RESUMO
TAFRO syndrome is a rare disorder that manifests as thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. Although this disease often follows a severe clinical course, the cause remains unknown. The coronavirus disease 2019 (COVID-19) pandemic is a major global problem. Vaccination against COVID-19 has been successful; however, there are concerns about severe adverse events. Herein, we report a rare presentation of TAFRO syndrome triggered by the COVID-19 vaccine with a fatal clinical course. A 42-year-old Japanese man presented to our hospital complaining of fever lasting for 2 weeks that occurred a day after receiving the BNT162b2 mRNA (Pfizer-BioNTech) COVID-19 vaccine. The patient had a low platelet count, ascites, reticulin myelofibrosis, renal failure, and lymphadenopathy and was diagnosed with TAFRO syndrome. Despite administering several immunosuppressive drugs, the condition did not improve. The patient repetitively developed and eventually died of bacteremia caused by multidrug-resistant Klebsiella pneumoniae. We highlight the first reported case of TAFRO syndrome after COVID-19 vaccination.
Assuntos
COVID-19 , Hiperplasia do Linfonodo Gigante , Mielofibrose Primária , Adulto , Vacina BNT162 , COVID-19/diagnóstico , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Edema/diagnóstico , Edema/tratamento farmacológico , Febre/tratamento farmacológico , Humanos , Masculino , Mielofibrose Primária/tratamento farmacológico , RNA Mensageiro , Reticulina , Vacinação/efeitos adversosRESUMO
We encountered a case of refractory adult-onset Still's disease (AOSD) with two relapses. Prednisolone and methotrexate were begun as induction therapy, resulting in the patient's first relapse during tapering of prednisolone. After the introduction of tocilizumab, she achieved remission. However, she experienced a second relapse following prednisolone tapering. While lactate dehydrogenase (LDH) levels and white blood cell (WBC) counts increased in both relapses, interleukin-6 (IL-6) suppression resulted in stable C-reactive protein and ferritin levels in the second relapse. A comparison of the two relapses indicated that increases in both WBC counts and LDH levels can aid in the diagnosis of AOSD relapse.
Assuntos
Doença de Still de Início Tardio , Adulto , Feminino , Humanos , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Prednisolona/uso terapêutico , Contagem de Leucócitos , Recidiva , Lactato DesidrogenasesRESUMO
A 62-year-old woman, who had a 16-year history of JAK2V617F-mutated myeloproliferative neoplasm (MPN), developed Burkitt leukemia (BL) 16 months after treatment with ruxolitinib to control hydroxyurea-refractory conditions. BL cells were CD10+, CD19+, CD20-, CD34-, cytoplasmic CD79a+, and TdT+, and lacked surface immunoglobulins but expressed the cytoplasmic µ heavy chain. In the bone marrow, nuclear MYC+ BL cells displaced the MPN tissues. t(8;14)(q24;q32) occurred at a CG dinucleotide within MYC exon 1 and at the IGHJ3 segment, and an N-like segment was inserted at the junction. The V-D-J sequence of the non-translocated IGH allele had the unmutated configuration. DNA from peripheral blood at a time of the course of MPN exhibited homozygous JAK2V617F mutation, while that at BL development included both JAK2V617F and wild-type DNAs. Although the association between JAK1/2 inhibitor therapy for MPN and secondary development of aggressive B-cell neoplasm remains controversial, this report suggests that, in selected patients, close monitoring of clonal B-cells in the BM is required before and during treatment with JAK1/2 inhibitors.