A recent case report published in Cureus describes the diagnosis of acute myeloid leukemia (AML) associated with the chronic autoimmune disorder systemic lupus erythematosus (SLE).

“The association of AML-SLE is very rare and simultaneous diagnosis is probably a coincidence,” the authors wrote in their report . “The diagnosis is difficult because of the common hematological abnormalities seen in both diseases.”

Although studies of the association of AML-SLE are limited, increased risk of hematologic malignancies in patients with SLE has been reported previously, with immunosuppressive treatment as the main risk factor.

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The 62-year-old woman was admitted for etiological diagnosis of weight loss (7 kg) and severe anemic syndrome with asthenia, skin pallor, headache, palpitations, and intermittent fever. She had a history of hypertension for 10 years with ischemic heart disease, treated with acetylsalicylic acid, carvedilol and amlodipine, and a history of epilepsy for 15 years, treated with valproic acid and phenobarbital.

The patient’s blood count showed anemia (hemoglobin, 7g/dl), a mean corpuscular volume of 86 fl, reticulocyte rate of 44 000/mm3, thrombocytopenia (platelets, 30 000/mm3), leukopenia (neutrophils, 40/mm3), and lymphocytes at 520/mm3. Blood smear revealed blastoid cells, and subsequent bone marrow aspiration showed 65% myeloid blast cells. The diagnosis of AML was confirmed with immunophenotyping.

SLE with renal involvement was diagnosed according to the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology classification. An anti-nuclear antibodies assay was positive, and typing of the antibodies revealed positive anti-Sjogren’s syndrome type A antigen, anti-Sjogren’s syndrome type B antigen, and anti-DNA antibodies as well as anti-phospholipid antibodies positive for circulating lupus-type antibodies.

“The prognosis of this association is very poor,” the authors wrote. “The main complication and cause of death is infection.”

The therapeutic management of patients with simultaneous diagnosis of AML and SLE is challenging due to multiple comorbidities. In this case, patient was given palliative treatment with a low dose of cytarabine for AML. She was also administered corticosteroid (prednisone, 1 mg/kg/day), without any improvement. The patient died with septic shock 3 months after diagnosis.


Tahri S, Alaoui H, Bachir H, Hamaz S, Serraj K. Acute myeloid leukemia with systemic lupus erythematosus: rare association or coincidence. Cureus. Published online December 26, 2021. doi:10.7759/cureus.20714