Akademik Veri Yönetim Sistemi
IV. International Eurasian Congress of Hematology, Antalya, Türkiye, 9 - 13 Ekim 2013, ss.1-2
Introduction: Rheumatoid arthritis is a chronic inflammatory disease that affects the joints. Secondary amyloidosis is one of the most serious complications of chronic inflammatory diseases. Familial Mediterranean fever is a common and important cause of secondary amyloidosis in Turkey, whereas inflammatory diseases such as rheumatoid arthritis and ankylosing spondylitis, major causes of secondary amyloidosis in Western countries.
The five cardinal signs and symptoms of thrombotic thrombocytopenic purpura are thrombocytopenia, microangiopathic hemolytic anemia, neurological disorders, renal failure and fever. Secondary causes of thrombotic thrombocytopenic purpura are immunological diseases, such as rheumatoid arthritis. Plasmapheresis, in which pathologic plasma is removed by an apheresis device and replaced with a substitution fluid, is the primary treatment modality of thrombotic thrombocytopenic purpura.
Case
Report: 51
year-old female patient with a 20-year history of rheumatoid arthritis was
diagnosed with thrombotic thrombocytopenic purpura. She had proteinuria of 1.96
g/24 hours. The patient was also diagnosed with AA amyloidosis due to
positivity of the Congo red, crystal violet and amyloid A staining results on
the bone marrow biopsy specimen. Intravenous methylprednisolone 80mg/day was
started and 14 sessions of plasmapheresis was performed on a daily basis. At
the end of therapy, the patient was in complete remission. However, she
relapsed after one month. Plasmapheresis was repeated only once and the dose of
oral methyl-prednisolone was increased. Oral cyclophosphamide 50 mg was added
to the existing treatment. The patient has been in remission for ten months
with low-dose oral methylprednisolone and cyclophosphamide Discussion: The
therapy of choice for TTP is plasma exchange with fresh frozen plasma, and more
than 70% of patients show either partial or complete response to the treatment.
On the other hand, 10-20% of patients with relapsed/refractory disease may
remain unresponsive to treatment despite daily plasmapheresis. Patients who do
not respond to treatment in seven days are considered refractory although
remission may occur in 7-20 days. Thus, appropriate therapy including
plasmapheresis should be given at least 1-2 weeks. Immunomodulatory agents or
immunosuppressives such as corticosteroids should be added to treatment if
there is no response. There are some data suggesting that addition of
vincristine to the therapy may increase the remission rate. Moreover, addition
of cyclophosphamide, cyclosporine A, azathioprine to treatment was found to
produce successful results in a
small cohort.
Our case responded well to combination of standard plasmapheresis and MP. Although she developed relapse one month after tapering the dose of MP, one session of plasmapheresis and oral cyclophosphamide plus MP were successful in regaining remission.