Essential thrombocythemia is a myeloproliferative neoplasm. Ischemic stroke is frequently the first manifestation of essential thrombocythemia. We herein report a patient with JAK2V617 mutation-positive essential thrombocythemia who developed recurrent ischemic stroke with rapid development of intracranial artery stenosis and subsequently underwent successful mechanical thrombectomy. The high JAK2V617F allele burden in our patient (58.4%) may have affected the patient's condition. We discuss similar reports in the literature and the possible pathophysiologic mechanism of large artery involvement in these patients.
Primary central nervous system vasculitis (PCNSV) is an uncommon vasculitis restricted to the small- and medium-sized vessels in the brain and spinal cord. Previously, only 9 cases have been reported that initially manifested as an isolated spinal cord lesion with subsequent brain involvement, where the longest interval from the onset to brain involvement was 1 year and 11 months. We herein report the case of an isolated spinal cord lesion with subsequent brain involvement appearing seven years and five months later. This case shows that brain lesions can develop after an extended interval from spinal onset in PCNSV.
A case of therapy-related myelodysplastic syndromes (t-MDS) in 66-year-old male patient is reported. The patient was diagnosed as having multiple myeloma in July 1983. Cyclophosphamide was given since September 1984, and melphalan was added since June 1986. Radiation therapy was not performed. Mild, slowly aggravating pancytopenia developed in July 1987. By December 1987, the hemoglobin level dropped to 6.0 g/dl, leukocytes to 2,800/microliters, and platelets to 15,000/microliters. At that time, 27% of the bone marrow cells were blasts and 23.3% monocytoid cells. Based on these findings, a diagnosis of t-MDS was made. He was managed by supportive care only, but the monocytoid cells increased rapidly in number and he died of pulmonary bleeding in March 1988. Chromosomal banding studies of the bone marrow cells revealed dir ins [inv (17) (p13q21); 21] (q21; p13q22) in all the 11 metaphases examined, but chromosomes No. 5 and 7 were normal. However, Keldsen et al reported that chromosome 21q rearrangements were nonrandomly associated with t-MDS and t-acute nonlymphocytic leukemia.
To examine whether we can safely shorten the periods of antibiotic administration for septic patients with procalcitonin (PCT) measurements compared with no PCT measurements.
A 26-year-old man was diagnosed with B/T-type mixed-phenotype acute leukemia (MPAL-B/T) based on blasts being positive for CD19, cytoplasmic CD3, and cyCD79a, but negative for myeloperoxidase. Acute lymphoblastic leukemia-based chemotherapy was started, but the leukemia was refractory. He underwent cord blood transplantation with the conditioning regimen of total body irradiation plus cyclophosphamide and cytarabine with granulocyte-colony stimulating factor priming. Prophylaxis for graft versus host disease was performed with short-term methotrexate and cyclosporin. The leukemia relapsed in bone marrow 20 months later. At that time, he was treated with inotuzumab ozogamicin because the blasts expressed CD22 (75.4%), but this was ineffective. He was next administered blinatumomab with dexamethasone pretreatment, resulting in a complete remission (CR). He subsequently underwent human leukocyte antigen-haploidentical peripheral blood stem cell transplantation. He has still maintained a CR for 12 months. Blinatumomab might be a promising treatment and a bridge to stem cell transplantation even in relapsed/refractory CD19-expressing MPAL-B/T.
