Slowly progressing acute lymphoblastic leukemia with prolonged leukopenia.

Acute lymphoblastic leukemia is typically characterized by leukocytosis, resulting from the uncontrolled proliferation of malignant cells. However, we report an atypical case of acute lymphoblastic leukemia that presented with leukopenia and exhibited a protracted clinical course spanning 6 months. The patient, a 45-year-old female, initially presented to our hospital with recurrent fever and was found to have lymphoblasts in a hypoplastic bone marrow. Upon further investigation, the patient was diagnosed with B-cell lymphoblastic leukemia, not otherwise specified, based on cell surface antigen expression and genetic abnormalities. Notably, the patient demonstrated persistently low white blood cell and neutrophil counts, without evidence of increasing lymphoblast infiltration in the bone marrow during the ensuing 6-month period. Subsequent chemotherapy led to normalization of hematopoiesis and disappearance of lymphoblasts, resulting in complete remission of the disease.

Real-world efficacy of DA-EPOCH-R/HD-MTX regimen in CD5-positive diffuse large B cell lymphoma: a single-institute analysis.

CD5-positive diffuse large B cell lymphoma (CD5+ DLBCL) is a high-risk lymphoma type. Recently, the PEARL5 (a Phase II trial of DA-EPOCH and Rituximab with HD-MTX therapy for newly diagnosed DLBCL with CD5 expression) study demonstrated the efficacy of the DA-EPOCH-R (cyclophosphamide, etoposide, doxorubicin, vincristine, prednisone, and rituximab)/HD-MTX (high-dose methotrexate) regimen for CD5+ DLBCL. In this report, we revealed the impact of the DA-EPOCH-R/HD-MTX regimen on the clinical course of CD5+ DLBCL in the real-world. We retrospectively compared CD5+ and CD5- DLBCL patients diagnosed from January 2017 to December 2020 and analyzed their clinicopathological characteristics, treatment, and prognosis. There was no difference in age, sex, clinical stage, and cell of origin; however, the CD5-positive group had higher lactate dehydrogenase levels and a worse performance status than the CD5-negative group (p=0.00121 and p=0.0378, respectively). International prognostic index (IPI) was worse in the CD5-positive group than in the CD5-negative group (p=0.0498), but NCCN-IPI (National Comprehensive Cancer Network-IPI) was no different between the two groups. The CD5-positive group was more frequently treated with the DA-EPOCH-R/HD-MTX regimen than the CD5-negative group (p =0.001857). Complete remission rate and 1-year overall survival did not differ between the CD5-positive and -negative groups (90.0% vs 81.4%, p=0.853; 81.8% vs 76.9%, p=0.433). We conclude that the DA-EPOCH-R/HD-MTX regimen is effective for CD5+ DLBCL in this single institute analysis.

Therapy-related myeloid neoplasms of recipient origin after allogeneic hematopoietic stem cell transplantation for acute leukemia.

Some allogeneic stem cell transplantation (allo-SCT) recipients develop therapy-related myeloid neoplasms (t-MNs) of recipient origin with features including karyotypically abnormal hematopoiesis without cell dysplasia and myeloblast increase. However, due to their rarity their clinical course remains unclear. We report six cases of t-MN in patients with chromosomal abnormalities (CAs) after autologous recovery following allo-SCT for acute leukemia. CAs were first detected at a median interval of 422 (range 30-1941) days from allo-SCT. The fraction of CA-bearing cells of recipient origin increased with time, and cytogenetic relapse of underlying disease was not observed. Continuous emergence of identical autologous CAs was observed in one patient who did not receive total body irradiation (TBI). The other five patients received TBI, and complex karyotypes with the appearance of different types of CAs were the most dominant feature. Despite the persistence of complex abnormalities in the irradiated patients, no patient developed therapy-related acute myeloid leukemia (t-AML). TBI appears to be the major cause of t-MN of recipient origin with different types of CAs. Although t-MNs in patients receiving TBI do not initially seem to evolve to overt t-AML, they were associated with higher risk of underlying disease and greater oncogenic potential of irradiation.

Refractory thrombotic thrombocytopenic purpura achieving complete remission with rituximab treatment.

Some patients with thrombotic thrombocytopenic purpura (TTP) are refractory to standard treatment regimens comprised of plasma exchange (PEX) and steroids. This report describes a 40-year-old woman with refractory TTP who achieved complete remission (CR) in response to rituximab. She was referred to our institution from a rural hospital with purpura of the extremities, severe thrombocytopenia, anemia, and rapidly progressive disturbance of consciousness. TTP was diagnosed based on the clinical symptoms of TTP, low ADAMTS13 activity (<0.5%), and high ADAMTS13 inhibitor (4.4 BU/ml) titers. High-dose prednisolone was immediately administered and PEX was started. This approach was initially effective, but the thrombocytopenia and disturbance of consciousness worsened on the sixth day of treatment. We considered this patient to have refractory TTP and administered weekly rituximab. CR was achieved on day 20, and the disease status of this patient has remained stable over the long term. Our experience with this patient and five others who were similarly treated at our hospital over the past eight years indicates that rituximab is effective for refractory TTP.