Immunoglobulin G4-Related Disease Accompanied by Primary Myelofibrosis: Case Report

Frontiers in Medicine

Jun Feng¹  Wen Zhang²  Mengtao Li²  Xiaofeng Zeng²  Shangzhu Zhang²  Ziwei Liu² 

[1] Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China;Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China;

关键词: immunoglobulin G4–related disease;    primary myelofibrosis;    acute myeloid leukemia;    lymphadenopathy;    myeloproliferative neoplasm;   

DOI  :  10.3389/fmed.2021.638794

来源: Frontiers

【 摘 要 】

Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous autoimmune fibrosing disorder that presents common pathologic features but with unclear etiology. We report a rare case of IgG4-RD accompanied by primary myelofibrosis that eventually transformed into acute myeloid leukemia. A 50-year-old woman suffered from progressive lacrimal and parotid gland enlargement, diaphoresis, and rapid weight loss. Important clinical findings included remarkable leukocytosis, hyperglobulinemia, and splenomegaly. IgG4-RD was confirmed by salivary gland biopsy. Meanwhile, myelofibrosis was diagnosed according to histopathological findings of bone marrow and genetic mutation test of peripheral blood. The patient was on corticosteroid treatment. However, she developed into acute myeloid leukemia (AML) in the 8th month of follow-up. Our case suggested that myeloproliferative neoplasm (MPN) may co-occur with IgG4-RD. Bone morrow aspiration and genetic tests are helpful for throughout evaluation. An active search for hematological malignancies is warranted at diagnosis and during follow-up for patients who present with unexplained leukocytosis, pancytopenia, splenomegaly, or weight loss.

【 授权许可】

CC BY