Single agent vemurafenib or rituximab-vemurafenib combination for the treatment of relapsed/refractory hairy cell leukemia, a multicenter experience.

BACKGROUND: Hairy cell leukemia (HCL) is a rare mature B-cell malignancy that is primarily treated with purine analogues. However, relapse remains a significant challenge, prompting the search for alternative therapies. The BRAF V600E mutation prevalent in HCL patients provides a target for treatment with vemurafenib. PATIENTS AND METHODS: This multicenter retrospective study included nine patients with relapsed/refractory (R/R) HCL from six different centers. Patient data included demographics, prior treatments, clinical outcomes, and adverse events. RESULTS: Patients received different treatment regimens between centers, including vemurafenib alone or in combination with rituximab. Despite the differences in protocols, all patients achieved at least a partial response, with seven patients achieving a complete response. Adverse events were generally mild with manageable side effects. The absence of myelotoxic effects and manageable side effects make BRAF inhibitors attractive, especially for patients ineligible for purine analogues or those with severe neutropenia. CONCLUSION: Single agent vemurafenib or in combination with rituximab appears to be a promising therapeutic option for R/R HCL. Further research is needed to establish standardized treatment protocols and to investigate long-term outcomes.

Relapsed refractory multiple myeloma with CNS involvement successfully treated with Elranatamab: first reported case.

Central nervous system (CNS) involvement in multiple myeloma (MM) is a rare and challenging complication associated with poor prognosis and limited treatment options. Emerging T-cell directing therapies, such as bispecific antibodies (bsAbs) and chimeric antigen receptor T cells (CAR-T), have shown remarkable success in treating MM, but their efficacy in CNS involvement remains unclear. Elranatamab, a humanized bispecific antibody targeting B-cell maturation antigen (BCMA) and CD3-expressing T cells, has demonstrated promising results in relapsed refractory MM. However, its efficacy in treating CNS-MM has not been reported. We present a case of a 37-year-old male MM patient with CNS involvement who has been successfully treated with Elranatamab.

Plerixafor in autologous stem cell transplantation: Does it affect engraftment kinetics?

Plerixafor increases stem cell mobilization by reversibly binding to the chemokine receptor CXCR4. In our study, we examined the results of mobilization with plerixafor and granulocyte colony-stimulating factor (G-CSF) and revealed their effects on autologous stem cell transplantation (ASCT) engraftment kinetics. The study included all cases of ASCT performed in the Adult Bone Marrow Transplantation Unit of xxx University between January 2014 and January 2022. It included a total of 300 patients. The total number of CD34 + cells collected was 7.44 ± 4.19 in patients with plerixafor and 9.53 ± 6.09 in patients without plerixafor. The mean neutrophil and platelet engraftment took longer in plerixafor-mobilized patients (neutrophil: 12 ± 4.1 vs. 10.2 ± 2.7 days; platelet: 21.6 ± 13.9 vs. 14.2 ± 5.9 days; p = 0.008 and p = 0.002). The number of febrile neutropenia attacks was significantly higher in plerixafor-mobilized patients (p = 0.04). In the chemo-mobilized patient subgroup, plerixafor-mobilized patients experienced more febrile neutropenia attacks (p = 0.04). The mean time to both neutrophil and platelet engraftment was longer in patients mobilized with plerixafor. In the subgroup of patients with MM, the mean time to platelet engraftment was longer in patients mobilized with plerixafor. Plerixafor and its effect on engraftment kinetics should be evaluated with further studies in a larger population with survival analysis.

Should Core Needle Lymph Node Biopsy be a Relevant Alternative to Surgical Excisional Biopsy in Diagnostic Work Up of Lymphomas?

OBJECTIVE: Surgical excisional biopsy is accepted as the standard of care approach in the diagnosis of lympho- mas. Financial issues related to the increased cost and the invasive nature of the procedure forced physicians to use some alternative diagnostic methods. Percutaneous core needle biopsy, which gained a reputation for the diagnosis of lymphomas with the advent of improved pathological, immunohistochemical, and molecular analysis, made it possible to have an accurate diagnosis with limited tissue samples. In this retrospective study, we aimed to compare the diagnostic yield of surgical excisional biopsy and core needle biopsy. MATERIALS AND METHODS: This study included 131 patients who were diagnosed with lymphoma with a nodal biopsy which was acquired via surgical excisional biopsy or core needle biopsy between 2014 and 2020 in our center. Around 68 patients underwent surgical excisional biopsy and the remaining 63 underwent core needle biopsy. Samples that allowed to the identification of the exact tumor type and/or subtype were accepted as fully diagnostic. Sufficient amount of tissue that the pathologist could have any suspicious findings considering malignant lymphoma was classified as partial diagnostic group. Inadequate samples were the ones who were not enough to report any final diagnosis. RESULTS: The patients who underwent a core needle biopsy were significantly older than the patients who underwent to surgical excisional biopsy (56.8 vs. 47.6, P = .003). Despite the full diagnostic ability of surgical excisional biopsy outperformed core needle biopsy (95.2 % vs. 83.8 %, P=.035), in 92.6% of the patients whose tissue samples were obtained via core needle biopsy were accepted to have a sufficient diagnosis to initiate the treatment and not required a second biopsy, which was comparable with the ones achieved by surgical excisional biopsy (92.6% vs. 95.2%, P = .720). CONCLUSION: According to the results obtained in our study, we may conclude that core needle biopsy is a viable and comparable alternative to surgical excisional biopsy, offering a less invasive and less-expansive approach.

Prognostic Factors and Intensive Care Outcome in Post-Transplant Phase of Hematopoietic Stem Cell Transplantation: Intensive Care Outcome in Hematopoietic Stem Cell Transplantation.

Introduction: To identify new clinical and biologic parameters associated with short-term survival in allogeneic or autologous hematopoietic stem cell transplantation (HSCT) patients who were admitted to the intensive care unit (ICU) during their post-transplant period. Materials and methods: 40 patients who were admitted to the ICU in our center during their post-transplant period were evaluated retrospectively between Jan 2014 - Jun 2021. Baseline patient characteristics before the transplant, reasons for ICU admissions, laboratory and clinical findings, supportive treatment in ICU and short-term survival were analyzed. Results: We found 8.8% ICU admission rate in all patient group (n = 450). Mortality rate of the patients who were admitted to ICU was 75%. Invasive mechanic ventilation, need for vasopressor, heart rate was significantly different between survivor and non-survivor group (p = 0.001, p = 0.001, p = 0.004). Elevated INR was associated with poor survival on ICU (p = 0.033). APACHE II score was an independent predictor of ICU mortality (p = 0.045). Conclusion: Despite the recent advances in transplant conditioning protocols, prophylaxis strategies and improvements of management in ICU, overall survival for HSCT patients in ICU is still poor. In this study INR level was described as a new prognostic factor in ICU for first time in the literature.

Clinical Characteristics and Outcomes of COVID-19 in Turkish Patients with Hematological Malignancies

Objective: Patients with solid malignancies are more vulnerable to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection than the healthy population. The outcome of SARS-CoV-2 infection in highly immunosuppressed populations, such as in patients with hematological malignancies, is a point of interest. We aimed to analyze the symptoms, complications, intensive care unit admissions, and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey. Materials and Methods: In this multicenter study, we included 340 adult and pediatric patients diagnosed with SARS-CoV-2 from March to November 2020. Diagnosis and status of primary disease, treatment schedules for hematological malignancies, time from last treatment, life expectancy related to the hematological disease, and comorbidities were recorded, together with data regarding symptoms, treatment, and outcome of SARS-CoV-2 infection. Results: Forty four patients were asymptomatic at diagnosis of SARS-CoV- 2 infection. Among symptomatic patients, fever, cough, and dyspnea were observed in 62.6%, 48.8%, and 41.8%, respectively. Sixty-nine (20%) patients had mild SARS-CoV-2 disease, whereas moderate, severe, and critical disease was reported in 101 (29%), 71 (20%), and 55 (16%) patients, respectively. Of the entire cohort, 251 (73.8%) patients were hospitalized for SARS-CoV-2. Mortality related to SARS-CoV-2 infection was 26.5% in the entire cohort; this comprised 4.4% of those patients with mild disease, 12.4% of those with moderate disease, and 83% of those with severe or critical disease. Active hematological disease, lower life expectancy related to primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission, and first-line therapy used for coronavirus disease-2019 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azithromycin were associated with a higher rate of mortality in comparison to favipiravir use. Conclusion: Patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.