A young woman with steroid‐responsive, IgG4‐positive plasma cell‐enriched cystic lymphangioma and chylous ascites

Zaki, Alaa Al; Mann, Shawna; Carruthers, Mollie; Slack, Graham W.; Chen, Luke Y C

doi:10.1002/ccr3.1536

Cited by 2 publications

(2 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, the pathological examination did not reveal any fibrosis or obliterative phlebitis, and the authors concluded that their patient did not have IgG4-RD. 18 Both retroperitoneal fibrosis and lymphadenopathy have been observed in IgG4-RD, 19 but cystic forms of retroperitoneal fibrosis are an absolute rarity; only three of Length of lower image border: 2.33 mm (a, b) or 0.23 mm c, d).…”

Section: Discussionmentioning

confidence: 99%

“…However, the pathological examination did not reveal any fibrosis or obliterative phlebitis, and the authors concluded that their patient did not have IgG4-RD. 18 Both retroperitoneal fibrosis and lymphadenopathy have been observed in IgG4-RD, 19 but cystic forms of retroperitoneal fibrosis are an absolute rarity; only three of these cases are described in the literature, 20,21 and only one of them was punctured with evidence of chylous fluid. Fibrosis is more likely to cause a hydrocele or varicocele testis as a consequence of gonadal vessel involvement.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

Liedtke

Käding

Döring

et al. 2021

SAGE Open Medical Case Reports

View full text Add to dashboard Cite

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.

show abstract

Section: Discussionmentioning

confidence: 99%

“…However, the pathological examination did not reveal any fibrosis or obliterative phlebitis, and the authors concluded that their patient did not have IgG4-RD. 18 Both retroperitoneal fibrosis and lymphadenopathy have been observed in IgG4-RD, 19 but cystic forms of retroperitoneal fibrosis are an absolute rarity; only three of these cases are described in the literature, 20,21 and only one of them was punctured with evidence of chylous fluid. Fibrosis is more likely to cause a hydrocele or varicocele testis as a consequence of gonadal vessel involvement.…”

Section: Discussionmentioning

confidence: 99%

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

Liedtke

Käding

Döring

et al. 2021

SAGE Open Medical Case Reports

View full text Add to dashboard Cite

show abstract

Post-Transfusion Hemophagocytosis Without Hemophagocytic Lymphohistiocytosis

McGinnis

Medvedev

Richards

et al. 2019

Mayo Clinic Proceedings: Innovations, Quality & Outcomes

View full text Add to dashboard Cite

Hemophagocytosis refers to ingestion of hematopoietic elements or mature blood cells by another cell, typically by cells conventionally associated with phagocytic capacity. Although the finding of hemophagocytosis as a prominent feature in a patient's bone marrow might prompt consideration of a hemophagocytic syndrome (HPS) such as hemophagocytic lymphohistiocytosis (HLH) in a clinician's or pathologist's differential diagnosis, this morphologic feature can be nonspecific in the absence of other clinical and laboratory features of pathologic immune activation, which is the sine qua non of HPS/HLH. We describe three patients whose clinical presentations included transfusion-dependent anemia and whose bone marrow aspirates showed unexpectedly brisk hemophagocytosis of mature red blood cells. Despite striking morphologic hemophagocytosis, no patient met criteria for diagnosis of an HPS. Transfusion-associated hemophagocytosis and hyperferritinemia must be carefully distinguished from HLH through clinical and laboratory assessment. Biomarkers of pathologic immune activation are important diagnostic aids.

show abstract

A young woman with steroid‐responsive, IgG4‐positive plasma cell‐enriched cystic lymphangioma and chylous ascites

Cited by 2 publications

References 8 publications

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

Post-Transfusion Hemophagocytosis Without Hemophagocytic Lymphohistiocytosis

Contact Info

Product

Resources

About