POEMS syndrome differential diagnosis: Difference between revisions

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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>
| 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>
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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>
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* MRI contrast enhancement and enlargement of T2 spinal segments<ref name="pmid23564314">{{cite journal |vauthors=Dimachkie MM, Barohn RJ |title=Chronic inflammatory demyelinating polyneuropathy |journal=Curr Treat Options Neurol |volume=15 |issue=3 |pages=350–66 |date=June 2013 |pmid=23564314 |pmc=3987657 |doi=10.1007/s11940-013-0229-6 |url=}}</ref>
* MRI contrast enhancement and enlargement of T2 spinal segments<ref name="pmid23564314">{{cite journal |vauthors=Dimachkie MM, Barohn RJ |title=Chronic inflammatory demyelinating polyneuropathy |journal=Curr Treat Options Neurol |volume=15 |issue=3 |pages=350–66 |date=June 2013 |pmid=23564314 |pmc=3987657 |doi=10.1007/s11940-013-0229-6 |url=}}</ref>
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* EFNS/PNS criteria<ref name="urlonlinelibrary.wiley.com">{{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>
* Koski criteria<ref name="pmid19091330">{{cite journal |vauthors=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 |title=Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy |journal=J. Neurol. Sci. |volume=277 |issue=1-2 |pages=1–8 |date=February 2009 |pmid=19091330 |doi=10.1016/j.jns.2008.11.015 |url=}}</ref>
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* Corticosteroids
* Intravenous immunoglobulin (IVIG)
* Imuunosupressants (Alemtuzemab Azathioprine Cyclophosphamide Cyclosporin Etanercept Interferon-alpha)
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Multifocal Motor Neuropathy'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Multifocal Motor Neuropathy'''

Revision as of 04:13, 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)
Metabolic Syndrome (Axonal pathology)
  • Diabetes mellitus
Vitamin Deficiencies (Axonal Pathology)
  • Vitamin B12 deficiency
  • Vitamin B1 deficiency
Guillain-Barre Syndrome (Demyelinating)
  • Viral infections:
    • Epstein Barr virus
    • HIV
    • Cytomegalovirus
    • Varicella Zoster virus
  • Bacterial infections:
    • 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
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Demyelinatiing)[8][9]
  • Abnormal immune (both IgG based humoral and T-Cell mediated) response to unknown antigen (possible culprits include myelin proteins P0, P2 and PMP22)[10][11][12]
  • 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)[13]
  • Slowed motor nerve conduction velocities[14]
  • Prolonged distal motor latencies (period between F wave and initial stimulation)
  • Delayed F wave latencies (recorded from the feet, hence called "F" waves)[15]
  • MRI contrast enhancement and enlargement of T2 spinal segments[16]
  • EFNS/PNS criteria[17]
  • Koski criteria[18]
  • 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) POEMS syndrome
  • Monoclonal plasma cell proliferation
  • Cytokine storm (IL-1, IL-6, IL-12, TNF alpha, VEGF)
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) POEMS Syndrome
  • Monoclonal plasma cell proliferation
  • Cytokine storm (IL-1, IL-6, IL-12, TNF alpha, VEGF)
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.[19][20][21][22][23]

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. ↑ "Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network".
  9. ↑ "onlinelibrary.wiley.com".
  10. ↑ 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.
  11. ↑ Gabriel CM, Gregson NA, Hughes RA (May 2000). "Anti-PMP22 antibodies in patients with inflammatory neuropathy". J. Neuroimmunol. 104 (2): 139–46. PMID 10713353.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ "onlinelibrary.wiley.com".
  18. ↑ 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.
  19. ↑ Sherer Y, Bardayan Y, Shoenfeld Y (1997). "Thymoma, thymic hyperplasia, thymectomy and autoimmune diseases (Review)". Int. J. Oncol. 10 (5): 939–43. PMID 21533467.
  20. ↑ 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.
  21. ↑ 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.
  22. ↑ 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.
  23. ↑ 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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