Hypergammaglobulinemic purpura of Waldenström

Lewin, John M.; Hunt, Raegan; Fischer, Max; Kamino, Hidelo; Franks, Andrew G

doi:10.5070/d300c6t853

Cited by 6 publications

(7 citation statements)

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“…A more specific feature may be the finding of immune complexes including IgG and IgA RF, which are believed to be involved in the development of the disease. 8 Although our patient had a negative RF, standard RF assays only assess for the presence of IgM RF and do not detect for IgG or IgA RFs. The pathology of HGPW may reveal mild perivascular lymphocytic infiltrate and erythrocyte extravasation, or LCV as was seen in the present case (Figure 2).…”

Section: Discussionmentioning

confidence: 71%

“…Many cases are idiopathic, but associations include autoimmune, granulomatous, and hematological disorders as well as lymphoma and myeloma. [3][4][5]8 Therefore, screening for underlying disorders at the initial presentation of HGPW is important. Henoch-Schonlein purpura 14 Lymphoma 8 Myeloma 3-5 Rheumatoid arthritis 6 Sarcoidosis [14][15][16][17] Sjögren's syndrome 6,7 Systemic lupus erythematosus 12,13 Tuberculosis 14,18…”

Section: Discussionmentioning

confidence: 99%

“…Many cases are idiopathic, but associations include autoimmune, granulomatous, and hematological disorders as well as lymphoma and myeloma. 3–5,8 Therefore, screening for underlying disorders at the initial presentation of HGPW is important.…”

Section: Discussionmentioning

confidence: 99%

“…1 Sjögren syndrome is the commonest association; however, rare occurrences of lymphoma or myeloma have been reported. 1,3–8 A common link between these disease entities is polyclonal immunoglobin formation with immune complex deposits in blood vessels of the lower limbs. 1 The differential of HGPW includes non-inflammatory purpuras like pigmented purpuric dermatosis, as well as other forms of LCV.…”

Section: Introductionmentioning

confidence: 99%

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Hypergammaglobulinemic purpura of Waldenström—Unusual and impressive case in a patient with myeloma: A case report

DeBiasio

Cyr

Petkiewicz

et al. 2022

SAGE Open Medical Case Reports

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Background: Hypergammaglobulinemic purpura of Waldenström is an uncommon disease, which presents mostly in women on the lower extremities. It is sometimes associated with underlying immune dysregulation. Sjögren syndrome is the most common association; however, rare occurrences of the self-resolving syndrome with lymphoma or myeloma have been reported. Case Summary: We describe an unusual and impressive presentation of hypergammaglobulinemic purpura of Waldenström in an elderly female patient with myeloma. Notably, the patient did not have any concurrent connective tissue diseases. Despite her florid presentation, her hypergammaglobulinemic purpura of Waldenström spontaneously resolved within a few days. Conclusion: Hypergammaglobulinemic purpura of Waldenström is a self-resolving but recurrent syndrome, which may be associated with autoimmune disorders or rarely myeloma. Early diagnosis of the syndrome may avoid unnecessary treatment interventions and should prompt screening for underlying diseases.

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Section: Discussionmentioning

confidence: 71%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Hypergammaglobulinemic purpura of Waldenström—Unusual and impressive case in a patient with myeloma: A case report

DeBiasio

Cyr

Petkiewicz

et al. 2022

SAGE Open Medical Case Reports

View full text Add to dashboard Cite

show abstract

“…Some argue that small circulating immune complexes may play an important role. 1 When an underlying cause cannot be found out, it is considered as primary hypergammaglobulinemic purpura of Waldenström, even some of them could develop features of autoimmune disease during the course of the disease. The most common association is Sjögren's syndrome and occasionally rheumatoid arthritis, lupus erythematosus, and hematologic malignancy.…”

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confidence: 99%