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Scandinavian Journal of Haematology May 1979A case of prolymphocytic lymphoma/leukaemia (PL) sensu Galton in a 32-year-old man is presented. The leucocyte count was 19.0 x 10(9)/1 at presentation and tartrate...
A case of prolymphocytic lymphoma/leukaemia (PL) sensu Galton in a 32-year-old man is presented. The leucocyte count was 19.0 x 10(9)/1 at presentation and tartrate resistent acid phosphatase was present in most prolymphocytes. Immunological investigation of prolymphocytes from lymph nodes, spleen and peripheral blood revealed the surface marker phenotype: SmIg + (mu, (delta), lambda), IgG-Fc-receptor +, C3-receptor +. The prolymphocytes from lymph nodes and spleen were C3-receptor + in a high percentage, while only a few were IgG-Fc-receptor +. This proportion was reversed in the blood prolymphocytes. The histology of lymph nodes was unique and strongly suggested a preferential involvement (homing phenomenon) of the mantle zone of the lymphatic follicle. These results may indicate that emission of prolymphocytes from lymph nodes to circulation involves a change of surface receptors. It is finally suggested to consider the diagnosis of not only hairy-cell leukaemia but also PL in the case of tartrate resistent acid phosphatase-positive lymphoma/leukaemia.
Topics: Acid Phosphatase; Adult; B-Lymphocytes; Cells, Cultured; Fluorescent Antibody Technique; Histocytochemistry; Humans; Immunoglobulin Fc Fragments; Leukemia, Lymphoid; Lymph Nodes; Male; Receptors, Antigen, B-Cell; Rosette Formation; Spleen
PubMed: 382343
DOI: 10.1111/j.1600-0609.1979.tb00438.x -
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 -
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 Nov 1978The clinical, histopathological, and cytochemical features of eight patients with prolymphocytic leukemia, a rare variant of chronic lymphocytic leukemia, were reviewed....
The clinical, histopathological, and cytochemical features of eight patients with prolymphocytic leukemia, a rare variant of chronic lymphocytic leukemia, were reviewed. Six of the patients had clinical evidence of "massive" splenomegaly at the time of diagnosis, and in four of these this clinical impression was confirmed by splenic weights in excess of 2000 g. No patient had significant lymph node enlargement. The initial leukocyte count was elevated in seven patients and was greater than 100 X 10(9)/1 in four of them. The absolute prolymphocyte count ranged from 16.3 to 378.1 X 10(9)/1 and was greater than 100 X 10(9)/1 in four patients. Splenectomy in four patients had no lasting effect on the peripheral leukocyte count. In the four patients in whom the disease was shown by surface marker or immunocytochemical studies to be of B-cell origin, the histopathologic features were distinctive and were characterized by a pattern of infiltration which was nodular and diffuse in both the splenic red pulp and the bone marrow, whereas involvement of the lymph nodes was pseudonodular. In one patient in whom the prolymphocytes had cytochemical characteristics suggestive of T-cells, the distribution of the abnormal cellular proliferation in the lymph nodes was paracortical and the infiltrations of the spleen and the bone marrow were diffuse.
Topics: Aged; Bone Marrow; Female; Histocytochemistry; Humans; Leukemia, Lymphoid; Lymph Nodes; Male; Middle Aged; Receptors, Antigen, B-Cell; Splenomegaly
PubMed: 719613
DOI: 10.1002/1097-0142(197811)42:5<2360::aid-cncr2820420537>3.0.co;2-1 -
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 -
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 -
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 -
Leukemia & Lymphoma Oct 1999Hairy cell leukemia-variant (HCL-V) is an extremely rare chronic B-cell lymphoproliferative disorder clinically and morphologically distinct from classic hairy cell...
Hairy cell leukemia-variant (HCL-V) is an extremely rare chronic B-cell lymphoproliferative disorder clinically and morphologically distinct from classic hairy cell leukemia (HCL). HCL-V is thought to represent a hybrid between prolymphocytic leukemia and HCL, the nucleus more closely resembling a prolymphocyte and the cytoplasm a hairy cell. The clinical course of HCL-V is aggressive with short survivals. Since single courses of cladribine have profound activity in HCL, inducing durable complete responses in 91% of patients, we administered cladribine to 4 patients with HCL-V over a 7-year period. During this time interval 357 patients with classic HCL received cladribine at Scripps Clinic. Each patient received cladribine at 0.1 mg/kg per day by continuous intravenous infusion for 7 days, repeated at 28-day intervals depending on response status. The 4 patients ranged in age from 28 to 70. Two presented with B-symptoms, 1 had peripheral adenopathy, and all 4 displayed massive splenomegaly. Peripheral blood counts were notable for lymphocytosis associated with mild anemia and thrombocytopenia. Only 1 of the 4 patients had received prior treatment. Peripheral blood immunophenotypic analysis revealed monoclonal B cells with expression of CD11c in 3 patients, lack of CD25 expression in 3 patients and expression of CD103 in all but 1 patient. The number of cladribine courses administered ranged from two to five. Of these 4 patients, 1 (25%) achieved a complete response and 2 (50%) partial responses, for an overall response rate of 75%. Three patients underwent splenectomy after cladribine. Cladribine is an active agent in HCL-V albeit with a lower response rate than in classic HCL. The role of other treatment modalities, such as splenectomy, interferon-alpha, and 2'-deoxycoformycin, alone or in combination with cladribine awaits further evaluation.
Topics: Adult; Aged; Antineoplastic Agents; Cladribine; Female; Humans; Infusions, Intravenous; Leukemia, Hairy Cell; Male; Middle Aged; Splenectomy; Treatment Outcome
PubMed: 10706459
DOI: 10.3109/10428199909145739