Title : Disseminated fusariosis - Management conundrum - A fatal case in an immunocompromised patient
Abstract:
Disseminated fusariosis is a rare but often fatal opportunistic fungal infection that predominantly affects immunocompromised individuals with a mortality rate of over 80%. Prognosis is determined by the degree of immunosuppression and extent of infection.
A 55-year-old caucasian female was admitted to the haematology unit after seeing her GP for fatigue, bruising, recurrent pyrexia and mouth ulcers. She was found to be profoundly cytopenic hence requiring admission and further investigations. The haematology team diagnosed this patient with aplastic anaemia following a bone marrow aspirate. She was commenced on a prophylactic course of acyclovir, levofloxacin and posaconazole. She developed a suspected drug rash shortly thereafter which was attributed to the posaconazole and therefore this was switched to fluconazole resulting in a resolution of the rash.
The underlying cause of the aplastic anaemia was unclear prompting further investigations including an autoimmune panel which revealed a positive ANA and dsDNA, positive crithidia with normal complement levels (C3 and C4). An early CT thorax, abdomen and pelvis showed no acute abnormal findings except for a large pericardial effusion which was aspirated showing macrophages and no evidence of malignancy. The patient was treated with a short course of high dose oral steroids (60mg once daily) by the rheumatology team for suspected systemic lupus erythematosus. Following the completion of the one-week course of oral steroids, an acute rash was observed with erythematous papules, nodules with areas of central necrosis and haemorrhagic crust.
A skin biopsy was performed revealing the presence of short, branching, septate fungal hyphae within the superficial and mid-dermis. On deeper sectioning, there were occasional vessels within the superficial dermis showing fibrin thrombi and possible infiltration by fungal hyphae. The patient was commenced on high dose antifungal - Amphotericin B (5mg/kg) following involvement of the microbiology team. A tissue culture confirmed the presence of Fusarium species which was resistant to all anti-fungals except for Amphotericin B.
Despite two weeks of treatment with high-dose Amphotericin B, the patient continued to develop new cutaneous lesions. She was persistently cytopenic and the rheumatology team intermittently treated her with low doses of methylprednisolone to try and treat her underlying likely autoimmune-related cytopenia. A further skin biopsy revealed fungal hyphae in the dermis once more with evidence of angio-invasion. The patient deteriorated with systemic sepsis and required ITU admission and despite inotropic support, treatement with IVIG and GCSF, she did not recover and subsequently died from systemic disseminated fusariosis.
This case highlights the rare but serious complication of disseminated fungal infections and the complexity of treating such in a patient with suspected systemic lupus erythematosus (SLE) and confirmed aplastic anaemia.
