Epstein-Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab.

Histopathologic findings in the lymph nodes of patients with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome are similar to those of idiopathic multicentric Castleman's disease (iMCD), but TAFRO syndrome is different from iMCD in how it can progress rapidly and be fatal. These patients present scarce lymphadenopathy and low immunoglobulin levels. We present a case of cutaneous and systemic plasmacytosis (C/SP) that caused TAFRO syndrome-like symptoms which were successfully treated with rituximab. A 67-year-old woman presented with fever and a pruritic skin rash. Numerous plasma cells were observed in the peripheral blood and imaging revealed organomegaly, anasarca, and generalized lymphadenopathy. Subsequently, she rapidly developed thrombocytopenia as well as renal and heart failure. She tested positive for the Epstein-Barr virus (EBV), elevated immunoglobulins, and C/SP, which are also atypical for TAFRO syndrome, thereby complicating the diagnosis. However, after using the Japanese TAFRO Syndrome Research Group diagnostic criteria, we promptly administered rituximab to treat the C/SP with TAFRO-like symptoms and saved her life. Finally, histopathological observations of the lymph node biopsy helped confirm EBV-positive hypervascular-type iMCD. Therefore, diagnosing TAFRO-like syndromes based on the Japanese diagnostic criteria and following the associated treatment even without a confirmed diagnosis is crucial to improving the patient outcomes.

[Successful treatment with ponatinib in combination with hyper-CVAD for chronic myeloid leukemia in the lymphoid blastic phase].

The prognosis of chronic myeloid leukemia (CML) has improved dramatically with the introduction of tyrosine kinase inhibitors. Although the use of second-generation tyrosine kinase inhibitors is now available for initial cases, a small number of patients with CML unfortunately still experience progression to the accelerated or blastic phase of the disease. We recently managed a patient with chronic-phase CML, who developed a T315 mutation early in the course of treatment with dasatinib and progressed to the lymphoid blastic phase. The patient responded quickly to ponatinib therapy in combination with hyper CVAD, leading to cord blood transplantation. We report here the first case of a patient with CML in the lymphoid blastic phase treated with ponatinib in combination with hyper CVAD, which was tolerable despite adverse events such as infection, bilirubin elevation, and hypertension, and who was able to proceed to transplantation after achieving a complete molecular response.