Nuno V. Vieira e Brito, Portugal
Centro Hospitalar Lisboa Norte Medicina IAPresenter of 2 Presentations
A CASE OF PARANEOPLASIC ANCA VASCULITIS
Abstract
Background and Aims
Vasculitis as a paraneoplasic syndrome is rare and the pathofiosology is yet unclear. Association between ANCA vasculitis and solid tumour is an even rarer condition with very few cases in literature.
Methods
73 year old woman admitted for vision loss with migraine headache, weight loss, asthenia and low fever. She had diabetes, arterial hypertension, prothrombin gene mutation and aortic and mitral valve biological prosthesis. Ophthalmology suggested isquemic optic neuropathy and was referred to internal Medicine.
Results
She was anaemic, high erythrocyte sedimentation rate, positive rheumatoid factor with normal complement and negative HIV and hepatitis. Doppler of the temporal, facial and occipital arteries showed a thickness without halo and biopsy was absent for giant cells. Serologies were positive for C-ANCA antibodies with anti-PR3 of 361. Methylprednisolone was given with partial recuperation of sight. Biopsy of nose turbinate and CT scan of sinus and thorax where absent for granuloma. The patient responded well lowering the erythrocyte sedimentation rate and continued improvement of sight but without lowering of C-ANCA title, 621 at a second evaluation. After discharge was readmitted for myocardial infarction this admission was complicated by endocarditis to streptococcus oralis and hematochezia. Colonoscopy revealed adenocarcinoma and underwent hemicolectomy with adenectomy after undergoing 2 cycles of chemotherapy and suspending all steroids there was no evidence of vasculitis with a lowering of the antibody title to 300.
Conclusions
The authors present a case of a tumor induced vasculitis whose diagnosis precedes that of the tumor and whose symptoms and antibody titer improve only with tumor control and treatment.
AN IDIOPATHIC CASE OF COMBINED WARM AND COLD AIHA
Abstract
Background and Aims
Autoimmune hemolytic anemia (AIHA) is usually either warm or cold. In some rare cases patients present with both autohemagglutinins. In this text authors report a case of a male presenting with idiopathic combined warm and cold AIHA which had complete resolution of the anemia following treatment with corticosteroids
Methods
An 81-year-old male was referred to the emergency department (ED) by his primary care physician for asthenia, vomiting, upper abdominal pain and darkened urine in the past 2 months
Results
He presented hemoglobin of 7,1g/dL, leukocytosis, hyponatremia increased total bilirubin. Urine elevated urobilinogen. Worsening anemia with hemoglobin 5,4 g/dL, thrombocytosis, increased LDH and total bilirubin and unmeasurable haptoglobin. Peripheral blood smear showed anisocytosis. Positive DAT test (IgG1/IgG3 and C3d positive) without specificity and cold antibody, anti-I specific with 16 titer. Secondary causes wielded positive serologies for Chlamydia Pneumoniae. Serum protein electrophoresis demonstrated slight polyclonal hypergammablobulinemia, immunofixation demonstrated slight oligoclonal profile with increased lambda chains, urine immunofixation without Bence-Jones protein. No other test results were altered including normal bone marrow biopsy and autoimmune screening. Prednisolone was administered with resolution of hemolysis. At 4 weeks no increase in titer was noted on C. Pneumoniae serologies and presents resolution of hemolysis under no treatment.
Conclusions
An increasing number of mixed or combined warm and cold AIHA cases have been reported in the last few decades. Patients presenting with this disease have been described as severely anemic and having very good response to corticosteroid therapy. In accordance we report another case in which an idiopathic form of this rare disease presented itself in our practice.