China
Africa
Explore chapters and articles related to this topic
Chronic Leukemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Scott J. Graham, James D. Cotelingam
T-cell prolymphocytic leukemia (T-PLL) accounts for about one-third of cases morphologically consistent with PLL. Most patients are elderly adults presenting with fatigue, weight loss, and skin lesions. Table 8 contains the most common clinical and hematologic manifestations.
A Curious Case of Morphologically Deceptive Pediatric B-Lymphoblastic Leukemia with Granular “Blebbed” Blasts and DLBCL-Like Biopsy Findings
Published in Fetal and Pediatric Pathology, 2023
Sunita Sharma, Kavita Gaur, Tatton Perme, Amrita Singh
B-acute lymphoblastic leukemia (ALL) is a neoplasm of precursor lymphoid cells (lymphoblasts), confined to the B-cell lineage. It primarily occurs in children and has a good prognosis, whereas in adults it typically pursues and aggressive course. From a morphological perspective, the blasts in B-ALL are variable in appearance, some being, small sized displaying condensed chromatin and inconspicuous nucleoli with scant basophilic agranular cytoplasm. Other cells may have larger size, fine chromatin, few prominent nucleoli and moderate basophilic, agranular cytoplasm. Cytoplasmic blebbing in the blasts of ALL is an unusual morphological feature. Blebbing is known to occur in cases of acute megakaryoblastic leukemia with few reports described in T-cell prolymphocytic leukemia [1, 2]. In cases of B-ALL such morphology has been described only recently in only two reports [3, 4].
Keeping up with venetoclax for leukemic malignancies: key findings, optimal regimens, and clinical considerations
Published in Expert Review of Clinical Pharmacology, 2021
Maria Siddiqui, Marina Konopleva
Venetoclax has also been studied in T cell prolymphocytic leukemia (T-PLL), a rare and aggressive mature T cell malignancy. Preclinical BH3 profiling studies with T-PLL patient samples showed a dependence on BCL-2 and MCL-1 for survival. Herbaux et al. reported preclinical synergy of ruxolitinib and venetoclax and used it clinically in two patients with relapsed/refractory T cell PLL with good response [93]. Hampel et al. reported their experience in a retrospective review, which included 9 patients. The median number of preceding treatments were 3, with 2 patients having undergone HSCT. Venetoclax was administered as monotherapy in 4 patients and together with bendamustine in 5 patients. The results showed an ORR of 56% with PR as the best response. However, the ORR was 80% in the bendamustine–venetoclax group. The most common grade 3 and above toxicities were edema, neutropenia, and infections (pneumonia and cellulitis). Though survival was protracted (range 34–201 days), the study shows that venetoclax in combination with a less myelosuppressive chemotherapy backbone could offer a potential avenue of treatment in patients with T-PLL [94].
Advances in the pharmacotherapeutic options for primary nodal peripheral T-cell lymphoma
Published in Expert Opinion on Pharmacotherapy, 2021
Anna Wolska-Washer, Piotr Smolewski, Tadeusz Robak
JAK/STAT pathway inhibition was an interesting approach studied in a phase 2 trial with ruxolitinib (Jakafi®, Novartis) in patients with relapsed/refractory peripheral T-cell lymphoma/leukemia [112]. The patients were divided into three cohorts, based on the JAK/STAT mutations (cohort 1), JAK/STAT activation (cohort 2), and neither of these (cohort 3). The treatment was relatively well tolerated, with the most common grade ≥3 AEs being neutropenia, anemia, thrombocytopenia, and lymphopenia. In 48 patients, the ORR was 23%, with an additional 12.5% of stable disease over a period of 6 months (clinical benefit rate). Median DOR was 7.3 months. Patients in cohorts 1 and 2 benefitted significantly from ruxolitinib, compared to cohort 3. The responses were more often observed in AITL, T-follicular helper phenotype PTCL, T-cell prolymphocytic leukemia, and large granular lymphocyte leukemia. The loss of response to ruxolitinib was paralleled by an increased expression of proteins (i.e. S6 kinase) involved in the phosphatidylinositol 3-kinase/Akt/mammalian target of rapamycin (PI3K/Akt/mTOR) pathway.