POEMS syndrome differential diagnosis: Difference between revisions

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** Symmetrical, ascending chronic progressive [[polyneuropathy]] with both [[Sensory system|sensory]] (pin-prick and vibration) and motor disability (motor > sensory)
** Symmetrical, ascending chronic progressive [[polyneuropathy]] with both [[Sensory system|sensory]] (pin-prick and vibration) and motor disability (motor > sensory)
** Pain
** Generalized/extermity pain
** Areflexia
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**Increased number of [[Platelet|thrombocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
**Increased number of [[Platelet|thrombocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
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* Symmetric sensorimotor polyneuropathy
* Symmetric sensorimotor polyneuropathy
* Autonomic neuropathy
* Autonomic neuropathy
* "Glove and stocking" type pain
* Muscle wasting
* Hammer toes
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* Uncontrolled hyperglycemia
* Slowed nerve conduction
* Small fiber dysfunction
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* Fasting blood sugar level greater than equal to 126 mg/dl on 2 separate ocassions
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* Anti-diabetic therapy
* Gabapentin
* Carbamazepine
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Vitamin Deficiencies (Axonal Pathology)'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Vitamin Deficiencies (Axonal Pathology)'''
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* DVT/PE prevention
* DVT/PE prevention
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Demyelinatiing)'''<ref name="urlChronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network">{{cite web |url=https://jamanetwork.com/journals/jamaneurology/article-abstract/589258 |title=Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria &#124; JAMA Neurology &#124; JAMA Network |format= |work= |accessdate=}}</ref><ref name="urlonlinelibrary.wiley.com2">{{cite web |url=https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1468-1331.2009.02930.x |title=onlinelibrary.wiley.com |format= |work= |accessdate=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)'''<ref name="urlChronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network">{{cite web |url=https://jamanetwork.com/journals/jamaneurology/article-abstract/589258 |title=Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria &#124; JAMA Neurology &#124; JAMA Network |format= |work= |accessdate=}}</ref><ref name="urlonlinelibrary.wiley.com2">{{cite web |url=https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1468-1331.2009.02930.x |title=onlinelibrary.wiley.com |format= |work= |accessdate=}}</ref>
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* Abnormal immune (both IgG based humoral and T-Cell mediated) response to unknown antigen (possible culprits include myelin proteins P0, P2 and PMP22)<ref name="pmid2440998">{{cite journal |vauthors=Milner P, Lovelidge CA, Taylor WA, Hughes RA |title=P0 myelin protein produces experimental allergic neuritis in Lewis rats |journal=J. Neurol. Sci. |volume=79 |issue=3 |pages=275–85 |date=July 1987 |pmid=2440998 |doi= |url=}}</ref><ref name="pmid10713353">{{cite journal |vauthors=Gabriel CM, Gregson NA, Hughes RA |title=Anti-PMP22 antibodies in patients with inflammatory neuropathy |journal=J. Neuroimmunol. |volume=104 |issue=2 |pages=139–46 |date=May 2000 |pmid=10713353 |doi= |url=}}</ref><ref name="pmid10025777">{{cite journal |vauthors=Bouchard C, Lacroix C, Planté V, Adams D, Chedru F, Guglielmi JM, Said G |title=Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy |journal=Neurology |volume=52 |issue=3 |pages=498–503 |date=February 1999 |pmid=10025777 |doi= |url=}}</ref>
* Abnormal immune (both IgG based humoral and T-Cell mediated) response to unknown antigen (possible culprits include myelin proteins P0, P2 and PMP22)<ref name="pmid2440998">{{cite journal |vauthors=Milner P, Lovelidge CA, Taylor WA, Hughes RA |title=P0 myelin protein produces experimental allergic neuritis in Lewis rats |journal=J. Neurol. Sci. |volume=79 |issue=3 |pages=275–85 |date=July 1987 |pmid=2440998 |doi= |url=}}</ref><ref name="pmid10713353">{{cite journal |vauthors=Gabriel CM, Gregson NA, Hughes RA |title=Anti-PMP22 antibodies in patients with inflammatory neuropathy |journal=J. Neuroimmunol. |volume=104 |issue=2 |pages=139–46 |date=May 2000 |pmid=10713353 |doi= |url=}}</ref><ref name="pmid10025777">{{cite journal |vauthors=Bouchard C, Lacroix C, Planté V, Adams D, Chedru F, Guglielmi JM, Said G |title=Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy |journal=Neurology |volume=52 |issue=3 |pages=498–503 |date=February 1999 |pmid=10025777 |doi= |url=}}</ref>

Revision as of 05:11, 8 February 2019

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Overview

POEMS syndrome must be differentiated from other similar conditions which lead to multiple endocrine disorders such as autoimmune polyendocrine syndrome, Hirata syndrome, Kearns–Sayre syndrome and Wolfram syndromes.

Differentiating POEMS Syndrome From Other Diseases

The table below summarizes how to differentiate POEMS syndrome from other conditions that have a similar presentation:[1][2][3][4][5][6][7]

Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
P = Polyneuropathy POEMS syndrome (Demyelinating)
  • Monoclonal plasma cell proliferation
  • Cytokine storm (IL-1, IL-6, IL-12, TNF alpha, VEGF)
    • Symmetrical, ascending chronic progressive polyneuropathy with both sensory (pin-prick and vibration) and motor disability (motor > sensory)
    • Generalized/extermity pain
    • Areflexia
  • International Myeloma Working Group (IMWG) clinical and laboratory diagnostic criteria
Metabolic Syndrome (Axonal pathology)
  • Diabetes mellitus
  • Symmetric sensorimotor polyneuropathy
  • Autonomic neuropathy
  • "Glove and stocking" type pain
  • Muscle wasting
  • Hammer toes
  • Uncontrolled hyperglycemia
  • Slowed nerve conduction
  • Small fiber dysfunction
  • Fasting blood sugar level greater than equal to 126 mg/dl on 2 separate ocassions
  • Anti-diabetic therapy
  • Gabapentin
  • Carbamazepine
Vitamin Deficiencies (Axonal Pathology)
  • Vitamin B12 deficiency
  • Vitamin B1 deficiency
Guillain-Barre Syndrome (Demyelinating)[13]
  • Anti-ganglioside and anti-myelin antibodies[14]
  • Viral infections:[15]
    • Epstein Barr virus
    • HIV
    • Cytomegalovirus
    • Varicella Zoster virus
  • Bacterial infections:[16]
    • Campylobacter infection
    • Mycoplasma pneumoniae
  • Rapid onset and quick progression
  • Progression stops after 2-3 weeks
  • Bilateral ascending paraesthesias and paralysis (generalized)
  • Weakness
  • Ataxia
  • Areflexia
  • No fever
  • 4 sub-types:
    • Acute inflammatory demyelinating polyneuropathy
    • Acute motor axonal neuropathy
    • Acute motor and sensory axonal neuropathy
    • Miller Fisher syndrome
  • Clinical diagnostic criteria (progressive weakness of more than two limbs, areflexia, and progression for no more than four weeks)[18]
  • Intravenous immunoglobulins[19]
  • Plasma exchange[20]
  • Respiratory support
  • DVT/PE prevention
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)[21][22]
  • Abnormal immune (both IgG based humoral and T-Cell mediated) response to unknown antigen (possible culprits include myelin proteins P0, P2 and PMP22)[23][24][25]
  • Slow onset and gradual progression
  • Relapsing and remitting course
  • Symmetrical proximal and distal motor and sensory weakness (legs>arms)
  • Foot drop
  • Numbness, tingling and pain
  • Areflexia
  • Elevated CSF protein (oligoclonal bands with normal WBCs)[26]
  • Slowed motor nerve conduction velocities[27]
  • Prolonged distal motor latencies (period between F wave and initial stimulation)
  • Delayed F wave latencies (recorded from the feet, hence called "F" waves)[28]
  • MRI contrast enhancement and enlargement of T2 spinal segments[29]
  • EFNS/PNS criteria[30]
  • Koski criteria[31]
  • Corticosteroids
  • Intravenous immunoglobulin (IVIG)
  • Imuunosupressants (Alemtuzemab Azathioprine Cyclophosphamide Cyclosporin Etanercept Interferon-alpha)
Multifocal Motor Neuropathy
Environmental Toxicity
Organ System Involvement Differential Diagnosis Causes Features Therapy
O = Organomegaly (Hepatosplenomegaly and Lymphadenopathy) Malaria
Kala-azar
Infective Hepatitis
Chronic Myelogenous Leukemia (CML)
Lymphoma
Amyloidosis
Gaucher's Disease
Organ System Involvement Differential Diagnosis Causes Features Therapy
E = Endocrinopathy (Hypogonadism, Hypothyroidism, Hypopituitarism) Hypogonadism
Hypothyroidism Primary Hypothyroidism
  • Congenital hypothyroidism
  • Autoimmune (Hashimoto's) thyroiditis
  • Resistance to TSH
Secondary Hypothyroidism
  • Pituitary mass lesions, especially pituitary adenomas
  • Brain cysts and abscesses
  • Meningiomas
  • Dysgerminomas
  • Metastatic tumors
  • Craniopharyngiomas
  • Pituitary apoplexy
  • Sheehan syndrome (postpartum pituitary necrosis)
  • Idiopathic isolated TSH deficiency
  • Lymphocytic or granulomatous hypophysitis
Tertiary Hypothyroidism
  • Hemochromatosis
  • Histiocytosis
  • Developmental abnormalities
  • Internal carotid aneurysms
  • Idiopathic isolated TRH deficiency
Hypopituitarism
Organ System Involvement Differential Diagnosis Causes Features Therapy
M = M-protein ( Hematological Abnormality/Plasma Cell Dyscrasias) Multiple myeloma
Monoclonal gammopathy of undetermined significance (MGUS) Observation
Asymptomatic Plasma Cell Myeloma

(Smoldering and Indolent plasma cell myeloma)

Observation
Plasma Cell Leukemia Chemotherapy
Plasmacytoma Surgery
Bone Lesions Osteoporosis
Osteomalacia
Osteogenesis imperfecta
Skin Changes Scurvy
Homocystinuria

Other Differentials

POEMS syndrome must also be differentiated from other similar conditions which lead to multiple endocrine disorders such as autoimmune polyendocrine syndrome, Hirata syndrome, Kearns–Sayre syndrome and Wolfram syndromes.[32][33][34][35][36]

Disease Addison's disease Type 1 diabetes mellitus Hypothyroidism Other disorders present
POEMS syndrome + Less common Less common Hypoparathyroidism
Candidiasis
Hypogonadism
APS type 2 + + + Hypogonadism
Malabsorption
APS type 3 - + + Malabsorption
Thymoma + - + Myasthenia gravis
Cushing syndrome
Chromosomal abnormalities
(Turner syndrome,
Down's syndrome) 		- + + Cardiac dysfunction
Kearns–Sayre syndrome - + - Myopathy
Hypoparathyroidism
Hypogonadism
Wolfram syndrome - + - Diabetes insipidus
Optic atrophy
Deafness
POEMS syndrome - + - Polyneuropathy
Hypogonadism
Plasma cell dyscrasias

References

  1. ↑ "Myeloma - SEER Stat Fact Sheets". Retrieved 17 February 2014.
  2. ↑ Zuo QY, Wang H, Li W, Niu XH, Huang YH, Chen J; et al. (2017). "Treatment and outcomes of tumor-induced osteomalacia associated with phosphaturic mesenchymal tumors: retrospective review of 12 patients". BMC Musculoskelet Disord. 18 (1): 403. doi:10.1186/s12891-017-1756-1. PMC 5609032. PMID 28934935.
  3. ↑ Shaker JL, Albert C, Fritz J, Harris G (2015). "Recent developments in osteogenesis imperfecta". F1000Res. 4 (F1000 Faculty Rev): 681. doi:10.12688/f1000research.6398.1. PMC 4566283. PMID 26401268.
  4. ↑ Kumar A, Palfrey HA, Pathak R, Kadowitz PJ, Gettys TW, Murthy SN (2017). "The metabolism and significance of homocysteine in nutrition and health". Nutr Metab (Lond). 14: 78. doi:10.1186/s12986-017-0233-z. PMC 5741875. PMID 29299040.
  5. ↑ Soh KT, Tario JD, Wallace PK (December 2017). "Diagnosis of Plasma Cell Dyscrasias and Monitoring of Minimal Residual Disease by Multiparametric Flow Cytometry". Clin. Lab. Med. 37 (4): 821–853. doi:10.1016/j.cll.2017.08.001. PMC 5804349. PMID 29128071.
  6. ↑ Kyle RA, Rajkumar SV (January 2009). "Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma". Leukemia. 23 (1): 3–9. doi:10.1038/leu.2008.291. PMC 2627786. PMID 18971951.
  7. ↑ Rajkumar SV, Landgren O, Mateos MV (May 2015). "Smoldering multiple myeloma". Blood. 125 (20): 3069–75. doi:10.1182/blood-2014-09-568899. PMC 4432003. PMID 25838344.
  8. ↑ 8.0 8.1 Nozza A (2017). "POEMS SYNDROME: an Update". Mediterr J Hematol Infect Dis. 9 (1): e2017051. doi:10.4084/MJHID.2017.051. PMC 5584767. PMID 28894560.
  9. ↑ 9.0 9.1 9.2 Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (July 1980). "Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature". Medicine (Baltimore). 59 (4): 311–22. PMID 6248720.
  10. ↑ 10.0 10.1 10.2 10.3 10.4 Takatsuki K, Sanada I (September 1983). "Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases". Jpn. J. Clin. Oncol. 13 (3): 543–55. PMID 6315993.
  11. ↑ Nobile-Orazio E, Terenghi F, Giannotta C, Gallia F, Nozza A (March 2009). "Serum VEGF levels in POEMS syndrome and in immune-mediated neuropathies". Neurology. 72 (11): 1024–6. doi:10.1212/01.wnl.0000344569.13496.ff. PMID 19289745.
  12. ↑ Güneş HN, Bilecenoğlu NT, Şener U, Yoldaş TK (April 2015). "POEMS syndrome with peripheral and central nervous system demyelination: case report". Neurologist. 19 (4): 101–3. doi:10.1097/NRL.0000000000000017. PMID 25888197.
  13. ↑ Winer JB (December 2001). "Guillain Barré syndrome". MP, Mol. Pathol. 54 (6): 381–5. PMC 1187127. PMID 11724912.
  14. ↑ Rees JH, Gregson NA, Hughes RA (November 1995). "Anti-ganglioside GM1 antibodies in Guillain-Barré syndrome and their relationship to Campylobacter jejuni infection". Ann. Neurol. 38 (5): 809–16. doi:10.1002/ana.410380516. PMID 7486873.
  15. ↑ Winer JB, Hughes RA, Anderson MJ, Jones DM, Kangro H, Watkins RP (May 1988). "A prospective study of acute idiopathic neuropathy. II. Antecedent events". J. Neurol. Neurosurg. Psychiatry. 51 (5): 613–8. PMC 1033063. PMID 3404161.
  16. ↑ Yuki N, Koga M (October 2006). "Bacterial infections in Guillain-Barré and Fisher syndromes". Curr. Opin. Neurol. 19 (5): 451–7. doi:10.1097/01.wco.0000245367.36576.e9. PMID 16969154.
  17. ↑ Kimura J (April 1978). "Proximal versus distal slowing of motor nerve conduction velocity in the Guillain-Barré syndrome". Ann. Neurol. 3 (4): 344–50. doi:10.1002/ana.410030412. PMID 666277.
  18. ↑ Winer JB (December 2001). "Guillain Barré syndrome". MP, Mol. Pathol. 54 (6): 381–5. PMC 1187127. PMID 11724912.
  19. ↑ "Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group". Lancet. 349 (9047): 225–30. January 1997. PMID 9014908.
  20. ↑ "Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group". Neurology. 35 (8): 1096–104. August 1985. PMID 4022342.
  21. ↑ "Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network".
  22. ↑ "onlinelibrary.wiley.com".
  23. ↑ Milner P, Lovelidge CA, Taylor WA, Hughes RA (July 1987). "P0 myelin protein produces experimental allergic neuritis in Lewis rats". J. Neurol. Sci. 79 (3): 275–85. PMID 2440998.
  24. ↑ Gabriel CM, Gregson NA, Hughes RA (May 2000). "Anti-PMP22 antibodies in patients with inflammatory neuropathy". J. Neuroimmunol. 104 (2): 139–46. PMID 10713353.
  25. ↑ Bouchard C, Lacroix C, Planté V, Adams D, Chedru F, Guglielmi JM, Said G (February 1999). "Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy". Neurology. 52 (3): 498–503. PMID 10025777.
  26. ↑ Yan WX, Archelos JJ, Hartung HP, Pollard JD (September 2001). "P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy". Ann. Neurol. 50 (3): 286–92. PMID 11558784.
  27. ↑ Barohn RJ, Kissel JT, Warmolts JR, Mendell JR (August 1989). "Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria". Arch. Neurol. 46 (8): 878–84. PMID 2757528.
  28. ↑ Barohn RJ, Kissel JT, Warmolts JR, Mendell JR (August 1989). "Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria". Arch. Neurol. 46 (8): 878–84. PMID 2757528.
  29. ↑ Dimachkie MM, Barohn RJ (June 2013). "Chronic inflammatory demyelinating polyneuropathy". Curr Treat Options Neurol. 15 (3): 350–66. doi:10.1007/s11940-013-0229-6. PMC 3987657. PMID 23564314.
  30. ↑ "onlinelibrary.wiley.com".
  31. ↑ Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, Gorson K, Hahn AF, Hughes RA, Katz J, Lewis RA, Parry GJ, van Doorn P, Cornblath DR (February 2009). "Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy". J. Neurol. Sci. 277 (1–2): 1–8. doi:10.1016/j.jns.2008.11.015. PMID 19091330.
  32. ↑ Sherer Y, Bardayan Y, Shoenfeld Y (1997). "Thymoma, thymic hyperplasia, thymectomy and autoimmune diseases (Review)". Int. J. Oncol. 10 (5): 939–43. PMID 21533467.
  33. ↑ Nozza, Andrea (2017). "POEMS SYNDROME: AN UPDATE". Mediterranean Journal of Hematology and Infectious Diseases. 9 (1): e2017051. doi:10.4084/mjhid.2017.051. ISSN 2035-3006.
  34. ↑ Maceluch JA, Niedziela M (2006). "The clinical diagnosis and molecular genetics of kearns-sayre syndrome: a complex mitochondrial encephalomyopathy". Pediatr Endocrinol Rev. 4 (2): 117–37. PMID 17342029.
  35. ↑ Rigoli L, Di Bella C (2012). "Wolfram syndrome 1 and Wolfram syndrome 2". Curr. Opin. Pediatr. 24 (4): 512–7. doi:10.1097/MOP.0b013e328354ccdf. PMID 22790102.
  36. ↑ Husebye, Eystein S.; Anderson, Mark S. (2010). "Autoimmune Polyendocrine Syndromes: Clues to Type 1 Diabetes Pathogenesis". Immunity. 32 (4): 479–487. doi:10.1016/j.immuni.2010.03.016. ISSN 1074-7613.

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