Did you mean: prolymphocytes count
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British Journal of Haematology May 1974
Topics: Adrenal Cortex Hormones; Age Factors; Aged; Blood Cell Count; Blood Platelets; Cell Nucleolus; Chromatin; Chronic Disease; Cytoplasm; Fatigue; Female; Hemoglobins; Hepatomegaly; Humans; Immune Adherence Reaction; Immunoglobulin A; Lectins; Leukemia, Lymphoid; Leukocyte Count; Lymphocytes; Male; Microscopy, Electron; Middle Aged; Neutrophils; Prognosis; Sex Factors; Splenomegaly; gamma-Globulins
PubMed: 4137136
DOI: 10.1111/j.1365-2141.1974.tb06769.x -
Journal of Hematology Apr 2023B-cell prolymphocytic leukemia (B-PLL) is a rare B-cell neoplasm that typically presents with splenomegaly, a rising white blood cell count, and may or may not have B...
B-cell prolymphocytic leukemia (B-PLL) is a rare B-cell neoplasm that typically presents with splenomegaly, a rising white blood cell count, and may or may not have B symptoms. The diagnosis usually requires a bone marrow biopsy and aspirate with flow cytometry and cytogenetic studies. At least 55% of the lymphocytes in the peripheral blood must be prolymphocytes to be defined as B-PLL. A thorough differential diagnosis would include mantle cell lymphoma, chronic lymphocytic leukemia (CLL) with prolymphocytes, hairy cell leukemia, and splenic marginal zone lymphoma. B-PLL is managed with regimens utilized for CLL, such as ibrutinib and rituximab but is tailored for each individual. The authors report a rare case of B-PLL in a patient with no known history of CLL. The authors discuss this entity in context of the 2017 and 2022 World Health Organization (WHO) classifications, the latter of which no longer recognizes B-PLL as a distinct entity. The authors hope that this article helps practitioners with the diagnosis and treatment of B-PLL. Perhaps with better recognition, and better documentation of histopathologic features of these rare cases going forward, it may prove to be a distinct entity again in future classifications.
PubMed: 37187496
DOI: 10.14740/jh1096 -
Blood Jul 1993Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL. Patients with T-LGL leukemia have clonal proliferations of CD3+ LGL... (Review)
Review
Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL. Patients with T-LGL leukemia have clonal proliferations of CD3+ LGL typically associated with chronic neutropenia and autoimmune features. NK-LGL leukemia is characterized by clonal CD3- LGL proliferation with an acute clinical presentation marked by massive hepatosplenomegaly and systemic illness. However, most patients with increased numbers of CD3- LGL do not have clinical features of NK-LGL leukemia and have a chronic clinical course. X-linked gene analyses have supported a polyclonal LGL lymphocytosis in this syndrome. Further studies are needed to determine whether clonal progression can occur in these patients.
Topics: CD3 Complex; Clone Cells; Female; Hematopoiesis; Humans; Killer Cells, Natural; Leukemia, Prolymphocytic, T-Cell; Leukocyte Count; Lymphocyte Subsets; Lymphoproliferative Disorders; Male; Middle Aged; Prognosis; T-Lymphocytes
PubMed: 8324214
DOI: No ID Found -
Journal of the Royal Society of Medicine Feb 1979
Topics: Aged; Antineoplastic Agents; Humans; Leukapheresis; Leukemia, Lymphoid; Leukocyte Count; Male; Splenectomy
PubMed: 552479
DOI: 10.1177/014107687907200215 -
American Journal of Hematology Jun 2023T-cell prolymphocytic leukemia (T-PLL) is a rare, post-thymic T-cell neoplasm with a diverse clinical course. T-PLL is typically associated with a poor prognosis;...
T-cell prolymphocytic leukemia (T-PLL) is a rare, post-thymic T-cell neoplasm with a diverse clinical course. T-PLL is typically associated with a poor prognosis; however, a subset of patients have inactive disease on initial presentation. There is a lack of accurate delineation of the disease based on initial clinical presentation and pathological assessment, hindering clinical decision-making. To characterize and delineate disease subtypes based on initial clinical presentation and pathologic assessment, we retrospectively reviewed 81 patients with T-PLL treated at our institution. We compared patients with T-PLL who initially presented with a relatively indolent or stable disease course to those with an aggressive disease course. Clinicopathologic characteristics, overall survival (OS), and prognostic factors were analyzed. Patients with inactive disease had a significantly longer OS than patients with active disease. At diagnosis, presence of B symptoms, low hemoglobin, low platelet count, lymphocyte doubling time of fewer than 3 months, and abnormal cytogenetics were associated with shorter OS. Cell morphology, immunophenotype, absolute lymphocyte count, lactate dehydrogenase levels, involvement of liver, spleen, skin or central nervous system, presence of TCL1 rearrangement or inv (14)/t(14;14), presence of chromosome 8 abnormalities, and presence of deletion of 11q were not associated with significant OS difference among the patients. Receiving alemtuzumab as first-line treatment and consolidation with allogeneic hematopoietic stem cell transplant were associated with better outcomes. T-PLL inactive and active disease subtypes can exhibit overlapping yet different clinical and pathological features. We describe several prognostic factors at diagnosis that can be used for risk stratification and aid in guiding treatment decisions.
Topics: Humans; Leukemia, Prolymphocytic, T-Cell; Prognosis; Retrospective Studies; Hematopoietic Stem Cell Transplantation; Chromosome Aberrations; Disease Progression
PubMed: 36964941
DOI: 10.1002/ajh.26918 -
Annales de Dermatologie Et de... Dec 2014Sezary syndrome (SS) presents clinically as erythroderma, which may be pigmented, and pruritic, associated with peripheral lymphadenopathies. Erythroderma may also occur... (Review)
Review
BACKGROUND
Sezary syndrome (SS) presents clinically as erythroderma, which may be pigmented, and pruritic, associated with peripheral lymphadenopathies. Erythroderma may also occur in a broad range of reactive and malignant conditions including T-cell prolymphocytic leukemia (T-PLL). We report a case initially diagnosed as SS but ultimately diagnosed as T-PLL based upon skin involvement.
CASE REPORT
A 70-year-old man was referred by his hematologist for management of SS. Physical examination revealed lymphadenopathies and mild diffuse erythema without infiltration. His WBC count was elevated at 8.3 G/L. A peripheral blood smear showed Sezary-like cells. Flow cytometry of peripheral blood revealed prolymphocytic T-cells staining positively for CD2, CD3, CD4 and CD7. Cytogenetic studies showed chromosomal abnormalities in terms of number and structure with missing chromosomes 6 and13, as well as deletion of chromosome 17. Finally, a diagnosis of T-PLL was made. Pentostatin was initiated pending treatment with alemtuzumab, but the patient's overall condition deteriorated rapidly and he died 10 days later.
DISCUSSION
Diagnosis of LPLT is based upon a number of factors. In the case presented herein, the clinically atypical nature of the skin lesions prompted the dermatologist to review the diagnosis. The morphology of the circulating T-lymphocytes and their immunologic and phenotypic characteristics finally ruled out the diagnosis of Sezary syndrome, while their association with compatible cytogenetic anomalies enabled a diagnosis of prolymphocytic leukemia to be made instead.
CONCLUSION
Prolymphocytic leukemia involves complex differential diagnosis with regard to Sezary syndrome, posing potential pitfalls for hematologists and dermatologists.
Topics: Adenocarcinoma; Aged; Antimetabolites, Antineoplastic; Capecitabine; Chromosome Deletion; Combined Modality Therapy; Delayed Diagnosis; Diagnostic Errors; Fatal Outcome; Humans; Immunophenotyping; Leukemia, Prolymphocytic, T-Cell; Male; Neoplasms, Second Primary; Pentostatin; Rectal Neoplasms; Sezary Syndrome; Skin Neoplasms; T-Lymphocytes
PubMed: 25433931
DOI: 10.1016/j.annder.2014.09.012 -
Cancer Treatment Reviews Feb 2011Hairy-cell leukemia variant (HCl-V) is a district clinico-pathological entity with intermediate features between classical HCl (HCl-C) and B-cell prolymphocytic... (Review)
Review
Hairy-cell leukemia variant (HCl-V) is a district clinico-pathological entity with intermediate features between classical HCl (HCl-C) and B-cell prolymphocytic leukemia. HCl-V is now included in the World Health Organization (WHO) classification as a provisional entity. It is an uncommon disorder accounting for approximately 0.4% of chronic lymphoid malignancies and 10% of all HCl cases. In contrast to HCl-C, HCl-V is a more aggressive disease and according to the new WHO classification it is no longer considered to be biologically related to HCl-C. Patients with HCl-V have an elevated white blood count, easy-to-aspirate bone marrow and weak reactivity to tartrate - resistant acid phosphatase (TRAP). Immunophenotypically, HCl-V cells are positive for CD103 and CD11c and negative for CD25. The HCl-V cells express also the B-cell antigens, CD19, CD20 and CD22. The HCl-V patients have frequently an unmutated Ig gene configuration. Currently, the principles of therapy for this rare disease derive from uncontrolled single institutional studies, or even single case reports. In contrast to HCl-C, the HCl-V response to purine nucleoside analogs (PNA) is limited to partial responses in approximately 50% of patients. However, complete responses were observed in patients treated with rituximab and anti-CD22 immunotoxins. In Japan, a distinct subtype of HCl known as HCl-Japanese variant (HCl-JV) has been identified. As with HCl-V, patients with HCl-JV have leukocytosis, weak TRAP activity in leukemic cells, and lack of CD25 antigen. In this review, the biology, diagnostic criteria, and current therapeutic options in HCl-V and HCl-JV are presented.
Topics: Antibodies, Monoclonal, Murine-Derived; Antigens, CD; B-Lymphocytes; Humans; Immunity, Cellular; Immunologic Factors; Immunotoxins; Leukemia, Hairy Cell; Rituximab
PubMed: 20558005
DOI: 10.1016/j.ctrv.2010.05.003 -
The New England Journal of Medicine Jun 2019
Topics: Flow Cytometry; Humans; Leukemia, Prolymphocytic, T-Cell; Leukocyte Count; Lymphocytes; Male; Middle Aged; Proto-Oncogene Proteins
PubMed: 31189039
DOI: 10.1056/NEJMicm1814629 -
Clinical Chemistry and Laboratory... Jul 2021
Topics: Cell Transformation, Neoplastic; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphocyte Count; Lymphocytes; Lymphoma, Large B-Cell, Diffuse
PubMed: 33629575
DOI: 10.1515/cclm-2020-1799 -
Cancer Control : Journal of the Moffitt... Jan 1998BACKGROUND: T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy with aggressive clinical course. Although T-PLL has been referred to under...
BACKGROUND: T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy with aggressive clinical course. Although T-PLL has been referred to under different designations, it is a distinct clinico-biological entity and should be distinguished from other T-cell disorders. METHODS: The literature on T-PLL is reviewed. Experience on the clinical and laboratory features, differential diagnosis, and therapy on a large series of T-PLL patients is presented. RESULTS: T-PLL affects adults and occurs more frequently in men. The principal disease characteristics are organomegaly, skin lesions, and a raised lymphocyte count. Immunological markers show a post-thymic T-cell phenotype (TdT- CD2+ CD5+ CD3ñ) with strong expression of CD7. A CD4+ CD8- phenotype is seen in two thirds of cases. CD4 and CD8 are coexpressed in 25%, and a CD4- CD8+ phenotype is rare. Cytogenetics show a recurrent abnormality inv(14)(q11;q32) that is always associated to other aberrations (particularly iso8q or trisomy 8). Differential diagnosis between T-PLL and other T-cell malignancies is based on a constellation of clinical and laboratory features. Generally, T-PLL patients are refractory to the therapy used in lymphoid disorders. Median survival is short but is improving with the use of 2'-deoxycoformycin and the humanized monoclonal antibody, anti-CDw52 (Campath-1H). CONCLUSIONS: T-PLL is a distinct T-cell disorder with characteristic clinical and laboratory features and a poor prognosis. A precise diagnosis of this disease is important in determining patient management and treatment.
PubMed: 10761013
DOI: 10.1177/107327489800500102