POEMS syndrome differential diagnosis: Difference between revisions

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Revision as of 20:13, 6 February 2019

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]

	Differential Diagnosis	Causes	Features	Therapy
Polyneuropathy	POEMS syndrome
	Metabolic Syndrome
	Vitamin Deficiencies
	Guillain-Barre Syndrome
	Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
	Multifocal Motor Neuropathy
	Environmental Toxicity
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.^[8]^[9]^[10]^[11]^[12]

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.
↑ 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.

[pmid21533467-8] Sherer Y, Bardayan Y, Shoenfeld Y (1997). "Thymoma, thymic hyperplasia, thymectomy and autoimmune diseases (Review)". Int. J. Oncol. 10 (5): 939–43. PMID 21533467.

[Nozza2017-9] 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.

[pmid17342029-10] 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.

[pmid22790102-11] 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.

[HusebyeAnderson2010-12] 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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@@ Line 9: / Line 9: @@
 {|
+!
 ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
 ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
 ! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Features}}
 ! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Therapy}}
+|-
+| rowspan="7" |Polyneuropathy
+|POEMS syndrome
+|
+|
+|
+|-
+|Metabolic Syndrome
+|
+|
+|
+|-
+|Vitamin Deficiencies
+|
+|
+|
+|-
+|Guillain-Barre Syndrome
+|
+|
+|
+|-
+|Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
+|
+|
+|
+|-
+|Multifocal Motor Neuropathy
+|
+|
+|
+|-
+|Environmental Toxicity
+|
+|
+|
+|-
+| rowspan="6" |Plasma Cell Dyscrasias
+|'''POEMS Syndrome'''
+|
+* Monoclonal plasma cell proliferation
+* Cytokine storm (IL-1, IL-6, IL-12, TNF alpha, VEGF)
+|
+|
 |-
 | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multiple myeloma]]'''
@@ Line 75: / Line 120: @@
 * Clinical manifestations related to [[tumor]] mass and compression [[symptoms]]
 | style="padding: 5px 5px; background: #F5F5F5;" |[[Surgery]]
 |-
+| rowspan="3" |Bone Lesions
 | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Osteoporosis]]'''
 | style="padding: 5px 5px; background: #F5F5F5;" |
@@ Line 105: / Line 151: @@
 *[[Vitamin D3]] supplementation
 |-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Osteogenesis imperfecta]]'''
+| style="padding: 5px 5px; background: #F5F5F5;" |
+*[[Mutations]] in ''[[COL1A1]]'' or ''[[COL1A2]]''
+*Impaired [[Type I collagen|type I collagen synthesis]]
+| style="padding: 5px 5px; background: #F5F5F5;" |
+*[[Short stature]], [[scoliosis]], and propensity for [[Bone fracture|fractures]]
+*Blue discoloration of [[sclera]]
+| style="padding: 5px 5px; background: #F5F5F5;" |
+*[[Bisphosphonates]]
+*[[Physical therapy]]
+*Surgical fixation of brittle [[bones]]
+*Genetic counseling for offspring
+|-
+|Skin Changes
 | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Scurvy]]'''
 | style="padding: 5px 5px; background: #F5F5F5;" |
@@ Line 120: / Line 180: @@
 *Citrus fruits
 |-
-| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Osteogenesis imperfecta]]'''
+|
-| style="padding: 5px 5px; background: #F5F5F5;" |
-*[[Mutations]] in ''[[COL1A1]]'' or ''[[COL1A2]]''
-*Impaired [[Type I collagen|type I collagen synthesis]]
-| style="padding: 5px 5px; background: #F5F5F5;" |
-*[[Short stature]], [[scoliosis]], and propensity for [[Bone fracture|fractures]]
-*Blue discoloration of [[sclera]]
-| style="padding: 5px 5px; background: #F5F5F5;" |
-*[[Bisphosphonates]]
-*[[Physical therapy]]
-*Surgical fixation of brittle [[bones]]
-*Genetic counseling for offspring
-|-
 | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Homocystinuria]]'''
 | style="padding: 5px 5px; background: #F5F5F5;" |
@@ Line 143: / Line 191: @@
 *[[Betaine]] supplementation
 |}
 ==Other Differentials==