AML-215: Successful Management of an Unusual Fournier’s Gangrene Secondary to Post-Transplant AML

Abstract

Fournier's Gangrene is known to be one of the rarest and potentially lethal necrotizing infections of the perineum and genital region with high mortality. We report an unusual case of a 34-year-old male presenting to Saint Antoine Hospital in June 2020 after 6 months of severe pain, a mass in the anal and perineal area with blackish discoloration, and painful swelling of the scrotum and the penis. A likely diagnosis of necrotizing fasciitis associated with high-grade fever and sepsis was made. An immediate surgical debridement, colostomy, and suprapubic catheter were done, along with broad-spectrum antibiotics. Per medical history, in 1999, he had an amputation of the left foot after diagnosis with osteosarcoma and lung metastasis, followed by chemotherapy. A new evaluation was done in 2003 and showed progressive lung lesions treated with right lobectomy. In January 2010, the patient presented a blood test with pancytopenia and 14% of circulating blasts. Bone marrow aspiration (BMA) was done and showed an AML secondary to chemotherapy (t-AML) with complex karyotype of chromosome 5 and chromosome 17. Induction chemotherapy with 4 cycles of Vidaza + Revlimid from January 2010 to April 2010 led to partial response. In May 2010, bone marrow biopsy showed signs of dysmyelopoiesis with young elements of a dispersed blastic appearance of myeloid origin without excess blasts and no signs of osteosarcoma. In June 2010, the patient had a successful haploidentical allotransplant from his brother. After presenting with Fournier's Gangrene in June 2020, his blood tests showed anemia (Hb 8.6g/dL), thrombocytopenia (127g/L), and blast cells (12%). Biopsies of the genital mass showed diffuse proliferation of malignant cells, and immunohistochemistry tests came back as CD34+, CD117+, MPO-, Glycophorine +/-, CD68-/+, ACL-/+, CD163-, AE1/AE3-, Ki67%, and EBER-. BMA showed a rich marrow invaded by 50% of blasts with a cytologic aspect of AML. After controlling the Fournier's Gangrene by surgery and antibiotics, the patient was started on a new line of treatment with Vidaza/Venetoclax (July 2020). A new BMA done in September showed a complete biologic response with 1% blasts. The decision was made to continue 4 cycles of chemotherapy. The recovery phase was uneventful with satisfactory cosmetic results.