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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 -
Acta Cytologica 2008Prolymphocytes are nucleolated cells that are the defining features of the 2 chronic lymphoproliferative disorders, prolymphocytic leukemia (PLL) and chronic lymphocytic...
BACKGROUND
Prolymphocytes are nucleolated cells that are the defining features of the 2 chronic lymphoproliferative disorders, prolymphocytic leukemia (PLL) and chronic lymphocytic leukemia (CLL) with increased prolymphocytes. Prolymphocytes appear relatively unfamiliar in cytopathology practice, and, particularly when present in body fluids, may resemble blasts or adult T-cell leukemia/ lymphoma (ATLL) cells.
CASE
A 32-year-old man, referred to us with a diagnosis of acute leukemia, presented with shortness of breath for 2 months and loss of appetite for 3 months. He had enlarged liver and spleen, 6 and 8 cm, respectively, below the costal margin and pleural effusion. The raised total leukocyte count chiefly comprised prolymphocytes that, especially in the pleural fluid, had prominent nucleoli and significant pleomorphism, raising the possibility of blasts or ATLL.
CONCLUSION
Prolymphocytes in body fluids can be misinterpreted as blasts or even ATLL cells. Better awareness among cytopathologists about prolymphocytes and the disease states in which they occur, as well as insistence, in a clinical setting of leukemia, on interpreting the pleural fluid in relation to the clinical and laboratory findings, especially those of the peripheral blood and bone marrow, can prevent misdiagnosis. Equally importantly, immunophenotyping must be done in such situations.
Topics: Adult; Antigens, CD; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Diagnostic Errors; Doxorubicin; Humans; Immunohistochemistry; Immunophenotyping; Leukemia, Myelogenous, Chronic, BCR-ABL Positive; Leukemia, Prolymphocytic, T-Cell; Leukemia-Lymphoma, Adult T-Cell; Male; Pleural Effusion, Malignant; Precursor Cells, T-Lymphoid; Prednisone; Treatment Outcome; Vincristine
PubMed: 18500006
DOI: 10.1159/000325493 -
Urology Annals 2017An 82-year-old man presented with high-grade fever, left flank pain with dysuria. Urine culture revealed the growth of . Contrast-enhanced computed tomography features...
An 82-year-old man presented with high-grade fever, left flank pain with dysuria. Urine culture revealed the growth of . Contrast-enhanced computed tomography features were suggestive of xanthogranulomatous pyelonephritis (XPN) of the left kidney. Serial hemogram studies revealed markedly raised white cell count with the presence of blast cells. On further evaluation by peripheral blood smears and bone marrow biopsy studies, a background disease setting of acute prolymphocytic leukemia was diagnosed. This is a very rare case report of acute leukemia masquerading as a case of XPN, and the optimum treatment protocol is yet to be established in such a scenario.
PubMed: 28479773
DOI: 10.4103/0974-7796.204179 -
Haematologica Jan 2022T-cell prolymphocytic leukemia (T-PLL) is mostly characterized by aberrant expansion of small- to medium-sized prolymphocytes with a mature post-thymic phenotype, high...
T-cell prolymphocytic leukemia (T-PLL) is mostly characterized by aberrant expansion of small- to medium-sized prolymphocytes with a mature post-thymic phenotype, high aggressiveness of the disease and poor prognosis. However, T-PLL is more heterogeneous with a wide range of clinical, morphological, and molecular features, which occasionally impedes the diagnosis. We hypothesized that T-PLL consists of phenotypic and/or genotypic subgroups that may explain the heterogeneity of the disease. Multi-dimensional immuno-phenotyping and gene expression profiling did not reveal clear T-PLL subgroups, and no clear T-cell receptor a or β CDR3 skewing was observed between different T-PLL cases. We revealed that the expression of microRNA (miRNA) is aberrant and often heterogeneous in T-PLL. We identified 35 miRNA that were aberrantly expressed in T-PLL with miR-200c/141 as the most differentially expressed cluster. High miR- 200c/141 and miR-181a/181b expression was significantly correlated with increased white blood cell counts and poor survival. Furthermore, we found that overexpression of miR-200c/141 correlated with downregulation of their targets ZEB2 and TGFβR3 and aberrant TGFβ1- induced phosphorylated SMAD2 (p-SMAD2) and p-SMAD3, indicating that the TGFβ pathway is affected in T-PLL. Our results thus highlight the potential role for aberrantly expressed oncogenic miRNA in T-PLL and pave the way for new therapeutic targets in this disease.
Topics: Gene Expression Profiling; Humans; Leukemia, Prolymphocytic, T-Cell; Lymphocytes; MicroRNAs; Transforming Growth Factor beta; Zinc Finger E-box Binding Homeobox 2
PubMed: 33596640
DOI: 10.3324/haematol.2020.263756 -
Journal of Clinical Apheresis Dec 2023Chronic lymphocytic leukemia (CLL) is a clonal mature B-cell neoplasm with a typically indolent clinical course. Though most clinicians follow these neoplasms through... (Review)
Review
Chronic lymphocytic leukemia (CLL) is a clonal mature B-cell neoplasm with a typically indolent clinical course. Though most clinicians follow these neoplasms through observation alone, an aggressive transformation to prolymphocytic leukemia, diffuse large-B-cell lymphoma (Richter transformation) or classical Hodgkin lymphoma requires immediate attention. We present a case of extreme leukocytosis (>1 million/μL) in a previously diagnosed CLL patient. Due to symptomatic leukostasis, she was started on cytoreductive therapies including leukocytapheresis. After three rounds of leukocytapheresis (LCP) and concurrent chemotherapy, her white blood cell count decreased from a maximum 1262 × 10 /μL to 574 × 10 /μL. To our knowledge, CLL with symptomatic leukostasis that required therapeutic LCP is rarely reported in literature. We propose that therapeutic LCP is of value in such rare, yet dangerous settings like our case.
Topics: Female; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Leukapheresis; Leukostasis; Leukocyte Count; Leukocytosis
PubMed: 37519096
DOI: 10.1002/jca.22082 -
Cancer Genetics and Cytogenetics Jun 1997In a prospective study of 93 consecutive untreated patients with B-cell chronic lymphocytic leukemia, we examined the clinical relevance of surface immunoglobulin (sIg)... (Review)
Review
In a prospective study of 93 consecutive untreated patients with B-cell chronic lymphocytic leukemia, we examined the clinical relevance of surface immunoglobulin (sIg) heavy chain (HC) and light chain (LC) isotypes, CD11c or CD25 expression, and the presence of trisomy 12 by fluorescence in situ hybridization (FISH). Careful morphologic evaluation was performed to exclude patients with other forms of chronic lymphoid leukemias, including mantle cell lymphoma, prolymphocytic leukemia, and leukemia phase of lymphoma. In addition, clonally restricted sIg and CD5 surface determinant were expressed in all patients. Clinical presentation, including blood cell counts, clinical stage, and organomegaly, did not correlate with any of the measured variables. After a median follow-up period of 3 years, the particular HC or LC isotype or CD11c expression did not correlate with either disease progression or treatment-free survival. However, trisomy 12 and CD25 expressions were both associated with accelerated disease progression and a shorter treatment-free survival time. Our results confirm the adverse prognostic significance of trisomy 12 expression in chronic lymphocytic leukemia and suggest that CD25 expression may have an unfavorable clinical impact.
Topics: Adult; Aged; Aged, 80 and over; Chromosomes, Human, Pair 12; Female; Humans; Immunophenotyping; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Middle Aged; Trisomy
PubMed: 9169037
DOI: 10.1016/s0165-4608(96)00249-x -
Blood Aug 1995We studied 25 T-cell chronic lymphocytic leukemia (T-CLL) cases collected over a 15-year period. Immunophenotypic analysis was performed in each case; 12 cases were... (Review)
Review
We studied 25 T-cell chronic lymphocytic leukemia (T-CLL) cases collected over a 15-year period. Immunophenotypic analysis was performed in each case; 12 cases were evaluated by cytogenetics, and gene rearrangement studies were performed in 14 cases. The median age was 57 years with a male predominance (M:F, 15:10). The median presenting lymphocyte count was 36.3 x 10(9)/L (range, 3.9 to 438 x 10(9)/L). Fourteen patients (56%) had shotty adenopathy and ten (40%) had mild-to-moderate splenomegaly at presentation; four (16%) had erythematous skin lesions. The lymphocytes were predominantly small; some cases had a minor component of medium-sized cells (< 10%). The nuclear: cytoplasmic ratios were uniformly high with round to oval nuclei; however, a wide spectrum of nuclear outlines could be found, ranging from minimally to markedly convoluted. Nucleoli were either absent or small and inconspicuous. These lymphocytes did not have the morphology of prolymphocytes and did not contain cytoplasmic granules. Bone marrow infiltration was generally in an interstitial pattern; the degree of involvement ranged from 15% to 90%. Immunophenotyping showed that the lymphocytes were mature T-cells with a predominant CD4+ immunophenotype. Three cases displayed a CD8+ immunophenotype. The patients were treated with a variety of chemotherapeutic regimens with only a minimal response observed in two of 20 patients. We conclude that T-CLL is an uncommon chronic lymphoproliferative disorder (CLPD) that can be morphologically similar to B-CLL, is distinct from T-prolymphocytic leukemia, and has an aggressive clinical course that is refractory to therapy. It may also be difficult to distinguish T-CLL from other T-CLPD, especially the leukemic phase of peripheral T-cell lymphoma and some cases of Sézary syndrome.
Topics: Adult; Aged; Bone Marrow; Female; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; Humans; Immunophenotyping; Leukemia, Prolymphocytic, T-Cell; Male; Middle Aged; Survival Analysis
PubMed: 7620169
DOI: No ID Found -
Leukemia & Lymphoma Jan 1994A 64-year-old woman is reported with Stage I (Rai) chronic lymphocytic leukaemia (CLL), in whom hypercalcemia developed when an increased proportion of prolymphocytic... (Review)
Review
A 64-year-old woman is reported with Stage I (Rai) chronic lymphocytic leukaemia (CLL), in whom hypercalcemia developed when an increased proportion of prolymphocytic cells characterized a transformation of CLL in prolymphocytoid leukaemia (CLL/PL). Although hypercalcemia is more frequently found in T-cell leukaemia associated with human T lymphotropic lymphocyte type I, scattered reports indicate that patients with B-CLL can also be affected with this metabolic disturbance. The case described here, progressed with an indolent course of CLL for 26 months, when she was admitted with a very aggressive disease characterized by a high WBC count, splenomegaly and hypercalcemia. Despite an effort to achieve a clinical remission, she failed treatment and death was attributed to unresponsive hypercalcemia. The mechanism of hypercalcemia in such cases is unclear as no parathyroid adenoma or second malignant tumor was found ante mortem. This electrolytic disturbance would appear to be a direct consequence of the transforming leukaemia and a possible mechanism involving a secreted humoral factor that could lead to altered calcium metabolism.
Topics: Antigens, CD; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Chlorambucil; Cyclophosphamide; Female; Humans; Hypercalcemia; Immunophenotyping; Leukemia, Lymphocytic, Chronic, B-Cell; Leukemia, Prolymphocytic; Middle Aged; Prednisone; Vincristine
PubMed: 8167564
DOI: 10.3109/10428199409059606 -
Indian Journal of Pathology &... 2021We report a 52-year-old man who presented with erythroderma and nodular lesions on face manifesting as "Leonine facies". He had impaired sensation over the face and was...
We report a 52-year-old man who presented with erythroderma and nodular lesions on face manifesting as "Leonine facies". He had impaired sensation over the face and was initially diagnosed to have lepromatous leprosy and was treated with antileprosy drugs. Investigations showed a total Leukocyte count of 550 X 10/l with 90% atypical lymphoid cells with prominent central nucleolus suggestive of prolymphocytes. On flow cytometry, these cells were positive for cytoplasmic CD3, CD2, CD5, CD7, CD4, and CD38 (dim) and were negative for CD1a and TdT and diagnosis of T-prolymphocytic leukemia was made.
Topics: Antigens, CD; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Dermatitis, Exfoliative; Doxorubicin; Facies; Humans; Leukemia, Prolymphocytic, T-Cell; Lymphocyte Count; Male; Middle Aged; Prednisone; Skin; Vincristine
PubMed: 34673613
DOI: 10.4103/IJPM.IJPM_301_20