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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 -
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 -
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 -
Drugs May 1993Fludarabine is a fluorinated purine analogue with antineoplastic activity in lymphoproliferative malignancies. Noncomparative studies have shown response rates in... (Review)
Review
Fludarabine is a fluorinated purine analogue with antineoplastic activity in lymphoproliferative malignancies. Noncomparative studies have shown response rates in patients with chronic lymphocytic leukaemia (CLL) that appear to be at least equivalent to those achieved with conventional therapy. Prolymphocytic leukaemia, a form of CLL which is often resistant to conventional chemotherapy, has also responded to treatment with fludarabine. Fludarabine combined with prednisone has also induced good response rates in patients with CLL but the limited data suggest that this does not offer an advantage over fludarabine alone. Several clinical trials have demonstrated a good cytoreductive response in patients with advanced, low grade non-Hodgkin's lymphoma, particularly that of follicular histology. Preliminary evidence indicates fludarabine in combination with cytarabine has therapeutic activity as salvage therapy in patients with acute leukaemia. Myelosuppression has been the major dose-limiting adverse effect reported in clinical trials of fludarabine. A reduction in CD4+ cell count may be associated with the increased incidence of fever and opportunistic infections in fludarabine recipients. Nausea and vomiting have also been commonly reported, but these are generally mild to moderate in severity. Reversible neurotoxicity has also been occasionally reported. Thus, fludarabine appears to offer a viable alternative to currently used treatments in CLL and low grade non-Hodgkin's lymphoma. Other potential areas of use for fludarabine include acute leukaemias. Waldenstrom's macroglobulinaemia and mycosis fungoides. However, trials of fludarabine in comparison with currently used therapies and also in combination with other antineoplastic agents are required to fully define its role in the treatment of lymphoid malignancies. In addition, because relapse in these diseases is common, long term trials assessing improvement in survival duration and quality of life would be of value.
Topics: Animals; Antineoplastic Agents; Humans; Neoplasms; Neoplasms, Experimental; Vidarabine
PubMed: 7686467
DOI: 10.2165/00003495-199345050-00009 -
Indian Journal of Pathology &... 2022Anaplastic large cell lymphoma (ALCL) is a subcategory of the mature T-cell neoplasm characterized by sheets of cluster of differentiation (CD)30-positive pleomorphic... (Review)
Review
Anaplastic large cell lymphoma (ALCL) is a subcategory of the mature T-cell neoplasm characterized by sheets of cluster of differentiation (CD)30-positive pleomorphic large cells mostly present as lymphadenopathy. Here, we describe a case of Small cell variant ALCL with leukemic presentation without lymphadenopathy. A 68-year-old male presented with fatigue and weakness; examination revealed a total leukocyte count of 295,000/uL. The peripheral smear showed cells having cerebriform nuclei comprising 90% of the leukocytes. The flow cytometry showed that the cells were immunopositive for CD3 (weak), CD4, CD7, and negative for the rest of the markers. The cell blocks from the peripheral blood showed cells with immunopositivity for CD30, anaplastic lymphoma kinase (ALK), and Epithelial membrane antigen (EMA). A diagnosis of the small cell variant of ALK-positive ALCL was made. Due to the presence of atypical pleomorphic cells without lymphadenopathy, the case has a diagnostic dilemma with differential diagnosis of Sezary syndrome, T-cell prolymphocytic leukemia, and adult T-cell leukemia/lymphoma. Karyotyping and additional immunohistochemistry help for the confirmation of the diagnosis.
Topics: Adult; Aged; Humans; Ki-1 Antigen; Leukemia; Lymphadenopathy; Lymphoma, Large-Cell, Anaplastic; Male; Receptor Protein-Tyrosine Kinases
PubMed: 35900509
DOI: 10.4103/ijpm.ijpm_443_21 -
Translational Cancer Research Jul 2023B-cell prolymphocytic leukemia (B-PLL) is a rare mature B-cell tumor with an aggressive clinical course and poor prognosis. It is characterized by prominent splenomegaly...
BACKGROUND
B-cell prolymphocytic leukemia (B-PLL) is a rare mature B-cell tumor with an aggressive clinical course and poor prognosis. It is characterized by prominent splenomegaly and prolymphocytes exceeding 55% of the lymphoid cells in the blood. Purine analog-based chemo-immunotherapy is the first-line therapy for B-PLL. Owing to its rarity, there are few reports on the efficacy of bendamustine and rituximab (BR) regimen. Our study presents three cases of BR being effective in the treatment of B-PLL and provides experience for clinical treatment.
CASE DESCRIPTION
This report describes the cases of three male patients (median age: 66 years old) who initially presented with abdominal discomfort. Physical examinations and imaging revealed splenomegaly, while a peripheral blood (PB) smear revealed a prolymphocyte count exceeding 70% of the lymphoid cells. Therefore, the three patients were diagnosed with B-PLL. Further molecular detection showed that they harbored P53 abnormalities (17p deletion/ mutation) associated with resistance to conventional chemotherapies. In addition, one of the patients had a highly complex karyotype and multiple gene mutations. All patients underwent four cycles of BR, and two of them received two further cycles of rituximab monotherapy. Ultimately, the patients achieved a complete response (CR) that lasted for 25, 33, and 34 months, respectively, with a median follow-up time of 34 months. The adverse events of the BR mainly included a grade 3 haematological toxicities. Also, the treatment was well-tolerated.
CONCLUSIONS
This case series suggests that BR regimen is promising for bringing deep remission to patients with B-PLL. Prospective trials are still required for further elucidation.
PubMed: 37588745
DOI: 10.21037/tcr-23-828