During laparoscopic cholecystectomy, an 89-year-old man was discovered to have a prolonged APTT. He was transferred to our hospital for a thorough examination because wound bleeding necessitated a reoperation. Based on coagulation factor VIII activity (FVIII:C) of 3.6% and FVIII inhibitor levels of 48.5 BU/ml, he was diagnosed with acquired hemophilia A (AHA). Due to concerns about his advanced age and postoperative infection, immunosuppressive therapy with prednisolone 0.5 mg/kg/day was initiated. His clinical course was favorable, except hemorrhagic shock caused by intramuscular hemorrhage on the right back, although low FVIII inhibitor levels persisted for more than a month; additionally, lower leg edema and increased urinary protein were also observed. He was diagnosed as with AHA and secondary nephrotic syndrome, possibly because of early gastric cancer. As a result, radical endoscopic submucosal dissection (ESD) was performed while a recombinant coagulation factor VIIa preparation was administered. AHA improved rapidly following ESD, and coagulative remission was achieved. Simultaneously, the nephrotic syndrome improved. Because the control of malignant tumors may improve the status of AHA, the timing of malignant tumor intervention must be considered considering the risk of bleeding and infection associated with immunosuppression.
Neuropsychiatric symptoms comprise one of the five classic symptoms of autoimmune thrombotic thrombocytopenic purpura (aTTP). Although aTTP is typically transient, it is sometimes complicated by cerebral infarction with residual disability. This report presents the case of an 87-year-old man previously admitted to a different hospital with fever and transient consciousness loss. After receiving platelet transfusion with diagnosis of Evans syndrome, he was transferred to our hospital with worsening consciousness disturbance. He was subsequently diagnosed with aTTP with a PLASMIC score of 6 points, ADAMTS13 activity of less than 0.5%, and its inhibitor of 7.4 BU/ml. Platelet count and consciousness were rapidly improved with plasmapheresis and steroids, but motor aphasia emerged. MRI showed multiple cerebral infarctions, including a large infarction in the left frontal lobe. Thus, unfractionated heparin was administered. When his platelet count dropped once again on the 20th day, rituximab was added. The treatment eventually proved to be successful, and his aTTP remained in remission one year after the onset. Treatment for cerebral infarctions was switched to DOAC, and rehabilitation was continued. However, his ADL has not yet recovered. Advances in aTTP treatment have cured many similar cases. Thus, rituximab is now considered a treatment option for refractory cases. However, ischemic organ damage in acute phase and sequelae are observed. Therefore, early diagnosis and novel therapy are required.
We describe a patient with biphenotypic acute leukemia (BAL) with T-lymphoid lineage and myeloid lineage differentiation [BAL (T/M)]. Cytogenetic analysis revealed complex chromosomal abnormalities, including der(2)t(2 ; 11)(p21 ; q23). Neither leukemia cells nor T-cell receptor gene rearrangements were detected in the bone marrow samples after four courses of high dose cytosine arabinoside regimen. However, der(2)t(2 ; 11)(p21 ; q23) anomaly persisted in most of metaphases. Fluorescence in situ hybridization (FISH) analysis with a probe for MLL did not detect the split signal. Forty-five cases of hematological disorder with t(2 ; 11)(p21 ; q23) abnormality have been previously reported. The majority of such cases have been classified as myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). This is the first case BAL (T/M) associated with a t(2 ; 11)(p21 ; q23) anomaly.
Recent studies have revealed the clinical and biological features of stage I follicular lymphoma (FL), but information about patients with stage I FL who underwent total resection after tissue biopsy is limited. Among 305 FL patients diagnosed between 2001 and 2013, clinical stage I disease was observed in 36 patients. Of these, 18 patients underwent total resection after diagnostic tissue biopsy. We used 18F-fluorodeoxyglucose positron emission CT for staging assessment in 13 of 18 patients (72.2%). The median age was 56.5 years. Six patients (33.3%) were male. The soluble interleukin-2 receptor alpha concentration was significantly lower than in patients with residual disease. Among these 18 patients, 7 patients (38.9%) were treated with a "watch-and-wait" (WW) policy, 7 (38.9%) were treated with involved-field irradiation, and 4 (22.2%) received systemic chemotherapy. Patients with resected disease were treated with significantly different strategies from those with residual disease (p = 0.0026). Five patients experienced relapse during follow-up (median follow-up: 48.2 months). All relapses were distant from the primary site, irrespective of treatment strategy. Among all stage I patients, disease resection was not a significant factor for survival (p = 0.9294). Collectively, the choice of treatment strategy was significantly influenced by patient status. Resection status was not significantly associated with survival after several treatment strategies.
We herein report a 64-year-old man who was treated with pembrolizumab for relapsed Hodgkin lymphoma. After the third administration of pembrolizumab, he showed acute anemia with a positive direct anti-globulin test. Because of the markedly erythroid hypoplasia, he was diagnosed with pure red cell aplasia (PRCA) caused by pembrolizumab. He was initially treated with prednisolone, but the reticulocytes decreased after tapering prednisolone. He then received high-dose intravenous immunoglobulin (IVIG) with prednisolone, and PRCA was successfully treated. Although the pathogenesis of PRCA caused by immune checkpoint inhibitors (CPIs) remains unclear, IVIG treatment may be effective for some steroid-refractory CPI-induced PRCA cases.
