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Frontiers in Public Health 2021Follow-up observation of radiation accident in which a worker developed acute radiation disease and eventually died of leukemia. The case provided key practical...
Follow-up observation of radiation accident in which a worker developed acute radiation disease and eventually died of leukemia. The case provided key practical information for the study on clinical effects of radiation on the health of workers. We observed and followed-up the progression and effect of radiation exposure at various stages in a 28-year-old male patient. We examined the chromosomal morphology, white blood cell count, and sperm count. Laboratory tests for leukemia diagnosis and other clinical parameters were performed. After the patient was irradiated, the white blood cell level decreased, the sperm count dropped to 0, and the libido completely disappeared. The patient's chromosome aberration cell rate and total chromosome aberration cell rate were 7.33 and 7.66%, respectively. Examination of leukemia diagnostic experiments revealed that abnormal cells accounted for 60%; bone marrow examination showed that prolymphocytes abnormally proliferated, accounting for 89%, and had positive extracellular iron staining. After the initial treatment, the patient's white blood cell level increased and was finally maintained at a normal level, the sperm count returned to normal levels, and libido was restored. The patient died of acute lymphoblastic leukemia 34 years after the exposure. More attention has been paid to the long-term effects of ionizing radiation-induced malignant tumors. The occupational protection of radiographic inspection workers should be strengthened to reduce and avoid occupational injuries to protect the health and safety of workers.
Topics: Adult; Chromosome Aberrations; Humans; Male; Occupational Exposure; Occupations; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Radiation Exposure
PubMed: 34055721
DOI: 10.3389/fpubh.2021.657564 -
Cancer Jul 1980Over an 18-year period a distinctive large cell lymphoreticular neoplasm (Richter's transformation) developed in 9 patients with chronic lymphocytic leukemia. Clinical...
Over an 18-year period a distinctive large cell lymphoreticular neoplasm (Richter's transformation) developed in 9 patients with chronic lymphocytic leukemia. Clinical findings at the onset of Richter's transformation were remarkably uniform and consisted of the abrupt onset of fever, marked asymmetric lymphadenopathy with the formation of masses, splenomegaly, and hepatomegaly. All patients underwent rapid clinical deterioration followed by death within six and a half months. Earliest infiltrates of large lymphoreticular cells were identified in the lymph nodes in 3 of 4 patients and the bone marrow in 3 of 9 patients, while no patient had peripheral blood involvement. Autopsy examinations revealed extensive infiltrates of large lymphoreticular cells, predominantly in bone marrow, lymph nodes, liver, spleen, but also in kidney, lung, and gastrointestinal tract. In each case, these large lymphoblast-like and pleomorphic lymphoreticular cells were admixed with mature-appearing lymphocytes and intermediate forms (prolymphocytes). Electron microscopic and immunoperoxidase studies provided additional evidence that this highly aggressive lymphoreticular neoplasm represents a transformation or dedifferentiation of chronic lymphocytic leukemia.
Topics: Adult; Aged; Bone Marrow; Cytoplasm; Hodgkin Disease; Humans; Immunoglobulin Heavy Chains; Immunoglobulin Light Chains; Leukemia, Lymphoid; Leukocyte Count; Leukopenia; Lymph Nodes; Lymphocytes; Lymphoma, Large B-Cell, Diffuse; Male; Microscopy, Electron; Middle Aged; Platelet Count; Prognosis; Syndrome
PubMed: 6770990
DOI: 10.1002/1097-0142(19800701)46:1<118::aid-cncr2820460120>3.0.co;2-j -
Journal of Investigative Medicine High... 2021B-cell prolymphocytic leukemia (B-PLL) is a rare leukemia characterized by rapidly increasing leukocytosis with splenomegaly and lymphadenopathy. Treatment strategies...
B-cell prolymphocytic leukemia (B-PLL) is a rare leukemia characterized by rapidly increasing leukocytosis with splenomegaly and lymphadenopathy. Treatment strategies are largely based on studies of chronic lymphocytic leukemia (CLL). Antibodies against the cell surface protein CD20 are considered to be first-line therapy. A 76-year-old male with known CLL presented 2 weeks after starting chemoimmunotherapy for newly refractory CLL after failing ibrutinib therapy. White blood cell count was elevated at 226.7 × 10/µL. Fluorescent in situ hybridization analysis of a bone marrow specimen showed new development of complex cytogenetics. Flow cytometry revealed B cells appearing slightly dimmer on CD45 and brighter on CD20 compared with typical B-CLL suggestive of less mature lymphocyte forms. The patient was diagnosed with B-PLL and started on obinutuzumab and venetoclax with rapid normalization of white blood cells. This case recapitulates the challenges in diagnosing and treating B-PLL. Ibrutinib resistance is a growing area of study with several proposed mechanisms of acquired resistance. The pathogenesis of B-PLL is not completely understood, although mutations in are presumed to play a role.
Topics: Aged; Humans; Immunotherapy; In Situ Hybridization, Fluorescence; Leukemia, Lymphocytic, Chronic, B-Cell; Leukemia, Prolymphocytic; Male
PubMed: 33533282
DOI: 10.1177/2324709621990767 -
Hematology (Amsterdam, Netherlands) Dec 2023T-prolymphocytic leukemia (T-PLL) is an aggressive hematologic malignancy. A portion of patients can be cured with alemtuzumab induction followed by allogeneic...
T-prolymphocytic leukemia (T-PLL) is an aggressive hematologic malignancy. A portion of patients can be cured with alemtuzumab induction followed by allogeneic hematopoietic stem cell transplant, but patients who relapse after transplant have a poor prognosis, and there is no standard of care. We report a case of a 64-year-old man with relapsed JAK3-mutant T-PLL following allogeneic transplant who was treated with ruxolitinib and venetoclax. Treatment with ruxolitinib and venetoclax resulted in a partial response including stabilization of the peripheral lymphocyte count, improvement in thrombocytopenia, decrease in splenomegaly, and a numerical reduction in the percentage of bone marrow involved by T-PLL. The combination was well tolerated with the exception of neutropenic infections. This case adds to the growing body of literature supporting venetoclax and rituximab as a viable treatment option for relapsed/refractory T-PLL with JAK-STAT alterations.
Topics: Male; Humans; Middle Aged; Leukemia, Prolymphocytic; Nitriles; Pyrimidines; Leukemia, Prolymphocytic, T-Cell
PubMed: 37485976
DOI: 10.1080/16078454.2023.2237342 -
Science Translational Medicine Jun 2015T cell prolymphocytic leukemia (T-PLL) is a rare, mature T cell neoplasm with distinct features and an aggressive clinical course. Early relapse and short overall... (Clinical Trial)
Clinical Trial
T cell prolymphocytic leukemia (T-PLL) is a rare, mature T cell neoplasm with distinct features and an aggressive clinical course. Early relapse and short overall survival are commonplace. Use of the monoclonal anti-CD52 antibody alemtuzumab has improved the rate of complete remission and duration of response to more than 50% and between 6 and 12 months, respectively. Despite this advance, without an allogeneic transplant, resistant relapse is inevitable. We report seven complete and one partial remission in eight patients receiving alemtuzumab and cladribine with or without a histone deacetylase inhibitor. These data show that administration of epigenetic agents can overcome alemtuzumab resistance. We also report epigenetically induced expression of the surface receptor protein CD30 in T-PLL. Subsequent treatment with the anti-CD30 antibody-drug conjugate brentuximab vedotin overcame organ-specific (skin) resistance to alemtuzumab. Our findings demonstrate activity of combination epigenetic and immunotherapy in the incurable illness T-PLL, particularly in the setting of previous alemtuzumab therapy.
Topics: Aged; Alemtuzumab; Antibodies, Monoclonal, Humanized; Antineoplastic Combined Chemotherapy Protocols; Brentuximab Vedotin; Cell Proliferation; Chromatin; Drug Resistance, Neoplasm; Epigenesis, Genetic; Female; Gene Expression Regulation, Leukemic; Humans; Immunoconjugates; Ki-1 Antigen; Leukemia, Prolymphocytic, T-Cell; Leukocyte Count; Male; Middle Aged; Oligonucleotide Array Sequence Analysis; Promoter Regions, Genetic; RNA, Messenger; Real-Time Polymerase Chain Reaction; STAT5 Transcription Factor; Skin; Treatment Outcome
PubMed: 26109102
DOI: 10.1126/scitranslmed.aaa5079 -
British Journal of Haematology Apr 1991Among other patient and disease characteristics, different morphological lymphocyte subtypes were analysed in 146 patients with chronic lymphocytic leukaemia (CLL) to...
Among other patient and disease characteristics, different morphological lymphocyte subtypes were analysed in 146 patients with chronic lymphocytic leukaemia (CLL) to establish their clinical significance and prognostic value. The univariate analysis selected, among other well-known variables, the following lymphocyte subtypes as significant in prognosis: prolymphocytes, granulated lymphocytes, cleaved lymphocytes and small-size lymphocytes. The presence of prolymphocytes and cleaved lymphocytes was correlated with a poor prognosis, whereas granular lymphocytes and small-size lymphocytes were related to a good prognosis. A multivariate regression analysis showed that, besides clinical stages, haemoglobin level, WBC count, age, percentage of bone marrow erythroid cells, and sex, only prolymphocytes had independent prognostic significance. Prolymphocyte percentage correlated positively with characteristics expressing tumour mass such as WBC count, blood absolute lymphocyte count, serum lactate dehydrogenase level, number of enlarged lymph nodes, splenomegaly, and a high number of lymphocytes in bone marrow aspirate. Finally, a prolymphocyte threshold of 5 x 10(9)/l was found to be useful not only to separate two different groups of patients in the whole series but also in Rai's stages II and III + IV, and in Binet's stages A and C.
Topics: Aged; Aged, 80 and over; Analysis of Variance; Female; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Leukocyte Count; Lymphocytes; Male; Middle Aged; Multivariate Analysis; Prognosis
PubMed: 2025572
DOI: 10.1111/j.1365-2141.1991.tb08613.x -
Leukemia Research 1992Peripheral blood samples from 148 previously untreated patients with chronic B-lymphocytic leukemia (B-CLL) were analyzed with the Technicon H*1 flow cytometer. The...
Flow cytochemical analysis of peripheral lymphocytes in chronic B-lymphocytic leukemia. Prognostic role of the blast count determined by the H*1 system and its correlation with morphologic features.
Peripheral blood samples from 148 previously untreated patients with chronic B-lymphocytic leukemia (B-CLL) were analyzed with the Technicon H*1 flow cytometer. The absolute number and the percentage values of both LUCs (large unstained cells) and blasts were correlated with survival, as well as with well-known prognostic factors including morphological subtypes of lymphoid cells. Results showed that patients at the most advanced clinical stages (Rai: III and IV; Binet: C) had the highest percentage and count of both LUCs and blasts. Furthermore, the proportion of LUC positively correlated with the following prognostic factors: peripheral lymphocytosis (greater than 50 x 10(9)/l); marked splenomegaly (greater than 10 cm UCM); % of circulating prolymphocytes, % immunoblasts, and % LGL. Our data analysis further revealed that chemotherapy produced a greater reduction of both the LUCs and of the blast count than of that of small lymphocytes. An increase in LUC count was found to coincide with deterioration of clinical status (progressive changes in the clinical stages, occurrence of prolymphocytoid transformation). A rapid increase in blast count was found to occur in concomitance with the development of Richter's syndrome, and correlated positively with the number of peripheral immunoblasts determined by light microscopy. Moreover, a blast percentage higher than 7% had the strongest predictive relation to survival rate when compared with other hematological parameters (lymphocytosis greater than 50 x 10(9)/l, % of LUCs greater than 12%, LUC to lymphocyte ratio greater than 16%, LUCs count greater than 2.2 x 10(9)/l). In the light of these findings, it may be suggested that the presence both of larger proportions of LUCs and of blasts measured with the flow cytometry may be considered unfavorable prognostic factors in B-CLL. However, based on morphological and multivariate statistical analyses, the blast count proved to be the most important prognostic parameter determined by the H*1 system in B-CLL.
Topics: Adult; Aged; Aged, 80 and over; Female; Flow Cytometry; Follow-Up Studies; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Leukocyte Count; Lymphocyte Subsets; Lymphocytes; Male; Middle Aged; Multivariate Analysis; Neoplasm Staging; Prognosis; Proportional Hazards Models; Regression Analysis; Survival Rate
PubMed: 1635382
DOI: 10.1016/0145-2126(92)90014-x -
Medicine Sep 2018T-cell prolymphocytic leukaemia (T-PLL) is a rare aggressive lymphoid disease featured by a significant increased lymphocyte count and obvious hepatosplenomegaly with...
RATIONALE
T-cell prolymphocytic leukaemia (T-PLL) is a rare aggressive lymphoid disease featured by a significant increased lymphocyte count and obvious hepatosplenomegaly with poor prognosis. The concomitant presentation of T-PLL and visceral leishmaniasis (VL) has not previously been reported.
PATIENT CONCERNS
The patient initially suffered from anorexia, skin pigmentation, fever and hepatosplenomegaly. Bone marrow smear described leishmania and antibody test was positive. VL was diagnosed and he was given antimony gluconate therapy. His symptoms recurred.
DIAGNOSIS
A combination of serological rk39 test, morphologic evaluation and immunophenotyping by flow cytometry finally supported the diagnosis of concomitant VL and T-PLL.
OUTCOMES
Amphotericin B was used for the treatment of VL first and a referral for treating T-PLL after recovery from VL was suggested. Unfortunately, the patient requested to be discharged. Telephone follow-up indicated that he died a few days after leaving the hospital.
LESSONS
Due to the rarity of the disease combination, the pathogenesis association of T-PLL and VL is unclear. However, a duly diagnosis is crucial for treatment. In immunosuppressed patients due to malignancies and treatment, VL should be considered as an opportunistic infection. In VL infections, the clinical manifestations mimicking hematological malignancies may cover up the underlying disease. Under such conditions, a complete work-up based on laboratory test is necessary to achieve a correct diagnosis.
Topics: Amphotericin B; Antiprotozoal Agents; Fatal Outcome; Hepatomegaly; Humans; Immunophenotyping; Leishmania donovani; Leishmaniasis, Visceral; Leukemia, Prolymphocytic, T-Cell; Male; Middle Aged; Splenomegaly
PubMed: 30235714
DOI: 10.1097/MD.0000000000012410 -
British Journal of Haematology Jan 1987The prognostic value of biological, clinical and laboratory features was analysed in a series of 265 patients with chronic lymphocytic leukaemia (CLL) and prolymphocytic... (Comparative Study)
Comparative Study
The prognostic value of biological, clinical and laboratory features was analysed in a series of 265 patients with chronic lymphocytic leukaemia (CLL) and prolymphocytic leukaemia (PLL). On univariate analysis seven features were shown to influence significantly the survival of the whole group of patients: absolute prolymphocyte count (ABS PROL), percentage of prolymphocytes (%PROL), WBC, spleen size, age, intensity of surface-membrane immunoglobulin (SmIg) and mouse (M) rosettes. Multivariate regression analysis of these features showed that only ABS PROL and spleen size had independent prognostic significance. The survival in PLL (38 cases) was significantly shorter than in CLL (227 cases) (median survival = 3 and 8 years, respectively). Patients with CLL with an increased %PROL (11-55%), defined as CLL/PL, could be divided into two groups: those with ABS PROL less than or equal to 15 X 10(9)/l (26 cases) fell within the 'standard-prognostic risk' for typical CLL (i.e. less than or equal to 10% PROL), whereas the survival outlook for the cases with ABS PROL greater than 15 X 10(9)/l (40 cases) was as bad as for PLL. A scoring system was generated with the four features that showed high prognostic significance: ABS PROL, spleen size, SmIg and M-rosettes. The score proved to be superior to any single feature as a predictor of survival, being especially useful in the analysis of the CLL/PL group: cases with high scores (greater than 2) had a median survival of 2.5 years, while the median has not been reached for those with low scores (less than or equal to 2). We suggest that this scoring system may help to identify the cases of CLL/PL that behave as PLL, and as such may benefit from different treatment.
Topics: Humans; Leukemia, Lymphoid; Leukocyte Count; Prognosis
PubMed: 3468997
DOI: 10.1111/j.1365-2141.1987.tb06130.x -
Blood Advances Nov 2019This is the first report of successful treatment of therapy-resistant leptomeningeal T-PLL with intrathecal alemtuzumab. Intrathecal alemtuzumab is a potentially safe...
This is the first report of successful treatment of therapy-resistant leptomeningeal T-PLL with intrathecal alemtuzumab. Intrathecal alemtuzumab is a potentially safe and efficacious therapeutic alternative for treatment of leptomeningeal T-PLL.
Topics: Alemtuzumab; Antineoplastic Agents, Immunological; Biopsy; Blood Cell Count; Combined Modality Therapy; Drug Resistance, Neoplasm; Female; Humans; Immunophenotyping; Injections, Spinal; Leukemia, Prolymphocytic, T-Cell; Meningeal Neoplasms; Middle Aged; Molecular Targeted Therapy; Retreatment; Treatment Outcome
PubMed: 31698446
DOI: 10.1182/bloodadvances.2019000289