Aseptic Abscess Syndrome in Hematological Malignancies: Case Reports and Insights

Background: Aseptic abscess syndrome (AAS) is an atypical clinical entity associated with systemic inflammatory conditions that manifest as sudden onset fevers and painful abscesses without an underlying microbiologic etiology. This syndrome is rare, with only 61 published cases of this inflammatory condition as of 2019[i]. This abstract will discuss two of the first case reports of patients with acute myeloid leukemia (AML) and primary myelofibrosis who developed aseptic abscesses.

Cases: The first patient is a 66-year-old male with AML. Molecular studies were positive for mixed-lineage leukemia (MLL) and isocitrate dehydrogenase 1 (IDH1) mutations, and the patient was treated with a cycle of venetoclax and azacitidine but was lost to follow-up. Months later, the patient presented again, complaining of facial/ear pain and a new submandibular swelling. Computed Tomography (CT) neck was performed, which showed multiple cystic and necrotic neck masses. On presentation, the patient was febrile with a white cell count of 2.7 ×10⁹ /L. He was trialed on multiple antibiotic regimens without success and underwent numerous incisions and drainages, which yielded purulent fluid with moderate neutrophils but negative cytology. Steroids were started which caused significant improvement in his condition. As all blood and tissue cultures remained negative the patient's condition and fever improved after receiving steroids, and, a diagnosis of AAS was suspected.

The second patient is a 69-year-old male with primary myelofibrosis. After months of ruxolitinib treatment and six rounds of splenic radiation, the patient underwent a sibling-matched allogeneic stem cell transplant, complicated by delayed engraftment with 80% chimerism. Unfortunately, the chimerism declined to 5% despite restarting ruxolitinib and hydroxyurea. A few weeks later, the patient began experiencing severe shoulder and penile pain extending posteriorly to the anus. CT abdomen and pelvis showed a 7cm abscess in the dorsum of the penis, and an MRI of his left shoulder showed several fluid collections/abscesses. No growth was observed in blood, urine, or tissue cultures. Over four weeks, incisions and drainages were performed with moderate neutrophils and negative cytology. The patient received seven different antibiotics; however, no clinical or laboratory response was achieved. A diagnosis of AAS was not considered at the time, although in retrospect, we identified this case as similar to Case #1.

Conclusion: Ultimately, the emergence of AAS within hematological malignancies presents a perplexing challenge for clinicians and highlights the need to consider this diagnosis in patients with sterile abscesses that do not improve with antibiotic therapy. In these patients, we recommend quickly obtaining bacterial, fungal, and acid-fast bacilli cultures and initiating broad-spectrum antibiotics. If both blood and tissue cultures return negative, we recommend starting high-dose steroids and monitoring for improvement. With refractory treatment, we recommend considering steroid-sparing agents for treatment-resistant cases or beginning chemotherapy treatment for the underlying malignancy. Given the uniqueness of this condition, physicians must utilize laboratory and imaging findings, a thorough clinical history, and epidemiologic data to ensure the best possible outcome.

[i] Elessa D, Thietart S, Corpechot C, et al.. TNF-α antagonist infliximab for aseptic abscess syndrome. Presse Med 2019;48:1579-80. 10.1016/j.lpm.2019.09.056

No relevant conflicts of interest to declare.