[Guillain-Barré syndrome associated with Epstein-Barr virus and cytomegalovirus reactivation during treatment for chronic graft-versus-host disease after allogeneic bone marrow transplantation].

Guillain-Barré syndrome (GBS) is a rare neurological complication of allogeneic hematopoietic stem cell transplantation (HSCT). The pathogenesis of post-HSCT GBS is unclear. Here, we report a case of GBS coincident with Epstein-Barr virus (EBV) and cytomegalovirus (CMV) reactivation that occurred after HSCT in a patient with myelodysplastic syndrome. A 61-year-old man was admitted to our hospital because of gait disturbance due to lower limb muscle weakness, which arose during treatment for chronic graft-versus-host disease (GVHD) five months after allogeneic HSCT. He was diagnosed with GBS based on his clinical course, cerebrospinal fluid analysis, and a nerve conduction study. At that time, he exhibited EBV and CMV reactivation. GBS improved after intravenous injection of immunoglobulins. Our case suggests that reactivation of EBV and CMV during treatment for chronic GVHD may induce GBS, and that rapidly progressive muscular weakness coincident with EBV or CMV reactivation can be a diagnostic sign of GBS after allogeneic HSCT.

Severe systemic inflammation mimicking TAFRO syndrome following COVID-19.

TAFRO syndrome is a rare systemic inflammatory disease. Its pathogenesis mainly involves excessive cytokine secretion and autoimmune dysfunction. Although its etiology is unclear, some viral infections have been reported to cause it. Here, we report a case of severe systemic inflammation mimicking TAFRO syndrome that arose after COVID-19. A 61-years-old woman suffered from a continuous fever, ascites, and edema after contracting COVID-19. She developed progressive thrombocytopenia, renal failure, and elevated C-reactive protein levels. She was tentatively diagnosed with multisystem inflammatory syndrome in adults (MIS-A) and received steroid pulse therapy. However, she exhibited worsening fluid retention and progressive renal failure, which are not typical of MIS-A. A bone marrow examination showed reticulin myelofibrosis and an increased number of megakaryocytes. Although a definitive diagnosis of TAFRO syndrome was not made according to current diagnostic criteria, we determined that her symptoms were clinically consistent with those of TAFRO syndrome. Combination therapy, including steroid pulse therapy, plasma exchange, rituximab, and cyclosporine, improved her symptoms. There are pathological similarities between hyperinflammation that arises after COVID-19 and TAFRO syndrome in terms of the associated cytokine storms. COVID-19 may have triggered the development of systemic inflammation mimicking TAFRO syndrome in this case.

An evaluation based on relative treatment intensity in older patients treated with reduced-dose R-THP-COP therapy for diffuse large B-cell lymphoma: A multicenter retrospective cohort study.

INTRODUCTION: The number of older patients with diffuse large B-cell lymphoma (DLBCL) is increasing. Although the standard treatment for newly diagnosed younger patients with DLBCL has been established, no consensus has been reached regarding the optimal chemotherapy intensity and regimen for older patients with DLBCL. In addition, no method for evaluating treatment intensity in retrospective studies when different numbers of chemotherapy courses are administered has been elucidated. MATERIALS AND METHODS: A multicenter retrospective analysis was conducted to evaluate the outcomes of a reduced-dose R-THP-COP regimen, which included 30 mg/m2 of pirarubicin, in 54 patients with DLBCL who were aged ≥75. To assess treatment intensity, we defined the relative treatment intensity (RTI) as the number of courses administered multiplied by the relative dose intensity (RDI). RESULTS: The estimated four-year overall survival rates (OS) of the patients aged 75-80 and ≥ 80 were 55.1% and 60.6%, respectively. There was no significant difference in four-year OS between these age groups. In our cohort, there was no significant difference in the estimated four-year OS between the patients who received reduced-dose R-THP-COP at an RDI of ≥61% and those that received it at an RDI of <61% (P = 0.35). On the other hand, the patients who received reduced-dose R-THP-COP at an RTI of ≥2.7 exhibited a significantly higher estimated four-year OS than those treated at an RTI of <2.7 (68.5% vs. 28.7%; P < 0.001). Multivariate analysis revealed that the RTI was a significant independent predictor of OS. The cumulative incidence of treatment-related mortality (TRM) at one year was 4.2% and 3.4% in the 75-80 and ≥ 80 age groups, respectively. The cumulative incidence of TRM was significantly worse among the patients with Charlson Comorbidity Index (CCI) scores of ≥2 than among those with CCI scores of 0 or 1. DISCUSSION: Our study suggests that the reduced-dose R-THP-COP regimen is a suitable treatment option for older patients with DLBCL, especially those with CCI scores of <2. Our study also showed that the RTI may be a valuable tool for assessing treatment intensity in retrospective studies involving older patients.

Progressive Massive Splenomegaly in an Adult Patient with Kabuki Syndrome Complicated with Immune Thrombocytopenic Purpura.

Kabuki syndrome is characterized by multiple systemic anomalies and intellectual disability. It is complicated with immunodeficiencies and autoimmune disorders. The syndrome is caused by a mutation in the KMT2D gene. We herein report a case of a Kabuki syndrome with developing immune thrombocytopenic purpura (ITP) and progressive splenomegaly. Laparoscopic splenectomy was performed and the patients' symptoms quickly disappeared with platelet recovery. After this operation, the patient had no severe complications. A sequence analysis of the KMT2D gene identified a pathogenic mutation frequently associated with ITP. Laparoscopic splenectomy is therefore considered to be a good therapeutic option for recurrent ITP and symptomatic splenomegaly with Kabuki syndrome.

Two Episodes of Transfusion-related Acute Lung Injury (TRALI) Occurring within a Short Period.

Transfusion-related acute lung injury (TRALI) is a non-hemolytic adverse reaction that occurs ≤6 hours after receiving a transfusion. A 72-year-old man with leukemia developed severe hypoxemia after platelet transfusions on two occasions within a 4-day period. During the first episode, the transfused platelet preparation was positive for anti-human-leukocyte antigen antibodies. The pathogenesis of TRALI includes an antibody-mediated mechanism and a non-antibody-mediated mechanism, in which various factors combine to activate pulmonary neutrophils. In our case, it is considered that the patient's neutrophils reached the activation threshold for the development of TRALI after the accumulation of various factors besides anti-leukocyte antibodies.

[Refractory ascites developing in late-phase of cord blood transplantation improving with CART].

We managed a patient with acute myeloid leukemia (AML) who showed refractory ascites that developed in late-phase cord blood transplantation (CBT). The ascites obverted 5 months after CBT. The liver was atrophic, and serum hyaluronic acid was elevated at the onset, suggesting fibrotic changes in the liver. The ascites were transiently improved by cell-free and concentrated ascites reinfusion therapy (CART) and corticosteroid administration; however, the patient died from anasarca and recurrent AML 378 d after CBT. The etiology of the ascites is not well understood; therefore, additional studies on similar patients should be explored for proper management.