We presentthe case of a 23 year old female with Systemic Lupus Erethematosus [SLE] from
diagnosis followed for 7 years. She presented unusually, with acute autoimmune
hepatitis that has not recurred. Subsequently she developed significant
haematological involvement with complications of profound thrombocytopenia and
neutropenia. Ultimately she developed a rare malignancy-namely CD8+
epidermotropic T-cell lymphoma. We discuss the complications of SLE and the
development of the cutaneous T cell lymphoma.
A twentythree year old female with no past medical history was referred to the
Rheumatology clinic in 2009 following an acute illness managed by the
Hepatologists. She presented with jaundice and diarrhoea. She had no risk
factors for acute hepatitis and was not taking any hepatotoxic medication. Her
liver enzymes were significantly elevated, suggestive of acute hepatic injury.
Her bloods showed Bilirubin 300umol/L (0-20), ALT 1500IU/L (10-35), ALP 150IU/L
(35-104 INR 1.5 and Albumin was 35g/L (34-50). Further investigations
demonstrated a strongly positive anti-nuclear antibody (titre >1:5120).
Anti-smooth muscle and anti-mitochondrial antibodies were negative. IgG was
elevated at 23.18g/L (7.0-16.0 g/L). HIV and Hepatitis B&C serology was
negative for previous infection. Subsequently a liver biopsy demonstrated
evidence of acute hepatitis, with associated portal and lobular inflammation.
She was treated with a tapering dose of oral prednisolone in combination with
Azathioprine 75mg daily. Shortly thereafter she developed a macular rash,
arthralgia and alopecia with an associated thrombocytopenia and lymphopenia.Further investigations revealed positive antibodies to Ro and
double stranded DNA 208IU/ml (0-50). C3 was low at 0.77g/L (0.90- 1.80). The
ESR was elevated at 66mm/hr. The anti-phospholipid screen was negative with the
exception of an IgM anti-cardiolipin antibody. A diagnosis of Systemic Lupus
Erythematosus (SLE) was made with associated lupus hepatitis.
She responded well to the initial treatment for several months
with evidence of both clinical and serological remission. In early 2010 she
presented again with mucosal bleeding and a petechial rash. Profound
pancytopenia was noted with a platelet count of 0 (150-400 x109/l),
haemoglobin 54 (115-155 g/l) with positive direct antiglobulin test. This flare
was complicated by a retinal haemorrhage resulting in central vision loss. She
underwent a diagnostic Bone Marrow Aspirate and Trephine (BMAT) which
demonstrated red cell aplasia with normal megakaryocytic suggesting periphera destruction. She was treated with intravenous methyl
prednisolone, Cyclophosphamide and Rituximab for lupus-related pancyopenia.
After several weeks her blood counts normalized. Over the next four years she
remained relatively well although she required annual Rituximab infusions
predominantly for mild flares comprising of arthritis, rash and constitutional
symptoms.
In late December 2014, she developed serositis manifesting as
pleuritic chest pain (pleurisy). An echocardiogram demonstrated associated
pericarditis with a small pericardial effusion noted. She was again treated
with high dose intravenous corticosteroids and Rituximab and made a good
clinical recovery.