2021
DOI: 10.2169/internalmedicine.6468-20
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Aplastic Anemia in a Patient with Cronkhite-Canada Syndrome

Abstract: Cronkhite-Canada syndrome (CCS) is a rare polyposis disorder accompanied by alopecia and onychodystrophy. A 63-year-old man with a history of CCS and repeated embolism developed progressive thrombocytopenia and mild anemia. Laboratory testing, a bone marrow examination, and magnetic resonance imaging of the spine resulted in a diagnosis of concurrent aplastic anemia (AA). Paroxysmal nocturnal hemoglobinuria (PNH)-type cells were detected in a peripheral blood specimen. In addition, human leukocyte antigen (HLA… Show more

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Cited by 4 publications
(2 citation statements)
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“…The etiology and pathogenesis of CCS are still unclear, but it involves genetic abnormalities, abnormal proliferation and differentiation of intestinal epithelial cells, immune system abnormalities and stress. [ 10 ] At present, it is generally considered to be a chronic inflammatory disease associated with autoimmune mechanisms, and the current evidence mainly includes ANA positivity, [ 11 ] the level of IgG4 in the blood circulation was increased, [ 12 ] polyps infiltrated with IgG4-positive plasma cells, [ 13 ] and an overall good clinical response to immunosuppressive therapy. [ 2 ] In addition.…”
Section: Discussionmentioning
confidence: 99%
“…The etiology and pathogenesis of CCS are still unclear, but it involves genetic abnormalities, abnormal proliferation and differentiation of intestinal epithelial cells, immune system abnormalities and stress. [ 10 ] At present, it is generally considered to be a chronic inflammatory disease associated with autoimmune mechanisms, and the current evidence mainly includes ANA positivity, [ 11 ] the level of IgG4 in the blood circulation was increased, [ 12 ] polyps infiltrated with IgG4-positive plasma cells, [ 13 ] and an overall good clinical response to immunosuppressive therapy. [ 2 ] In addition.…”
Section: Discussionmentioning
confidence: 99%
“…CCS is generally considered to be a chronic inflammatory disease associated with autoimmune mechanisms. There is currently significant evidence including ANA-positive, elevated IgG4 levels in blood circulation, polyps infiltrating IgG4-positive plasma cells, and clinically good response to immunosuppressive therapy[ 3 - 5 ]. Currently, there are no internationally recognized diagnostic criteria or systematic treatments.…”
Section: Introductionmentioning
confidence: 99%