom they had follow-up information. (This excludes 29 patients for whom they lacked detailed follow-up about relapse or progression within the first five years.) The five-year progression-free survival rate was 58%, and the five-year overall survival rate was 78%. 92 patients died in the study period: causes of death were POEMS-related complications in 17 (19%), unrelated to POEMS in 16 (17%), and unknown in 59 (64%). Median follow up was still less than six years: 68 months for overall survival (range 0-378), and 67 months for progression-free survival (range 0-349).

Most of their recommendations repeat previous advice (found, e.g., in D'Souza 2012); for example, not recommending treating isolated VEGF relapse or progression. Two points struck me as new:

Patients with low albumin at diagnosis should be treated early and aggressively.
'This suggests that albumin is predictive of early death or progression and patients with a low albumin at diagnosis should be treated early and aggressively.'
Deep hematologic response (especially complete response) is more important than treatment type.
Patients who were treated by radiation or autologous stem cell transplant did better than patients receiving other treatments when looking only at treatment type, but not when other variables were considered (multivariate analysis). This suggests type of treatment was just an indication of something else; for example, patients treated with radiation probably have less serious disease, and patients who are treated by auto may be healthier.

They also provide further evidence for several earlier conclusions:

Patients who don't achieve a hematologic complete response after first therapy are more likely to progress or die.
5-year progression-free survival for patients who had hematologic complete response to first-line therapy: 88%
5-year progression-free survival for patients who had less than hematologic complete response: 50%
5-year progression-free survival for patients not evaluated for hematologic response: 37%
All patients should be followed up closely (every 3-6 months for 5 years and 6-12 months thereafter).
79 (30%) experienced relapse or progression; 52 (19%) of them experienced symptomatic relapse or progression
53 (20%) experienced relapse or progression within 5 years of diagnosis; 26 (10%) experienced relapse or progression after five years of diagnosis
Of the 79, 10 didn't respond to the first treatment, 42 had worse symptoms, and 27 had worse labs or radiographic evaluations
92% of the patients who relapsed responsed to the second treatment, and 5 of the 6 who didn't responded to the third treatment
In patients who do experience relapse or progression, look for new symptoms, especially problems with lung function and endocrine problems.
18 (23%) patients developed new symptoms: 8 lung function, 7 endrocine, 5 fluid overload, 2 thrombocytosis, 1 skin lesions, 1 organomegaly, 1 papilledema, 1 fever.

Perhaps heavy-light chain (HLC) assay results will also prove useful in following POEMS patients (Wang 2016). (See Wang 2014 for the first report of HLC results in POEMS.) For the patients whose HLC ratio was in the bottom third of the group (less than half of the lower limit of the reference range), the estimated 5-year risk of relapse was 81%, while for those whose HLC ratio was in the top third of the group, the estimated 5-year risk of relapse was only 7%. (My note: they didn't report whether HLC response was correlated with depth of hematological response after treatment. Comparing the two would be interesting.)


What we knew before

VEGF correlates well with clinical response (see for example D'Souza 2012 p.61).

What we learned

Misawa 2015 is a retrospective study of fifty POEMS patients who were treated with either autologous stem cell transplants or thalidomide. They found that serum VEGF six months after treatment (autologous stem cell transplant or thalidomide) is a very good indicator of relapse-free survival three years after treatment, with these estimated relapse-free survivals at 3 years:

     93% of patients with serum VEGF levels < 1040 pg/mL
     40% of patients with serum VEGF levels > or = 1040 pg/mL

This should be useful for researchers, who can use VEGF at six months as an indication of how successful treatment is, instead of having to wait years. (No doubt this conclusion is intended to support the methodology of the J-POST study.) It may also be useful for making treatment decisions in individual cases. At least, anyone whose serum VEGF is less than 1040 pg/mL six months after treatment should seriously consider waiting rather than starting a second treatment.

(In case this confuses anyone: their cut-off of 1040 pg/mL of serum VEGF had 100% sensitivity and 99% specificity--that is, everyone with POEMS in their study had VEGF that high, and 99% of people with VEGF that high in their study had POEMS. But that doesn't make 1040 pg/mL a better cut-off for diagnosis than the 1920 pg/mL in Wang 2014, since this study (unlike Wang 2014) compared POEMS patients with healthy people, and not with other people who might have other diseases that produce symptoms similar to POEMS.)


a new treatment?: autologous cytokine-induced killer (CIK) cells

Ma 2016 (which has been accepted but not yet published) treated five POEMS patients with autologous CIK cells and cyclophosphamide. No patients experienced severe adverse reactions, or had to discontinue therapy because of drug-related problems. The overall neuropathy limitation scale (ONLS) scores were reduced by at least 1 in all patients. Extravascular volume overload either reduced (in 3 patients) or was stable (in 2 patients). In one patient VEGF decreased after 1 cycle of treatment (from 8800 pg/mL before treatment to 688 pg/mL after treatment). (My notes: The article confusingly says both that they 'enrolled only patients with records of serum VEGF levels before and after treatment', and that 'the other patients [sc. other than patient 5] refused to undergo these tests'. As far as I can see, this article demonstrates that treatment with CIK cells and cytoxan is safe for POEMS patients, but doesn't show that treatment with CIK cells should replace autos--but perhaps further research will show that it is useful when auto's can't be carried out or as a suppement to other therapies.)


Katayama 2015 published the study protocol for the J-POST trial, which is investigating both safety and efficacy of thalidomide as a treatment for POEMS. It's the first prospective, double-blind, controlled trial for POEMS, which assigned eligible patients either to thalidomide-dexamethasone or placebo-dexamethasone. They evaluated efficacy 24 weeks after starting treatment (primarily using serum VEGF) and then continued a 48-week study of the safety of thalidomide. (Misawa 2015 is important background, arguing that VEGF at six months is a good indicator of long-term effects.)

autologous stem cell transplant

what we knew before about the effects of autos

D'Souza 2012 is still the largest, longest, follow-up of the effects of autologous stem cell transplants in POEMS. It reported 59 patients treated at Mayo between March 1999 and October 2011, with at least 12 months of follow-up available for 56 of them, and median follow-up of 45 months. 14 patients had progressed (usually radiological, and then VEGF, with hematological and clinical progression occurring more rarely); progression free survival was 98% at 1 year and 75% at 5 years. The 5-year survival rate was 94%.

what we learned about the effects of autos

Two articles published this year from Mayo focused on specific aspects of the effect of autologous stem cell transplants (improvements in pulmonary function and in neuropathy), but also extended the follow-up.

In particular, Karam 2015 reports sixty patients (including 58 of the 59 reported in D'Souza 2012, excluding one who did not have a formal assessment by a neurologist), with a median follow-up of 61 months (range 9 to 162 months). Six patients had died (compared to three in D'Souza 2012): 1 from relapsed POEMS (4 years after the auto), 1 from failed engraftment (described a bit more fully in Dispenzieri 2004), 4 from other cancers (2 myelodysplasic syndrome, 1 lymphoma, 1 metastatic lung cancer). Consistent with previous reports, twenty four of the patients (40%) experienced engraftment syndrome, defined using the modified Spitzer criteria in D'Souza 2012, which do not require that the syndrome occur within 96 hours of engraftment, and engraftment syndrome was 'usually well managed with short-term steroid treatment'.

effects on pulmonary function

Chandrashekeran 2015 found that pulmonary function improved, looking at 53 patients treated by autologous stem cell transplant at Mayo from 2000 to 2010. (These results are also briefly reported in Karam 2015.) They found the following median improvements:

forced expiratory volume180 ml
forced vital capacity315 ml
total lung capacity350 ml
maximal inspiratory pressure12.5%
maximal expiratory pressure10%
right ventricle systolic pressure (only 13 patients)7 mm Hg
[58% of the patients who had an echo had elevated right ventricle systoic pressure]
[VEGF and IL-6 weren't correlated with right ventricle systolic pressure]
VEGF334 pg/mL
IL-62 pg/mL

32 patients had a second PFT after the auto (median follow-up of 26.5 months). Forced vital capacity, maximal inspiratory pressure, and DLCO showed small continued improvement, and other measurements remained stable.

effects on neuropathy

Karam 2015 provides more evidence that autologous stem cell transplants improve neuropathy. All patients showed significant neurologic improvement, except for the one who died early, from failed engraftment. For example, before the auto, 27 (45%) needed a wheelchair and 17 (29%) needed a walker or foot brace; at the end of the follow-up, no patient was using a wheelchair, 3 (5%) were using a walker, 7 (12%) were using a cane or crutch, and 23 (38%) were using a foot brace. The median Neuropathy Impairment Score improved from 66 to 48 at 12 months and to 30 at the most recent follow-up. The median ulnar compound motor action potential amplitude improved from 4.3 to 7.6 mV (with the median amplitude at 6.5 mV at the first evaluation after the auto), and the median velocity improved from 34 to 51 m/s (with the median at 43 m/s at first evaluation after the auto).

Autonomic symptoms had improved in both patients with dysautonomia at the first evaluation after the auto (p.1984).

They confirmed that improvements in neuropathy took longer than other improvements (e.g. in extravascular volume overload, or VEGF). Further, the drop in VEGF levels after the auto was well correlated with improvement in neuropathy.

The duration before auto was not correlated with severity of neuropathy or response to treatment. They do note that '[w]hen patients with longer than 2 years between diagnosis and ASCT were excluded, those with longer times to diagnosis were more severely affected and response to treatment was less pronounced'. (However, the correlation coefficients weren't high: the Pearson correlation coefficient between time to treatment and neuropathy severity before the auto was 0.32, and the Pearson correlation coefficient between time to treatment and neuropathy improvement after the auto was -0.4 at the first visit after the auto, and -0.24 at the last visit after the auto. Pearson correlation coefficients are between -1.0 and 1.0, with 0 reflecting no correlation.)


In Pan 2015, three months after therapy, skeletal lesions were improved on PET/CT (that is, they showed decreased FDG avidity), matching the decrease in serum VEGF, but not reflecting whether it was a partial or complete response.


Articles published (or accepted) in 2015

Chandrashekaran 2014
Chandrashekaran S, Dispenzieri A, Cha SS, Kennedy CC. 'Pulmonary morbidity improves after autologous stem cell transplantation in POEMS syndrome'. Respiratory Medicine 2015 Jan;109(1):122-30. doi: 10.1016/j.rmed.2014.11.005.
Dispenzieri 2015
Dispenzieri A. 'POEMS syndrome: Update on diagnosis, risk-stratification, and management'. American journal of hematology 2015 Sep 1. doi: 10.1002/ajh.24171.
Hashimoto 2015
Hashimoto R ; Koike H ; Takahashi M ; Ohyama K ; Kawagashira Y ; Iijima M ; Sobue G. 'Uncompacted Myelin Lamellae and Nodal Ion Channel Disruption in POEMS Syndrome'. Journal of neuropathology and experimental neurology 2015 Dec; 74(12): 1127-36 DOI: http://0-dx.doi.org.library.unl.edu/10.1097/NEN.0000000000000257
Karam 2015
Karam C, Klein CJ, Dispenzieri A, Dyck PJ, Mandrekar J, D'Souza A, Mauermann ML. 'Polyneuropathy improvement following autologous stem cell transplantation for POEMS syndrome'. Neurology 84#19 (2015) 1981-1987.
Katayama 2015
Katayama K, Misawa S, Sato Y, Sobue G, Yabe I, Watanabe O, Nishizawa M, Kusunoki S, Kikuchi S, Nakashima I, Ikeda S, Kohara N, Kanda T, Kira J, Hanaoka H, Kuwabara S. 'Japanese POEMS syndrome with Thalidomide (J-POST) Trial: study protocol for a phase II/III multicentre, randomised, double-blind, placebo-controlled trial'. BMJ Open 2015 Jan 8;5(1):e007330. doi: 10.1136/bmjopen-2014-007330.
Kourelis 2016
Kourelis TV, Buadi FK, Gertz MA, Lacy MQ, Kumar SK, Kapoor P, Go RS, Lust JA, Hayman SR, Rajkumar V, Zeldenrust SR, Russell SJ, Dingli D, Lin Y, Leung N, Hwa YL, Gonsalves W, Kyle RA, Dispenzieri A. 'Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first line treatment'. Leukemia 2015 Dec 16. doi: 10.1038/leu.2015.344. [Epub ahead of print]
Krishnan 2015
Krishnan AV. 'Polyneuropathy in POEMS syndrome: Alterations in nerve function and morphology'. Clinical neurophysiology 2015 Feb 7. pii: S1388-2457(15)00070-X. doi: 10.1016/j.clinph.2015.01.015. [Epub ahead of print]
Ma 2016
Ma L, Wang Y, Bo J, Han W, Wang Y, Zhang L, Wu X, Yu S, Liu R. 'Autologous CIK cell immunotherapy combined with cyclophosphamide in 5 patients with POEMS syndrome'. Clin Exp Immunol. 2015 Dec 13. doi: 10.1111/cei.12755. [Epub ahead of print]
Misawa 2015
Misawa S, Sato Y, Katayama K, Hanaoka H, Sawai S, Beppu M, Nomura F, Shibuya K, Sekiguchi Y, Iwai Y, Watanabe K, Amino H, Ohwada C, Takeuchi M, Sakaida E, Nakaseko C, Kuwabara S. 'Vascular endothelial growth factor as a predictive marker for POEMS syndrome treatment response: retrospective cohort study'. BMJ Open 2015:5:e009157. doi 10.1136/bmjopen-2015-009157
Mitsuma 2015
Mitsuma S, Misawa S, Shibuya K, Isose S, Sekiguchi Y, Iwai Y, Beppu M, Watanabe K, Amino H, Kuwabara S. 'Altered axonal excitability properties and nerve edema in POEMS syndrome'. Clinical neurophysiology 2015 Oct;126(10):2014-8. doi: 10.1016/j.clinph.2015.01.018
Naddaf 2015
Naddaf E, Dispenzieri A, Mandrekar J, Mauermann ML. 'Thrombocytosis distinguishes POEMS syndrome from CIDP'. Muscle & Nerve. 52 #4 (October 2015) 658-659. doi: 10.1002/mus.24768.
Pan 2015
Pan Q, Li J, Li F, Zhou D, Zhu Z. 'Characterizing POEMS syndrome with 18F-Fludeoxyglucose positron emission tomography/computed tomography'. J Nucl Med. 2015 Jul 16. pii: jnumed.115.160507. [Epub ahead of print]
Shi 2015
Shi XF, Hu SD, Li JM, Luo XF, Long ZB, Zhu Y, Xi XD. 'Multimodal imaging and clinical characteristics of bone lesions in POEMS syndrome'. International journal of clinical and experimental medicine 2015 May 15;8(5):7467-76. eCollection 2015.
Shi 2016
Shi X, Hu S, Luo X, Luo M, You H, Zhu Y, Xi X. 'CT characteristics in 24 patients with POEMS syndrome'. Acta Radiologica 57 (Jan 2016) 51-57. DOI: 10.1177/0284185114564614.
Wang 2016
Wang C, Su W, Cai QQ, Cai H, Ji W, Di Q, Duan MH, Cao XX, Zhou DB, Li J. 'Prognostic value of serum heavy/light chain ratios in patients with POEMS syndrome'. European journal of haematology 2015 Sep 18. doi: 10.1111/ejh.12682. [Epub ahead of print]
Yokouchi 2015
Yokouchi H ; Baba T ; Misawa S ; Sawai S ; Beppu M ; Kitahashi M ; Oshitari T ; Kuwabara S ; Yamamoto S. 'Correlation between serum level of vascular endothelial growth factor and subfoveal choroidal thickness in patients with POEMS syndrome'. Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie 2015 Oct; 253(10): 1641-6 DOI: http://0-dx.doi.org.library.unl.edu/10.1007/s00417-014-2bstractSend to:
Yokouchi 2015a
Yokouchi H; Oshitari T ; Baba T ; Yamamoto S. 'Thalidomide reduces choroidal thickness and optic disc edema in a patient with POEMS syndrome'. Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie 2015 Jul; 253(7): 1195-8 DOI: http://0-dx.doi.org.library.unl.edu/10.1007/s00417-014-2892-z
Yokouchi 2015b
Yokouchi H, Baba T, Misawa S, Sawai S, Kitahashi M, Oshitari T, Kuwabara S, Yamamoto S. 'Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome'. British journal of ophthalmology 2015 Oct 26. pii: bjophthalmol-2015-307068. doi: 10.1136/bjophthalmol-2015-307068. [Epub ahead of print]

Other cited articles

Brazis 1990
Brazis PW, Liesegang TJ, Bolling JP, Kashii S, Trachtman M, Burde RM. 'When do optic disc edema and peripheral neuropathy constitute poetry?' Survey of ophthalmology 1990 Nov-Dec;35(3): 219-25.
Chong 2006
Chong ST; Beasley HS; Daffner RH. 'POEMS syndrome: radiographic appearance with MRI correlation.' Skeletal radiology 2006 Sep; 35(9): 690-5
Del Rio Prado 2015
Del Río Prado AF; Reza Albarrán AA; Gómez Pérez FJ. 'Hombre de 31 años con polineuropatía, postración e hipogonadismo. [Male aged 31 years with polyneuropathy, prostration, and hypogonadism]'. Gaceta médica de México 2015 Mar-Apr; 151(2): 256-9.
Dispenzieri 2003
Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR,Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA. 'POEMS syndrome: definitions and long-term outcome'. Blood 2003 Apr 1;101(7):2496-506.
Dispenzieri 2004
Dispenzieri A; Moreno-Aspitia A; Suarez GA; Lacy MQ; Colon-Otero G; Tefferi A; Litzow MR; Roy V; Hogan WJ; Kyle RA; Gertz MA 'Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature'. Blood 2004 Nov 15; 104(10): 3400-7
Dispenzieri 2007
Dispenzieri A. 'POEMS syndrome'. Blood reviews 2007 Nov; 21(6): 285-99
D'Souza 2012
D'Souza A, Lacy M, Gertz M, Kumar S, Buadi F, Hayman S, Dingli D, Zeldenrust S, Kyle R, Ansell S, Inwards D, Johnston P, Micallef I, Porrata L, Litzow M, Gastineau D, Hogan W, Dispenzieri A. 'Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience'. Blood 2012 Jul 5;120(1):56-62. doi: 10.1182/blood-2012-04-423178.
Dupont 2009
Dupont SA, Dispenzieri A, Mauermann ML, Rabinstein AA, Brown RD Jr. 'Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics'. Neurology 2009 Oct 20;73(16):1308-12. doi: 10.1212/WNL.0b013e3181bd136b.
Furuzono 1993
Furuzono H, Moritoyo T, Yamada H, Sugihara R, Nagamatsu K. '[A case of Crow-Fukase syndrome which developed seven years following myelopathy of unknown origin].' Rinsho Shinkeigaku 1993 Jan;33(1):56-60. [Article in Japanese]
Glazebrook 2014
Glazebrook K, Bonilla F L G, Johnson A, Leng S, Dispenzieri A. 'Computed tomography assessment of bone lesions in patients with POEMS syndrome'. European radiology 2015 Feb;25(2):497-504. doi: 10.1007/s00330-014-3428-y.
Kanai 2012
Kanai K, Sawai S, Sogawa K, Mori M, Misawa S, Shibuya K, Isose S, Fujimaki Y, Noto Y, Sekiguchi Y, Nasu S, Nakaseko C, Takano S, Yoshitomi H, Miyazaki M, Nomura F, Kuwabara S. 'Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome.' Neurology 2012 Aug 7;79(6):575-82. doi: 10.1212/WNL.0b013e318263c42b. Epub 2012 Jul 25.
Kanda 2013
Kanda T. 'Biology of the blood-nerve barrier and its alteration in immune mediated neuropathies'. Journal of neurology, neurosurgery, and psychiatry 2013 Feb;84(2):208-12. doi: 10.1136/jnnp-2012-302312.
Kaushik 2011
Kaushik M, Pulido JS, Abreu R, Amselem L, Dispenzieri A. 'Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome.' Ophthalmology 2011 Apr;118(4):778-82. doi: 10.1016/j.ophtha.2010.08.013. Epub 2010 Oct 29.
Kim 1992
Kim JW, Lee SK, Ha KM, Kim KH, Joh GY, Kim HJ, Yang SO, Hong SH. 'POEMS syndrome--a case report'. J Korean Med Sci 7 #1 (Mar 1992) 79-84.
Li 2011
Li J, Zhang W, Jiao L, Duan MH, Guan HZ, Zhu WG, Tian Z, Zhou DB. 'Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome'. Blood 2011 Jun 16;117(24):6445-9. doi: 10.1182/blood-2010-12-328112.
Lucchetta 2011
Lucchetta M, Pazzaglia C, Granata G, Briani C, Padua L. 'Ultrasound evaluation of peripheral neuropathy in POEMS syndrome.' Muscle & Nerve 2011 Dec;44(6):868-72.
Mauermann 2012
Mauermann ML, Sorenson EJ, Dispenzieri A, Mandrekar J, Suarez GA, Dyck PJ, Dyck PJ. 'Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP'. J Neurol Neurosurg Psychiatry 2012 May;83(5):480-6. doi: 10.1136/jnnp-2011-301472.
Michel 2003
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Montoriol 2011
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Wang 2014
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Yanik 2011
Yanik B, Conkbayir I, Keyik B, Yoldas TK. 'Sonographic findings in a case of polyneuropathy associated with POEMS syndrome.' J Clin Ultrasound 2011 Oct;39(8):473-6. doi: 10.1002/jcu.20838. Epub 2011 May 27.

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