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Unexplained Fever In Hematologic Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Clonal transformation of B-lymphocytes with surface immunoglobulin (Ig) restricted to a single Ig class and subclass is present in most CLL patients. T-CLL accounts for 5% of CLL worldwide. The latter typically presents with prominent hepatosplenomegaly and marked lymphocytosis. Skin infiltration is frequent. There is often poor response to treatment. Prolymphocytic leukemia, another variant of less maturely appearing lymphocytes, typically presents with massive splenomegaly, high lymphocyte count, and insignificant enlargement of peripheral lymph nodes. A particular form of lymphoproliferative disease with granulocytopenia and recurrent infections is associated with proliferation of large granular lymphocytes.68
Chronic Leukemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Scott J. Graham, James D. Cotelingam
In typical cases, most WBCs are small lymphocytes with round nuclei, condensed, blocky chromatin, absent to inconspicuous nucleoli, and scant cytoplasm (Fig. 1). Peripheral blood smears often contain numerous “smudge” or “basket” cells, which are a technical artifact. In a small percentage of cases, the nuclei have slight irregularities or plasmacytoid features. Up to 10% of circulating lymphocytes may be classified as prolymphocytes, which have more abundant cytoplasm, nuclei with more open chromatin, and a single prominent nucleolus. When prolymphocytes account for 11–55% of lymphoid cells, the designation prolymphocytic transformation of CLL (CLL/PLL) is appropriate. If >55% of lymphoid cells are prolymphocytes, the diagnosis of de-novo prolymphocytic leukemia (PLL) is likely.
Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma and B-Cell Prolymphocytic Leukemia
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Blood. The diagnosis of CLL requires the presence of ≥5 × 109/L B lymphocytes in the blood for the duration of at least 3 months. The clonality of the circulating B cells needs to be confirmed by flow cytometry (FC). The identification of >55% prolymphocytes favors the diagnosis of B-cell prolymphocytic leukemia (B-PLL). In the absence of lymphadenopathy or organomegaly, cytopenias, or disease-related symptoms, the presence of ≤5 × 109/L B lymphocytes in blood is defined as “monoclonal B lymphocytosis.” The cytopenia caused by a typical marrow infiltrate defines the diagnosis of CLL regardless of the number of blood B lymphocytes.
Partial response to venetoclax and ruxolitinib combination in a case of refractory T-prolymphocytic leukemia
Published in Hematology, 2023
Joel Brothers, Dan Ran Castillo, Won Jin Jeon, Bowon Joung, Yuliya Linhares
A 64-year-old man with no significant medical comorbidities was diagnosed with T-prolymphocytic leukemia after leukocytosis was incidentally discovered on a complete blood count. After a six-month period of observation, he developed rapidly progressive leukocytosis and underwent treatment with the anti-CD52 monoclonal antibody alemtuzumab intravenously. He attained complete response (CR) and underwent consolidative therapy with a 10/10 matched unrelated peripheral blood stem cell transplant with Flu/Mel/TBI reduced-intensity conditioning (melphalan 100 mg/m2 day −6, fludarabine 40 mg/m2/day on days −5 to −2, total body irradiation 200 cGy day −1). He received graft-versus-host (GVH) prophylaxis with cyclophosphamide (50 mg/kg day +3 and +4), tacrolimus, and mycophenolate. Day +30 and day +100 bone marrow evaluations demonstrated ongoing CR and >98% donor chimerism.
Combining epigenetic therapy with venetoclax overcomes alemtuzumab resistance in T-cell prolymphocytic leukemia. A case report of a 26-year-old man with a prior history of T-cell acute lymphoblastic leukemia and GI-T lymphoma
Published in Acta Oncologica, 2020
Michael Asger Andersen, Rebecca Valentin, Lene Dissing Sjö, Line Borgwardt, Kjeld Schmiegelow, Mette Klarskov Andersen, Rasmus L. Marvig, Christina Westmose Yde, Carsten Utoft Niemann
T-prolymphocytic leukemia (T-PLL) is a rare, mature T-cell leukemia representing less than 2% of mature lymphocytic leukemias [1,2]. T-PLL is generally resistant to conventional chemotherapy with a median survival of approximately 7 months. T-PLL primarily affects elderly adults with a median age at onset of 63 years, however in patients with ataxia telangiectasia T-PLL accounts for 3% of all malignancies with a much earlier onset (median age 30 years) [3]. We report a case of a 26-year-old male with alemtuzumab-refractory T-PLL 16 years after curatively treated T-cell acute lymphoblastic leukemia (T-ALL). Partly successful combination treatment with venetoclax, alemtuzumab, vorinostat, and cladribine is discussed along with clinical, morphological, immunophenotypic, molecular, genetic, and cytogenetic features.
Safety evaluation of the DTaP5-IPV-Hib-HepB vaccine: a review
Published in Expert Opinion on Drug Safety, 2022
Francesca Fortunato, Domenico Martinelli, Pier Luigi Lopalco, Rosa Prato
In the overall analysis, serious adverse events related to study vaccines, occurring after any vaccination dose, were reported in 9 subjects in the DTaP5-IPV-Hib-HepB group. These events included febrile convulsion (1 subject), idiopathic thrombocytopenic purpura (1), prolymphocytic leukemia (1), hypotonia (1), apparent life-threatening event (1), abdominal pain and crying (1), and fever ≥39.5°C (3) [16]. Three additional subjects had serious adverse events (intussusception, diarrhea, and gastroenteritis) which were considered exclusively related to the concomitantly administered rotavirus vaccine (RotaTeq®, manufacturer Merck Sharp and Dohme B.V., marketing authorization holder MSD VACCINS) [7].