Objective: Nearly half of the cases of autoimmune haemolytic anaemia (AIHA) are associated with an underlying disorder that leads to immune dysregulation, and malignancies is one of them. Although AIHA is reported in patients with a wide range of haematological malignancies, most frequently in Chronic Lymphocytic Leukameia and Non-Hodgkin Lymphoma, only 1–2% are associated with solid organ malignancy. This case report highlights malignant peritoneal mesothelioma as a rare cause of autoimmune haemolytic anaemia.
Case report: We report a case of a twenty-nine year old female who initially presented to her general practitioner with a six month history of symptoms suggestive of irritable bowel syndrome. Her blood count identified a significant anaemia (haemoglobin 53g/L) and thrombocytosis (platelets 1260×109/L), and was thus referred to haematology clinic. She was diagnosed with IgG-C3d AIHA. The patient was started on prednisolone 1mg/kg with a good initial response. To investigate the underlying cause, a whole body CT scan was performed, which identified significant abdominal ascites. Serum CA-125 was raised at 6715U/mL (range 0–35) and paracentesis revealed an LDH of 1203 SU suggesting underlying malignancy, but no malignant cells were found on the ascitic fluid cytology. The patient went on to have a PET scan, which confirmed FDG avid serosal disease, with update in the liver, omentum and peritoneum. Diagnostic laparotomy revealed widespread nodules on all serosal surfaces, and the biopsy confirmed a diagnosis of peritoneal epithelioid malignant mesothelioma. Whilst the patient had her workup with the oncology team, her AIHA became refractory to steroid treatment, and was commenced on Rituximab at 375mg/m2 weekly infusions. The patient did not respond to 4 doses of Rituximab, and continued to require regular transfusion support. She eventually started chemotherapy for the mesothelioma, which reduced the briskness of haemolysis, and reduced transfusion requirements; although haemolysis did not completely cease.
Conclusion: To our knowledge, this is the third case of AIHA with malignant peritoneal mesothelioma reported in literature. There is currently no established treatment for AIHA associated with solid organ malignancy. This case highlights the poor response to standard treatments, and only a partial response to the definitive treatment for the underlying malignancy.
