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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 -
Human Pathology Nov 1989The pathologic, immunologic, and clinical features of 25 cases of interfollicular (IF) small lymphocytic lymphoma (SLL) characterized by pseudofollicles (PFs) in the IF... (Review)
Review
The pathologic, immunologic, and clinical features of 25 cases of interfollicular (IF) small lymphocytic lymphoma (SLL) characterized by pseudofollicles (PFs) in the IF region of the lymph nodes and by multiple reactive follicles (RFs) were examined. IFSLL is characterized morphologically by variable numbers and sizes of prolymphocytes (nuclei showing one centrally located prominent nucleolus) in the PFs and by small round lymphocytes in the IF region. The lymph nodes in our cases had multiple RFs (100%) and patent or partially patent sinuses (72%), with moderate expansion of the IF region (48%) and typically absent or minimal perinodal infiltration (48%). In 48% of the cases, the PFs surrounded the RFs, producing a pseudo-mantle zone pattern. Immunologic study showed the medium and large prolymphocytes to be mildly LN 1- and LN 2-positive, whereas the small prolymphocytes and lymphocytes were LN 1-negative and moderately LN 2-positive. Few cells in the IF region stained with UCHL-1 antibody. These data indicate the marked preponderance of the non-follicular center cell type of B cells in the IF areas. In all 11 cases tested, a monoclonal B cell population was found. The mean age of the patients was 62 years, with a male to female ratio of 1:1.7. B symptoms were present in 20% of the patients. Nineteen percent of the patients had clinical stage I or II disease, whereas 81% had stage IV disease. The median absolute lymphocyte count was 3,239 X 10(6), with a range of 767 to 13,770 X 10(6) cells/L. In six cases, the lymphocyte count was above 4,000 X 10(6), and in no case was it more than 15,000 X 10(6). It was difficult to distinguish these cases of IFSLL from lymphadenitis and other non-Hodgkin's lymphomas because it was difficult to recognize the subtle PF pattern in the presence of a partially preserved lymph node architecture. Because of the partially retained lymph node architecture and the expansion of the IF region by PFs, this lymphoma is thought to originate from the IF small B lymphocytes, which displayed an in situ growth pattern. Moreover, because of the predominant disease in the lymph nodes and the similarity of features in PFs and follicles, we conclude that IFSLL is a disease that is primary to the lymph nodes. IFSLL should be distinguished from mantle zone lymphoma and chronic lymphocytic leukemia.
Topics: Adult; Aged; Aged, 80 and over; Antigens, Differentiation, B-Lymphocyte; Bone Marrow; Female; Flow Cytometry; Fluorescent Antibody Technique; Humans; Immunohistochemistry; Leukemia, Lymphocytic, Chronic, B-Cell; Leukocyte Count; Lymph Nodes; Lymphocytes; Male; Middle Aged; Phenotype; Stem Cells
PubMed: 2680893
DOI: 10.1016/0046-8177(89)90231-1 -
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 -
Human Pathology Nov 2012We describe 4 patients aged 62 to 79 years with splenomegaly and bone marrow involvement by splenic B-cell lymphoma who developed more than 55% prolymphocytes in blood....
We describe 4 patients aged 62 to 79 years with splenomegaly and bone marrow involvement by splenic B-cell lymphoma who developed more than 55% prolymphocytes in blood. The diagnosis of B-cell prolymphocytic leukemia was considered clinically based on a markedly elevated leukocyte (up to 131.5×10(9)/L) or prolymphocyte (up to 86%) count. Splenectomy was performed in all patients, and spleen weight ranged from 1500 to 2380 g. In 3 patients, the neoplasms were classified as splenic marginal zone lymphoma, and in 1 patient, the neoplasm was classified as splenic diffuse red pulp small B-cell lymphoma. In 2 patients, splenectomy preceded a B-cell prolymphocytic leukemia-like picture, and the spleen showed splenic marginal zone lymphoma or splenic diffuse red pulp small B-cell lymphoma with increased (10%-20%) nucleolated cells consistent with prolymphocytes. In the other 2 patients, a B-cell prolymphocytic leukemia-like picture prompted splenectomy. Initial examination of bone marrow in these patients suggested splenic marginal zone lymphoma. The spleen specimens showed extensive involvement by splenic marginal zone lymphoma with numerous prolymphocytes. Flow cytometry immunophenotyping in all cases showed lymphocytes positive for monotypic surface immunoglobulin (bright), pan-B-cell antigens, CD11c, CD22, and FMC7. Immunohistochemical analysis in all patients showed moderate to bright p53 expression in the spleen (n=3) or bone marrow (n=2). Annexin A1 and cyclin D1 were negative in all cases. Conventional cytogenetic analysis showed del(7q) in 3 patients. We conclude that splenic B-cell lymphoma of various types can undergo prolymphocytoid transformation with more than 55% prolymphocytes in the blood mimicking B-cell prolymphocytic leukemia.
Topics: Aged; Biomarkers, Tumor; Bone Marrow Cells; Cell Transformation, Neoplastic; Chromosome Deletion; Chromosomes, Human, Pair 7; Combined Modality Therapy; Diagnosis, Differential; Female; Flow Cytometry; Humans; Immunophenotyping; In Situ Hybridization, Fluorescence; Leukemia, Prolymphocytic, B-Cell; Lymphoma, B-Cell, Marginal Zone; Male; Middle Aged; Splenectomy; Splenic Neoplasms; Tumor Suppressor Protein p53
PubMed: 22520947
DOI: 10.1016/j.humpath.2012.01.003