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Hyperglobulinemic purpura

MedGen UID:
19585
•Concept ID:
C0034151
•
Disease or Syndrome
Synonyms: Benign Hyperglobulinemic Purpura of Waldenström; Hyperglobulinemic Purpura; Hyperglobulinemic Purpura of Waldenström; Hyperglobulinemic Purpura, Waldenstrom; Hyperglobulinemic Purpuras; Purpura, Hyperglobulinemic; Purpura, Waldenstrom Hyperglobulinemic; Purpuras, Hyperglobulinemic; Waldenstrom Hyperglobulinemic Purpura; Waldenström Hyperglobulinemic Purpura
SNOMED CT: Hyperglobulinemic purpura (402852007)
 
Monarch Initiative: MONDO:0006792

Definition

Purplish or brownish red discoloration of the skin associated with increase in circulating polyclonal globulins, usually gamma-globulins. This syndrome often occurs on the legs of women aged 20 to 40 years. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHyperglobulinemic purpura

Professional guidelines

PubMed

Hyperglobulinemic purpura; report of 14 cases fulfilling Waldenstrom's criteria.
WILSON SJ, BOLINGER RE
J Kans Med Soc 1957 Mar;58(3):166-9. PMID: 13406348

Recent clinical studies

Etiology

Hypergammaglobulinemic purpura of Waldenström in children.
Theisen E, Lee DE, Pei S, Schauder DM, Chiu YE, Brandling-Bennett H, Curran ML, Klein-Gitelman M, Co DO, Arkin LM
Pediatr Dermatol 2020 May;37(3):467-475. Epub 2020 Feb 27 doi: 10.1111/pde.14120. PMID: 32104943
Hypergammaglobulinemic purpura of Waldenström.
Frankel A, Ingraffea A, Massé M, Zhou H
Cutis 2010 Jul;86(1):23-4. PMID: 21049762
IgG2 deficiency in primary Sjögren's syndrome and hypergammaglobulinemic purpura.
Eriksson P, Almroth G, Denneberg T, Lindström FD
Clin Immunol Immunopathol 1994 Jan;70(1):60-5. doi: 10.1006/clin.1994.1011. PMID: 8261670
Hypergammaglobulinemic purpura of Waldenström. (A report of serologic and immunogenetic studies and long-term follow-up in 18 patients).
Invernizzi F, Cattaneo R, Fenini MG, Carella G, Granatieri C, Savi M, Zanussi C
Ric Clin Lab 1988 Jan-Mar;18(1):23-36. doi: 10.1007/BF02918816. PMID: 3259714
Benign hyperglobulinemic purpura of Waldenström: a report of seven patients and long-term follow-up.
Lozada JA, Maldonado N, Sánchez J, Fernández C, Cortés M
Bol Asoc Med P R 1988 Dec;80(12):455-9. PMID: 3233123

Diagnosis

Hypergammaglobulinemic purpura of Waldenström.
Lewin JM, Hunt R, Fischer M, Kamino H, Franks AG Jr
Dermatol Online J 2012 Dec 15;18(12):2. PMID: 23286792
Cryoglobulinemic purpura in visceral leishmaniasis.
Rizos E, Dimos G, Liberopoulos EN, Elisaf MS, Drosos AA
Rheumatol Int 2005 Aug;25(6):469-71. Epub 2004 Nov 10 doi: 10.1007/s00296-004-0533-2. PMID: 16133583
Hypergammaglobulinemic purpura in cystic fibrosis.
Nielsen HE, Lundh S, Jacobsen SV, Høiby N
Acta Paediatr Scand 1978 Jul;67(4):443-7. doi: 10.1111/j.1651-2227.1978.tb16351.x. PMID: 307897
Purpura hyperglobulinemica (Waldenstrom).
Keyhani MH, Veghar E, Akhoundzadeh H, Moghtader R
Acta Med Iran 1976;19(4):267-75. PMID: 1052727
NOT ALL ARTHRITIS IS RHEUMATOID.
DAVIS PL, DAVIS MS
Clin Med (Northfield) 1964 May;71:833-7. PMID: 15446159

Therapy

Refractory primary Sjögren syndrome successfully treated with bortezomib.
Jakez-Ocampo J, Atisha-Fregoso Y, Llorente L
J Clin Rheumatol 2015 Jan;21(1):31-2. doi: 10.1097/RHU.0000000000000210. PMID: 25539431
Cryoglobulinemic purpura in visceral leishmaniasis.
Rizos E, Dimos G, Liberopoulos EN, Elisaf MS, Drosos AA
Rheumatol Int 2005 Aug;25(6):469-71. Epub 2004 Nov 10 doi: 10.1007/s00296-004-0533-2. PMID: 16133583
Immunoblastic lymphadenopathy with purpura and cryoglobulinemia.
Wechsler HL, Stavrides A
Arch Dermatol 1977 May;113(5):636-41. PMID: 301009
Purpura hyperglobulinemica (Waldenstrom).
Keyhani MH, Veghar E, Akhoundzadeh H, Moghtader R
Acta Med Iran 1976;19(4):267-75. PMID: 1052727
Benign hypergammaglobulinemic purpura of Waldenström.
Kyle RA, Gleich GJ, Bayrid ED, Vaughan JH
Medicine (Baltimore) 1971 Mar;50(2):113-23. doi: 10.1097/00005792-197103000-00003. PMID: 4944119

Prognosis

Hypergammaglobulinemic purpura of Waldenström in children.
Theisen E, Lee DE, Pei S, Schauder DM, Chiu YE, Brandling-Bennett H, Curran ML, Klein-Gitelman M, Co DO, Arkin LM
Pediatr Dermatol 2020 May;37(3):467-475. Epub 2020 Feb 27 doi: 10.1111/pde.14120. PMID: 32104943
Refractory primary Sjögren syndrome successfully treated with bortezomib.
Jakez-Ocampo J, Atisha-Fregoso Y, Llorente L
J Clin Rheumatol 2015 Jan;21(1):31-2. doi: 10.1097/RHU.0000000000000210. PMID: 25539431
Hypergammaglobulinemic purpura of Waldenström. (A report of serologic and immunogenetic studies and long-term follow-up in 18 patients).
Invernizzi F, Cattaneo R, Fenini MG, Carella G, Granatieri C, Savi M, Zanussi C
Ric Clin Lab 1988 Jan-Mar;18(1):23-36. doi: 10.1007/BF02918816. PMID: 3259714
Benign hyperglobulinemic purpura of Waldenström: a report of seven patients and long-term follow-up.
Lozada JA, Maldonado N, Sánchez J, Fernández C, Cortés M
Bol Asoc Med P R 1988 Dec;80(12):455-9. PMID: 3233123
Purpura hyperglobulinaemia in association with rheumatoid arthritis and ulcerative colitis.
Wardle EN
Postgrad Med J 1968 Jun;44(512):443-6. doi: 10.1136/pgmj.44.512.443. PMID: 5665743Free PMC Article

Clinical prediction guides

Refractory primary Sjögren syndrome successfully treated with bortezomib.
Jakez-Ocampo J, Atisha-Fregoso Y, Llorente L
J Clin Rheumatol 2015 Jan;21(1):31-2. doi: 10.1097/RHU.0000000000000210. PMID: 25539431
IgG2 deficiency in primary Sjögren's syndrome and hypergammaglobulinemic purpura.
Eriksson P, Almroth G, Denneberg T, Lindström FD
Clin Immunol Immunopathol 1994 Jan;70(1):60-5. doi: 10.1006/clin.1994.1011. PMID: 8261670
Peripheral neuropathy in monoclonal gammapathy with cryoglobulinemia and arteritis.
Häggblom O, von Schéele C
Acta Med Scand 1979;205(3):247-9. doi: 10.1111/j.0954-6820.1979.tb06039.x. PMID: 218428
Clinical and immunologic observations in hypergammaglobulinemic purpura.
Capra JD
Mt Sinai J Med 1971 Jul-Aug;38(4):375-8. PMID: 5314783
Some observations on hyperglobulinemic purpura (Waldenstrom's syndrome).
SYMON WE, ROHN RJ, BOND WH
Am J Med Sci 1957 Aug;234(2):160-9. doi: 10.1097/00000441-195708000-00006. PMID: 13444268

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