POEMS syndrome differential diagnosis

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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	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]	Abnormal immune (both IgG based humoral and T-Cell mediated) response to unknown antigen (possible culprits include myelin proteins P0, P2 and PMP22)^[9]^[10]^[11]	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)^[12] Slowed motor nerve conduction velocities^[13] Prolonged distal motor latencies (period between F wave and initial stimulation) Delayed F wave latencies (recorded from the feet, hence called "F" waves)^[14] MRI contrast enhancement and enlargement of T2 spinal segments^[15]
		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
	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	Chromosomal aberrations or other genetic insults Malignant transformation of plasma cells Clonal plasma cell proliferation	Diffuse bone pain and tenderness with osteolytic lesions Renal failure Hypercalcemia Anemia		Induction chemotherapy with bortezomib, lenalidomide, and dexamethasone Bisphosphonates RANK ligand inhibitors (denosumab) Autologous stem cell transplantation
		Monoclonal gammopathy of undetermined significance (MGUS)	Chromosomal aberrations or other genetic insults Clonal plasma cell proliferation	Asymptomatic Serum M protein of <30 g/L Fewer than 10% plasma cells in the bone marrow No evidence of bone or organ damage		Observation
		Asymptomatic Plasma Cell Myeloma (Smoldering and Indolent plasma cell myeloma)	Chromosomal aberrations or other genetic insults Malignant transformation of plasma cells Clonal plasma cell proliferation	Asymptomatic Serum M protein of >30 g/L Greater than 10% plasma cells in the bone marrow No evidence of bone or organ damage		Observation
		Plasma Cell Leukemia	Chromosomal aberrations or other genetic insults Malignant transformation of plasma cells	Renal failure Hypercalcemia Cytopenias No bone lesions		Chemotherapy
		Plasmacytoma	Chromosomal aberrations or other genetic insults Malignant transformation of plasma cells	Solitary bone plasmacytoma (bone) Extramedullary plasmacytoma (soft tissues) Clinical manifestations related to tumor mass and compression symptoms		Surgery
Bone Lesions		Osteoporosis	Imbalance between bone resorption and bone formation Preceded by osteopenia Decreased bone mineral density	Acute musculoskletal pain if fractures develop Severe decrease in BMD on dual-energy X-ray absorptiometry (DEXA) test T score less than -2.5 on DEXA scan		Calcium and vitamin D supplementation Bisphosphonates Weight-bearing exercise Teriparatide RANK ligand inhibitors (denosumab)
		Osteomalacia	Inadequate mineralization of bone Deficiency of vitamin D calcium, or phosphorus Renal tubular acidosis Malabsorption	Diffuse bone pain, fatigue, and fractures Low bone mineral density (BMD) Hypocalcemia		Vitamin D3 supplementation
		Osteogenesis imperfecta	Mutations in COL1A1 or COL1A2 Impaired type I collagen synthesis	Short stature, scoliosis, and propensity for fractures Blue discoloration of sclera		Bisphosphonates Physical therapy Surgical fixation of brittle bones Genetic counseling for offspring
Skin Changes		Scurvy	Vitamin C deficiency Malabsorption Hemodialysis	Gum disease such as gum bleeding Loose teeth Easy bruising Impaired immune response Impaired wound healing		Vitamin C supplementation Citrus fruits
		Homocystinuria	Deficiency of cystathionine beta synthase Deficiency of folate, vitamin B12, or vitamin B6	Diffuse bone pain and musculoskeletal symptoms		High-dose vitamin B6 supplementation Betaine supplementation

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.^[16]^[17]^[18]^[19]^[20]

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

↑ "Myeloma - SEER Stat Fact Sheets". Retrieved 17 February 2014.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ "Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network".
↑ 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.
↑ Gabriel CM, Gregson NA, Hughes RA (May 2000). "Anti-PMP22 antibodies in patients with inflammatory neuropathy". J. Neuroimmunol. 104 (2): 139–46. PMID 10713353.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ Sherer Y, Bardayan Y, Shoenfeld Y (1997). "Thymoma, thymic hyperplasia, thymectomy and autoimmune diseases (Review)". Int. J. Oncol. 10 (5): 939–43. PMID 21533467.
↑ 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.
↑ 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.
↑ 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.
↑ 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.

Template:WikiDoc Sources

[seer-1] "Myeloma - SEER Stat Fact Sheets". Retrieved 17 February 2014.

[pmid28934935-2] 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.

[pmid26401268-3] 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.

[pmid29299040-4] 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.

[pmid29128071-5] 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.

[pmid18971951-6] 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.

[pmid25838344-7] 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.

[urlChronic_Inflammatory_Demyelinating_Polyradiculoneuropathy:_Clinical_Characteristics,_Course,_and_Recommendations_for_Diagnostic_Criteria_|_JAMA_Neurology_|_JAMA_Network-8] "Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network".

[pmid2440998-9] 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.

[pmid10713353-10] Gabriel CM, Gregson NA, Hughes RA (May 2000). "Anti-PMP22 antibodies in patients with inflammatory neuropathy". J. Neuroimmunol. 104 (2): 139–46. PMID 10713353.

[pmid10025777-11] 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.

[pmid11558784-12] 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.

[pmid2757528-13] 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.

[pmid27575282-14] 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.

[pmid23564314-15] 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.

[pmid21533467-16] Sherer Y, Bardayan Y, Shoenfeld Y (1997). "Thymoma, thymic hyperplasia, thymectomy and autoimmune diseases (Review)". Int. J. Oncol. 10 (5): 939–43. PMID 21533467.

[Nozza2017-17] 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.

[pmid17342029-18] 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.

[pmid22790102-19] 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.

[HusebyeAnderson2010-20] 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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POEMS syndrome differential diagnosis

Overview

Differentiating POEMS Syndrome From Other Diseases

Other Differentials

References

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