Mikulicz’s disease with severe thrombocytopenia following autologous stem cell transplantation in a multiple myeloma patient

Sakurai, Chihiro; Ohashi, Kazuteru; Sakaguchi, Kyogo; Hishima, Tsunekazu; Kamata, Noriko; Akiyama, Hideki; Sakamaki, Hisashi

doi:10.1007/s12185-009-0428-9

Cited by 2 publications

(3 citation statements)

References 13 publications

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“…10,16 In addition, impaired immunity due to autologous transplantation might also account for autoimmune thrombocytopenia. 3 In summary, this novel study described a rare case of IgG4-related MD complicated with autoimmune thrombocytopenia without any hematologic malignancies. Although IgG4-related MD had a favorable response to steroid therapy, the steroid dose was recommended to be high and immunosuppressive agents helped in reducing the dose of steroids for the patient suffering from IgG4-related MD with thrombocytopenia.…”

Section: Discussionmentioning

confidence: 76%

“…In that case, the patient developed severe thrombocytopenia after autologous PBSCT for MM. 3 This study reported the first case of IgG4-related MD with immune thrombocytopenia without hematologic malignancies. In the present case, the diagnosis of IgG4-related MD was confirmed by the pathological examination of submandibular glands.…”

Section: Discussionmentioning

confidence: 82%

“…One reason for thrombocytopenia might be the presence of anti-PLT antibody. 10,14 or IgG subclass (IgG1/3/4) of PLT antibody 3,13,[15][16][17] or autoantibody against a disintegrin and metalloproteinase with thrombospondin motifs 13 18,19 Another reason is the H. pylori infection that could lead to a decrease in PLT count. 10,16 In addition, impaired immunity due to autologous transplantation might also account for autoimmune thrombocytopenia.…”

Section: Discussionmentioning

confidence: 99%

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Mikulicz’s Disease With Immune Thrombocytopenia: A New Immunoglobulin G4-Mediated Disorder and Literature Review

Cong

Chen

et al. 2019

Arch Rheumatol

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Immunoglobulin G4 (IgG4)-related Mikulicz's disease (MD) is an immune-mediated fibroinflammatory condition of unknown etiology characterized by the swelling of multiple exocrine glands including lacrimal, salivary, and parotid glands. 1,2 Immune thrombocytopenia is common in many autoimmune diseases. However, IgG4-related MD is rarely associated with immune thrombocytopenia and its pathogenic mechanism remains largely unclear. To our knowledge, only one case has been reported to date in the literature. In that case, the patient developed severe thrombocytopenia after autologous peripheral blood stem cell transplantation (PBSCT) for multiple myeloma (MM). 3 This novel study aims to report the case of a 48-year-old male patient suffering from MD with severe immune thrombocytopenia without hematological malignancies and review all reported cases of IgG4-related disease (IgG4-RD) with thrombocytopenia. CASE REPORT A 48-year-old male patient was admitted to our department with enlarged lacrimal and parotid glands associated with thrombocytopenia. He underwent resection for bilateral swelling of submandibular glands three years ago. The pathological examination revealed no tumor cells or focal lymphocytic infiltration. However, he repeatedly had enlarged lacrimal and parotid glands and dry mouth, without dry eye or arthralgia. He was then diagnosed with Sjögren syndrome (SS) and received anti-rheumatic treatment with hydroxychloroquine and Tripterygium wilfordii (a traditional Chinese medicine having definite effects on autoimmune and inflammatory diseases) for two years. However, the patient suffered from repeated recurrences of enlarged glands and presented with thrombocytopenia after two years. Physical examination revealed nontender bilateral swelling of lacrimal and ABSTRACT Mikulicz's disease (MD), an immunoglobulin G4 (IgG4)-related disease, is a fibrosis-associated inflammatory disease characterized by painless bilateral swelling of lacrimal and salivary glands. Although thrombocytopenia is associated with many rheumatologic disorders, it is rare in IgG4-related MD. This novel study aims to report a rare case of IgG4-related MD complicated with severe autoimmune thrombocytopenia without any hematological malignancies and review all cases of IgG4-related disease associated with thrombocytopenia in the literature.

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Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 82%