Tofacitinib
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Thrombocytopenia with TAFRO syndrome-like features : case report A 74-year-old woman developed severe thrombocytopenia with thrombocytopenia, anasarca, fever, renal insufficiency and organomegaly (TAFRO) syndrome-like features during treatment with tofacitinib for rheumatoid arthritis (RA). The woman was hospitalised due to fever and thrombocytopenia for 1 month. She had been diagnosed with RA at the age of 69 years, for which she had subsequently started receiving tofacitinib [route and dosage not stated]. She had been in remission for several months. Her vital signs on admission were unremarkable. Physical examination showed swollen lymph nodes in her neck and pitting oedema in the legs. Laboratory analyses revealed the following: haemoglobin 8.3 g/dL, platelet count 34 × 103/µL, serum creatinine 0.92 mg/dL and CRP 4.98 mg/dL. Serum anti-nuclear antibody test was found positive, and her anti-centromere, antimitochondrial M2 antibody and anti-RNP tests were also found positive. Urinalysis and screening for infection were unremarkable. A CT scan showed bilateral axillary lymphadenopathy, bilateral pleural effusion, cervical lymphadenopathy, pericardial effusion, small ascites and hepato-splenomegaly. A left axillary lymph node biopsy demonstrated proliferation of small vessels and scattered CD38-positive plasma cells in the interfollicular region and atrophic germinal centres along with small vessels entering the germinal centers, without findings suggestive of malignant lymphoma. These findings were consistent with the histology of mixed-type Castleman’s disease. Bone marrow biopsy showed no specific findings. Her clinical presentation (thrombocytopenia, anasarca, elevated CRP and creatinine level, fever, hepato-splenomegaly and lymphadenopathy) and the histological findings of the axillary lymph node were consistent with TAFRO syndrome [duration of treatment to reaction onset not stated]. Additional laboratory analyses revealed serum interleukin-6 levels of 12 pg/mL and vascular endothelial growth factor levels of 67 pg/mL. The woman started receiving oral prednisolone 50 mg/day, after which the anasarca and systemic inflammation rapidly resolved, with dramatical improvement in fatigue. Her platelet count initially recovered, but decreased when the dose of prednisolone was tapered. Therefore, eltrombopag and tacrolimus were added. Thereafter, her platelet levels gradually increased and she was discharged. However, at the time of her first outpatient visit post discharge, she developed steroid psychosis. Hence, prednisolone was rapidly tapered. Two months later, her platelet count decreased to 49 × 103/µL. She was readmitted for further examination and treatment. During the second admission, laboratory analyses revealed thrombocytopenia, microcytic anaemia and slightly elevated CRP levels. A CT scan showed only mild cervical lymphadenopathy and splenomegaly. Pleural effusion, pericardial effusion and ascites were not observed. Reinduction with high-dose prednisolone was deferred due to the steroid psychosis,
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