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Leukemia & Lymphoma May 2022The study aim was to analyze incidence and presentation features of chronic lymphocytic leukemia (CLL) in Chile, in Amerindian population and in non-Native. Between 2012...
The study aim was to analyze incidence and presentation features of chronic lymphocytic leukemia (CLL) in Chile, in Amerindian population and in non-Native. Between 2012 and 2019, 912 patients were diagnosed, and 13 (1.4%) were Amerindian. The estimated incidence in Chilean population was 1.17/100,000 person per year, while in Amerindian, 0.09/100,000 person per year. Median age was 73 years. At diagnosis, 48, 27, and 25%, had low (0), intermediate (I/II) and high-risk (III/IV) disease on Rai classification. Diagnostic immunophenotypic Matutes score was ≥4 in 90%. Median follow-up was 37 months (range 2-87). 5-year OS was 56%, with median overall survival (OS) not reached. It was worse in men, ≥65 years, high-risk and those with increased prolymphocytes (CLL/PL). This study shows low incidence and worse OS in Chilean CLL patients, compared to those from European countries, despite similar clinical features. It also demonstrates that CLL is very uncommon in Amerindian population.
Topics: Aged; Chile; Humans; Immunophenotyping; Incidence; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphocyte Count; Male
PubMed: 34886754
DOI: 10.1080/10428194.2021.2012663 -
Annales de Pathologie Jan 2023T cell prolymphocytic leukemia (T-PLL) is a rare, aggressive neoplasm derived from post-thymic T cells. Patients are typically middle-aged with a slight male...
T cell prolymphocytic leukemia (T-PLL) is a rare, aggressive neoplasm derived from post-thymic T cells. Patients are typically middle-aged with a slight male predominance who present with a high white blood cell count, hepatosplenomegaly, lymphadenopathy, and other symptoms typically associated with leukemia. Although cutaneous involvement has been reported in up to 30% of cases of T-PLL, to our knowledge, none have presented with a presentation resembling livedoid vasculopathy. In the correct clinical context, an underlying hematolymphoid neoplasm should be included in the differential diagnosis of a patient presenting with livedoid vasculopathy.
Topics: Middle Aged; Humans; Male; Female; Leukemia, Large Granular Lymphocytic; T-Lymphocytes; Livedoid Vasculopathy
PubMed: 36494259
DOI: 10.1016/j.annpat.2022.09.004 -
British Journal of Haematology Aug 1981Twelve patients with B-cell prolymphocytic leukaemia (PLL) were treated with splenic irradiation at a weekly dose of 100 cGy to a maximum total dose of 1000 cGy. There...
Twelve patients with B-cell prolymphocytic leukaemia (PLL) were treated with splenic irradiation at a weekly dose of 100 cGy to a maximum total dose of 1000 cGy. There was no morbidity associated with this treatment. SEven patients responded. Three achieved a good response and four a partial response. Two of the patients who had a partial response have subsequently died of unrelated causes. Four of the five patients who failed to respond have died as a result of their disease. When more than 25% of the prolymphocytes formed rosettes with mouse red blood cells (MRBC) the patients appeared to respond better to splenic irradiation. There was no correlation between response and the initial white cell count or the size of the spleen.
Topics: Aged; B-Lymphocytes; Female; Humans; Leukemia, Lymphoid; Lymphocytes; Male; Middle Aged; Radiotherapy Dosage; Rosette Formation; Spleen; Splenomegaly
PubMed: 6974001
DOI: 10.1111/j.1365-2141.1981.tb02755.x -
Journal of the Indian Medical... Jul 1985
Topics: Humans; Leukemia, Myeloid, Acute; Leukocyte Count; Lymphocytes; Male; Middle Aged
PubMed: 3866798
DOI: No ID Found -
Seminars in Oncology Oct 1999Certain features that make an antigen a good candidate for antibody therapy have been defined. CD52 is a 21- to 28-kd nonmodulating cell surface... (Review)
Review
Certain features that make an antigen a good candidate for antibody therapy have been defined. CD52 is a 21- to 28-kd nonmodulating cell surface glycosylphosphatidylinositol-linked glycoprotein that is abundantly expressed (up to 5 x 10(5) molecules per cell) on most normal and malignant lymphocytes and monocytes. Its functions are unknown. CD52 is an excellent target for complement-mediated lysis and antibody-dependent cellular cytotoxicity. A series of rat and genetically reshaped human CD52 antibodies has been assessed for the ability to deplete lymphocytes in vivo to induce immunosuppression and for the treatment of lymphoid malignancies. CD52 antibodies that activate both complement and antibody-dependent cellular cytotoxicity consistently deplete lymphocytes from blood, spleen, and bone marrow but are less effective against lymph node disease or extranodal masses. CD52 antibodies provide effective therapy for chronic leukemias, such as T-cell prolymphocytic leukemia and some subtypes of B-cell chronic lymphocytic leukemia, that may be resistant to conventional chemotherapy. For example, most patients with T-cell prolymphocytic leukemia, including those with large tumor burdens and high peripheral white blood cell counts, will enter complete remission using the antibody CAMPATH-1H without any evidence of tumor lysis. In contrast, in chronic lymphocytic leukemia, CD52 antibodies may be more effective in the setting of minimal residual disease and may allow harvesting of uncontaminated stem cells. Further experiments to enhance the activity of CD52 antibodies in sites refractory to antibody alone are currently being undertaken.
Topics: Alemtuzumab; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Antibodies, Neoplasm; Antigens, CD; Antigens, Neoplasm; Antineoplastic Agents; CD52 Antigen; Glycoproteins; Humans; Leukemia; Lymphoma
PubMed: 10561018
DOI: No ID Found -
Annals of Diagnostic Pathology Feb 2023T-prolymphocytic leukemia (T-PLL) is a rare, aggressive T-cell leukemia, and patients typically present with marked peripheral blood lymphocytosis. Approximately... (Review)
Review
T-prolymphocytic leukemia (T-PLL) is a rare, aggressive T-cell leukemia, and patients typically present with marked peripheral blood lymphocytosis. Approximately 15-20 % of patients may present with moderate and relative stable lymphocytosis and an indolent clinical course that can persist for a few years. However, eventually these patients go on to develop marked lymphocytosis and rapidly progressive disease. We report a 72-year-old man who presented with multicompartmental lymphadenopathy and a normal complete blood count. Excision of left and right cervical lymph nodes showed replacement of the lymph node architecture by a small T-cell neoplasm positive for CD3, CD4, CD5, CD7 and TCL-1, and negative for CD8, CD20, CD30 and ALK. Subsequent bone marrow evaluation showed minimal bone marrow involvement by a T-cell neoplasm associated with TCL1A rearrangement in 11 % of cells supporting the diagnosis of T-PLL. Despite treatment, he showed progressive lymphadenopathy while remarkably maintaining normal white blood cell counts until he eventually developed leukocytosis of 110.9 × 10/uL 26 months later. Review of the literature identified only a single abstract reporting a patient with a similar lymphoma-like presentation and normal white blood cell count; however, that case showed significant bone marrow involvement in stark contrast to the current case. In summary, we report a highly unusual case of T-PLL can initially presenting with an aleukemic or lymphoma-like clinical picture, which can make establishing the diagnosis challenging.
Topics: Male; Humans; Aged; Lymphocytosis; Leukemia, Prolymphocytic; Leukemia, Prolymphocytic, T-Cell; Bone Marrow; Lymphadenopathy
PubMed: 36549077
DOI: 10.1016/j.anndiagpath.2022.152077 -
The American Journal of Case Reports Mar 2020BACKGROUND Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and mantle cell lymphoma (MCL) both have a common origin arising from mature CD5+... (Review)
Review
Composite Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma and Mantle Cell Lymphoma; Small Cell Variant: A Real Diagnostic Challenge. Case Presentation and Review of Literature.
BACKGROUND Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and mantle cell lymphoma (MCL) both have a common origin arising from mature CD5+ B-lymphocytes. Their distinction is crucial since MCL is a considerably more aggressive disease. Composite lymphoma consisting of CLL/SLL and MCL has been rarely reported. This type of composite lymphoma may be under-diagnosed as the 2 neoplasms have many features in common, both morphologically and immunophenotypically. CASE REPORT We report the case of a 57-year-old male patient who presented with a 4-month history of recurrent abdominal pain and distention with hepatosplenomegaly. Peripheral blood showed a high leukocytes count (46.7×10³/uL) with marked lymphocytosis of 35.0×10³/uL, mostly small mature-looking, with some showing nuclear irregularities, with approximately 3% prolymphocytes. Immunophenotyping by flow cytometry and immunohistochemistry revealed 2 immunophenotypically distinct abnormal CD5+monotypic B-cell populations. Fluorescence in situ hybridization (FISH) on peripheral blood demonstrated IGH/CCND1 rearrangement consistent with t(11;14) in 65% of cells analyzed. Accordingly, based on compilation of findings from morphology, flow cytometry, immunohistochemistry, and FISH, A diagnosis of composite lymphoma consisting of MCL; small cell variant and CLL/SLL was concluded. CONCLUSIONS We describe a case of composite lymphoma of MCL (small cell variant) and CLL/SLL that emphasizes the crucial role of the multiparametric approach, including vigilant cyto-histopathologic examination, immunophenotyping by flow cytometry and immunohistochemistry, as well as genetic testing, to achieve the correct diagnosis.
Topics: Biomarkers, Tumor; Composite Lymphoma; Diagnosis, Differential; Flow Cytometry; Gene Rearrangement; Humans; Immunohistochemistry; Immunophenotyping; In Situ Hybridization, Fluorescence; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphoma, Mantle-Cell; Male; Middle Aged; Oncogene Proteins, Fusion
PubMed: 32150530
DOI: 10.12659/AJCR.921131 -
Journal of Cutaneous Pathology Jul 2021T-cell prolymphocytic leukemia (T-PLL) is a rare, aggressive neoplasm derived from post-thymic T-cells. Patients are typically middle aged with a slight male...
T-cell prolymphocytic leukemia (T-PLL) is a rare, aggressive neoplasm derived from post-thymic T-cells. Patients are typically middle aged with a slight male predominance who present with a high white blood cell count, hepatosplenomegaly, lymphadenopathy, and other symptoms typically associated with leukemia. Although cutaneous involvement has been reported in up to 30% of cases of T-PLL, to our knowledge, none have presented with a presentation resembling livedoid vasculopathy. In the correct clinical context, an underlying hematolymphoid neoplasm should be included in the differential diagnosis of a patient presenting with livedoid vasculopathy.
Topics: Aged; Alemtuzumab; Antineoplastic Agents, Alkylating; Antineoplastic Agents, Immunological; Bendamustine Hydrochloride; Biopsy; Diagnosis, Differential; Disease Progression; Exanthema; Extremities; Fatal Outcome; Humans; Hyperpigmentation; Immunohistochemistry; Leukemia, Prolymphocytic, T-Cell; Male; Skin Neoplasms; Torso; Vascular Diseases
PubMed: 33837964
DOI: 10.1111/cup.14023 -
Haematologica Jan 2022T-cell prolymphocytic leukemia (T-PLL) is a poor-prognostic mature T-cell malignancy. It typically presents with exponentially rising lymphocyte counts, splenomegaly,...
T-cell prolymphocytic leukemia (T-PLL) is a poor-prognostic mature T-cell malignancy. It typically presents with exponentially rising lymphocyte counts, splenomegaly, and bone marrow infiltration. Effective treatment options are scarce and a better understanding of TPLL's pathogenesis is desirable. Activation of the TCL1 proto-oncogene and loss-of-function perturbations of the tumor suppressor ATM are TPLL's genomic hallmarks. The leukemic cell reveals a phenotype of active T-cell receptor (TCR) signaling and aberrant DNA damage responses. Regulatory networks based on the profile of microRNA (miR) have not been described for T-PLL. In a combined approach of small-RNA and transcriptome sequencing in 46 clinically and moleculary well-characterized T-PLL, we identified a global T-PLL-specific miR expression profile that involves 34 significantly deregulated miR species. This pattern strikingly resembled miR-ome signatures of TCR-activated T cells. By integrating these T-PLL miR profiles with transcriptome data, we uncovered regulatory networks associated with cell survival signaling and DNA damage response pathways. Despite a miR-ome that discerned leukemic from normal T cells, there were also robust subsets of T-PLL defined by a small set of specific miR. Most prominently, miR-141 and the miR- 200c-cluster separated cases into two major subgroups. Furthermore, increased expression of miR-223-3p as well as reduced expression of miR-21 and the miR-29 cluster were associated with more activated Tcell phenotypes and more aggressive disease presentations. Based on the implicated pathobiological role of these miR deregulations, targeting strategies around their effectors appear worth pursuing. We also established a combinatorial miR-based overall survival score for T-PLL (miROS-T-PLL), that might improve current clinical stratifications.
Topics: DNA Damage; Humans; Leukemia, Prolymphocytic, T-Cell; Lymphocyte Activation; MicroRNAs; T-Lymphocytes
PubMed: 33543866
DOI: 10.3324/haematol.2020.267500 -
Journal of Hematopathology Jun 2022Aggressive subtypes of non-Hodgkin lymphoma may uncommonly be referred to clinical oncologists for treatment of acute leukemia, due to an elevated or rapidly rising...
Aggressive subtypes of non-Hodgkin lymphoma may uncommonly be referred to clinical oncologists for treatment of acute leukemia, due to an elevated or rapidly rising white blood cell count (WBC), with circulating neoplastic cells that morphologically resemble leukemic blasts seen in acute myeloid or lymphoblastic leukemia. We describe six cases of non-Hodgkin lymphoma that mimicked acute leukemia and were identified in the pathology records of the Brigham and Women's Hospital. The patients were older adults (mean age 70 years), who presented with leukocytosis (mean 79.7 × 10/L) with circulating neoplastic cells (mean 57%), which mimicked leukemic blasts, thrombocytopenia, and anemia (4/6 patients). In each case, immunophenotypic analysis identified a population of mature B cells or mature T cells. We identified 15 additional cases of non-Hodgkin lymphoma in the literature that mimicked acute leukemia; considering all 21 cases, 11 had an appearance of acute lymphoblastic leukemia, 4 had an appearance of acute monocytic leukemia, and 6 had an appearance of acute leukemia unable to be further categorized. In general, patients exhibited poor overall survival. These cases illustrate the importance of comprehensive immunophenotypic analysis in the initial evaluation of hematolymphoid neoplasms, and that occasional cases of non-Hodgkin lymphomas can resemble acute leukemia at initial presentation.
PubMed: 35496359
DOI: 10.1007/s12308-022-00493-9