Connective tissue diseases combined with chronic myelomonocytic leukemia and myelofibrosis: a case report and literature review.

Connective tissue disease (CTD) is one of the common autoimmune diseases (AIDs). Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disease characterized by peripheral blood monocytosis and bone marrow dysplasia, and it is frequently associated with autoimmune complications and secondary myelofibrosis (MF). This article reports a 52-year-old male patient with recurrent arthritis and fever as the main manifestations, for whom CTD was initially considered, and who received hormone and immunosuppressant combination therapy for 2 years 10 months. The patient still suffered from recurrent fever and severe anemia; further bone marrow cytology examinations revealed abnormal bone marrow hematopoiesis, and the final diagnosis was CTD complicated with CMML and MF (MF-3 grade). After the patient was transferred to the hematology department, we adopted the azacytidine + ruxolitinib dual-target regimen to simultaneously target autoimmune inflammation, malignant clonal hematopoiesis, and severe myelofibrosis. After treatment, the patient's fever and other systemic symptoms were significantly relieved, monocytosis was corrected, and severe anemia and thrombocytopenia were markedly improved.