Comparison of platelet transfusion effectiveness between Helicobacter pylori-positive and -negative immune thrombocytopenia.

Immune thrombocytopenia (ITP) is characterized by early platelet destruction and impaired platelet production. Helicobacter pylori (H. pylori) infection seems to contribute to the pathogenesis in certain ITP patients in Japan. We compared the effectiveness of platelet transfusion in severe ITP in the presence or absence of H. pylori. The median corrected count increment (CCI) at 24 h after platelet transfusion (CCI-24) of the H. pylori-positive ITP patients was higher than that of the H. pylori-negative ITP patients (6463 vs. 754, p < 0.001), and the CCI-1 was also in the same direction but not significant (23 351 vs. 11 578). Multiple regression analyses showed that H. pylori infection was independently associated with CCI-24. Our study suggests that platelet transfusion may be more effective in H. pylori-positive ITP patients.

[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.

[Erdheim-Chester disease diagnosed with right atrium tumors].

Erdheim-Chester disease (ECD) is a very rare form of the non-Langerhans histiocytic multisystem disorder. The cardiac involvement is often challenging and is associated with poor prognosis. Transthoracic echocardiography was used to detect right atrium tumors in a 62-year-old man with heart failure who was admitted to our hospital. The circumferential soft tissue sheathing of the aorta (coated aorta) and fat infiltration around the kidneys (hairy kidneys) was seen on a contrast-enhanced computed tomography strongly suspecting ECD imaging. The patient was diagnosed with ECD based on histopathology reports of the surgical resection tumor. The characteristic imaging findings of ECD may contribute to an early and accurate diagnosis.

Central nervous system relapse after combination therapy including polatuzumab vedotin in patients with diffuse large B-cell lymphoma.

Preventing central nervous system (CNS) relapse is a major challenge in the treatment of diffuse large B-cell lymphoma (DLBCL). However, no previous studies have examined the efficacy of polatuzumab vedotin (PV)-containing regimens in preventing CNS relapse in patients with DLBCL. Here, we report two cases of CNS relapse after PV-containing chemotherapy for DLBCL. CNS relapse developed during combination therapy with PV, bendamustine, and rituximab (PV-BR) in one patient and six months after PV-BR in the other patient. PV-containing chemotherapy may be ineffective as a prophylaxis against CNS relapse; therefore, additional strategies for preventing CNS relapse in DLBCL patients are required.

Rituximab Monotherapy for Grade 2-3 Lymphomatoid Granulomatosis with Central Nervous System Involvement in a Patient Receiving Methotrexate for Rheumatoid Arthritis.

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder (LPD). The optimal management strategy of methotrexate (MTX) related-LPD with central nervous system (CNS) involvement and histological features of LYG remains unclear. We herein report a case of grade 2-3 LYG in a rheumatoid arthritis patient, in which an intracranial mass accompanied by hemorrhaging and pulmonary and skin lesions developed. The patient received successful rituximab monotherapy. The tumor cells in the skin and brain showed monoclonal and oligoclonal proliferation, respectively. Our case suggests that rituximab monotherapy may be effective against MTX-LPD with CNS involvement, especially in cases with LYG histology.

Acquired Thrombotic Thrombocytopenic Purpura Following BNT162b2 mRNA Coronavirus Disease Vaccination in a Japanese Patient.

A 57-year-old man without underlying diseases presented with fatigue, loss of appetite, and jaundice 1 week after receiving the first dose of the BNT162b2 mRNA coronavirus disease 2019 (COVID-19) vaccine and showed hemolytic anemia with fragmented erythrocytes and severe thrombocytopenia 2 weeks after receiving the vaccine. An a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity level of <10% and ADAMTS13 inhibitor positivity confirmed the diagnosis of acquired thrombotic thrombocytopenic purpura (TTP). Combination therapy with plasma exchange, corticosteroid, and rituximab improved the clinical outcome. We herein report the first Japanese case of TTP possibly associated with vaccination. Physicians should be alert for this rare but life-threatening hematological complication following COVID-19 vaccination.