A complex case of massive ascites, pleural effusions, and pancytopenia, with low complement levels in a patient with bowel malignancy
Rheumatology, Musgrave Park Hospital.
We present the case of a 78 year old gentleman who presented with a 4 month history of nausea, anorexia, black stools and 10kg weight loss.
Pre-morbidly this gentleman was fit and active. Over a 4 month period he developed the symptoms above and examination revealed bilateral pitting oedema, tender hepatomegaly and tense ascites. Initial investigations revealed a macrocytic anaemia and thrombocytopenia.
Further investigations were carried out, including paracentesis, OGD, Colonoscopy, CT CAP, and PET. There was large volume ascites and pleural and pericardial effusions present. The ascitic fluid had a high protein level; no malignant cells were detected. There was abnormal uptake in sigmoid colon and rectum on PET scan, but appearances at colonoscopy were non-malignant. Biopsy was not possible as platelets had dropped to <10.
Bone marrow biopsy showed marked fat necrosis with almost complete absence of megakaryocytes. Complement levels were low, and speckled ANA positive (low titre 80). ENA/ Anticardiolipin/ Beta 2 glycoprotein/ Lupus anticoagulant/ IgG4/ CEA/ Viral screen negative.
The patient developed foot drop and nerve conduction studies demonstrated a sensorimotor neuropathy causing left common peroneal nerve palsy.
The patient required nutritional and blood product support, and was treated with corticosteroids, rituximab and intravenous immunoglobulins with little clinical response. Under the care of Haematology he received Eltrombopag with the aim of platelet incrementation to facilitate colonic biopsy. Unfortunately this was not successful and the patient requested withdrawal of active treatment.
Post mortem examination revealed a moderately differentiated adenocarcinoma of the colon and gelatinous transformation of the bone marrow. There was marked serositis present, a very unusual histological finding, with no evidence of IgG4 disease. We are not convinced that the bowel malignancy accounts for all of the clinical features. We speculate that he had co-existing seronegative lupus to account for the pancytopenia, low complement levels, marked serositis and peripheral nephropathy.