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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 -
European Journal of Haematology May 2017T-cell prolymphocytic leukemia (T-PLL) is a very rare, aggressive T-cell neoplasm. Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is also a highly...
OBJECTIVES
T-cell prolymphocytic leukemia (T-PLL) is a very rare, aggressive T-cell neoplasm. Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is also a highly aggressive lymphoma. These two diseases can often be confused with each other; therefore, we aimed to determine the clinical and pathological differences between T-PLL and PTCL-NOS.
METHODS
We analyzed 15 T-PLL and 91 PTCL-NOS patients and also compared clinical features between T-PLL and PTCL-NOS with leukemic presentation. Peripheral blood images and biopsy specimens were analyzed, and treatment responses were determined via imaging modalities. The clinicopathological characteristics were statistically compared.
RESULTS
T-PLL cells were smaller in size than those of PTCL-NOS with leukemic presentation (P=.0068); moreover, PTCL-NOS cells with leukemic presentation were smaller than those of PTCL-NOS without leukemic presentation (P=.0017). Immunophenotypic patterns in T-PLL and PTCL-NOS were similar. Five-year overall survival rates of T-PLL and all PTCL-NOS patients were 57.5% and 36.8%, respectively. No significant differences were found in clinical manifestations or prognoses; T-PLL and PTCL-NOS with leukemic presentation had essentially equivalent characteristics.
CONCLUSION
T-PLL and PTCL-NOS may share common biological and clinical characteristics in Japanese patients.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Biomarkers; Biopsy; Child; Diagnosis, Differential; Female; Gene Rearrangement; Humans; Immunophenotyping; Leukemia, Prolymphocytic, T-Cell; Leukocyte Count; Lymphoma, T-Cell, Peripheral; Male; Middle Aged; Neoplasm Metastasis; Neoplasm Staging; Prognosis; Receptors, Antigen, T-Cell; Treatment Outcome; Young Adult
PubMed: 28129454
DOI: 10.1111/ejh.12856 -
[Rinsho Ketsueki] the Japanese Journal... Jan 2010CD5 is a T-cell marker that is expressed in mature B cell malignancies and other B cell chronic lymphoproliferative disorders, but the biologic function of CD5 is...
CD5 is a T-cell marker that is expressed in mature B cell malignancies and other B cell chronic lymphoproliferative disorders, but the biologic function of CD5 is unknown. We report a 68-year-old woman with B-cell prolymphocytic leukemia (B-PLL) expressing CD5 antigen. On admission, jaundice and hepatosplenomegaly were noted. Hematological examination demonstrated a platelet count of 2.8 x 10(4)/microl and a white blood cell count of 19,900/microl with 69% PLL cells. Surface marker analysis of the PLL cells was positive for CD5, CD19, CD20, sIgM, and was negative for CD23, and cyclin D1 was negative in immunostaining.
Topics: Aged; Biomarkers, Tumor; CD5 Antigens; Diagnosis, Differential; Fatal Outcome; Female; Humans; Leukemia, Prolymphocytic, B-Cell; Leukocyte Count; Platelet Count
PubMed: 20134145
DOI: No ID Found -
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 -
Cureus May 2020The association of warm autoimmune hemolytic anemia (wAIHA) with various lymphoproliferative disorders is well reported in the literature. But the association of wAIHA...
The association of warm autoimmune hemolytic anemia (wAIHA) with various lymphoproliferative disorders is well reported in the literature. But the association of wAIHA with T-cell prolymphocytic leukemia (T-PLL), a very rare lymphoproliferative disorder, has never been reported. A 71-year-old man was in his usual state of health until three years ago when he developed intermittent bouts of worsening anemia associated with mild peripheral blood lymphocytosis. He was diagnosed with wAIHA and steroid therapy was initiated, resulting in an improvement in the hemoglobin level of the patient. His lymphocyte count remained persistently elevated but he did not develop any malignancy-related signs or symptoms. A diagnosis of 'indolent' T-cell prolymphocytic leukemia (small cell variant) was made by combining distinctive clinical, morphologic, immunophenotypic, and cytogenetic analysis. His wAIHA went into complete remission and steroid therapy was successfully tapered off. He has not required any treatment for his T-PLL during the last two years' follow-up.
PubMed: 32523849
DOI: 10.7759/cureus.7994 -
Case Reports in Oncology 2023T-cell prolymphocytic leukemia (T-PLL) is a rare aggressive disease with a poor prognosis. Allogeneic stem cell transplantation (allo-SCT) followed by alemtuzumab...
T-cell prolymphocytic leukemia (T-PLL) is a rare aggressive disease with a poor prognosis. Allogeneic stem cell transplantation (allo-SCT) followed by alemtuzumab administration is the most promising treatment for T-PLL but is associated with a high risk of infections as alemtuzumab strongly suppresses cellular immunity, leading to high transplant-related mortality and unsatisfactory survival rates. In addition, for patients without human leukocyte antigen-matched donors, haploidentical stem cell transplantation (haplo-SCT) using post-transplant cyclophosphamide (PTCy) has been used because of the ready availability of donors and achievement of results comparable to those of transplantation with human leukocyte antigen-matched donors. However, there are no reports on the efficacy and safety, including infectious complications, of haplo-SCT with PTCy after alemtuzumab therapy in patients with. Here, we describe a 66-year-old Japanese male patient with T-PLL treated successfully with haplo-SCT after induction therapy of alemtuzumab for T-PLL. Approximately 3 months after the achievement of complete remission with alemtuzumab for T-PLL, haplo-SCT with reduced-intensity conditioning and PTCy was performed. Infectious complications were improved by early therapeutic interventions, and peripheral T cell counts gradually recovered. The patient was alive for more than 16 months after allo-SCT with no signs of relapse. Thus, haplo-SCT using PTCy should be considered as an option after alemtuzumab treatment for T-PLL.
PubMed: 37900793
DOI: 10.1159/000531471 -
Case Reports in Hematology 2013Prolymphocytic transformation of chronic lymphocytic leukemia is a rare but recognized entity. We present the case of a 76-year-old gentleman with a previous diagnosis...
Prolymphocytic transformation of chronic lymphocytic leukemia is a rare but recognized entity. We present the case of a 76-year-old gentleman with a previous diagnosis of chronic lymphocytic leukemia who presented with fatigue, fever, and a white blood cell count of 500 000 with prolymphocytes on peripheral blood examination. Chlorambucil and dexamethasone were initiated. He developed progressive anemia during his admission with no clear cause on initial CT examination. Bilateral hip pain began several days later and he was unfortunately diagnosed with a large spontaneous retroperitoneal hemorrhage postmortem. This condition is rare and generally occurs in those receiving therapeutic anticoagulation or dialysis, with known bleeding disorders or vascular malformation, none of which were present in our patient. Pathology revealed marked leukemoid engorgement of the vessels of many organs with prolymphocytes. We discuss the potential etiologies and relationships between these critical diagnoses.
PubMed: 24024050
DOI: 10.1155/2013/802376 -
Journal of Investigative Medicine High... 2018We report the case of a patient with B-cell prolymphocytic leukemia who was successfully treated with the novel humanized monoclonal antibody obinutuzumab. This patient...
We report the case of a patient with B-cell prolymphocytic leukemia who was successfully treated with the novel humanized monoclonal antibody obinutuzumab. This patient was previously treated with the combination of rituximab and bendamustine and had recurrent infusion reactions. Her treatment with rituximab and bendamustine was discontinued when she developed disease progression after 3 cycles of therapy. She was then treated with obinutuzumab 1000 mg on day 1 of every cycle and chlorambucil 0.5 mg/kg on days 1 and 15 every 28 days to which she had greater tolerability. After 4 cycles of treatment, she had resolution of her clinical symptoms, massive splenomegaly, and normalization of her white blood cell count.
PubMed: 30038912
DOI: 10.1177/2324709618788674 -
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 -
Cureus Sep 2019B-cell prolymphocytic leukemia (B-PLL) is a rare malignancy of mature B-cells with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features...
B-cell prolymphocytic leukemia (B-PLL) is a rare malignancy of mature B-cells with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features characterized by late onset (median age 69 years), an aggressive clinical course, refractoriness to chemotherapy, and median survival of around three years. Treatment is influenced by the presence or absence of specific high-risk genetic mutations like 17P/TP53 deletion, the presence of which translates into poor prognosis. Patients without 17P deletion, who are <70 years, without significant co-morbidities, are initially treated with a combination chemotherapy regimen used for chronic lymphocytic leukemia (CLL) such as fludarabine, cyclophosphamide, and rituximab. On the other hand, patients with a 17P deletion, age >70 years, with multiple co-morbidities, receive ibrutinib or alemtuzumab as the initial therapy. Relapsed or refractory cases are managed with BCL-2 signaling inhibitors like venetoclax. We discuss the case of an 84-year-old male with B-PLL (positive TP53 mutation), resistant to ibrutinib therapy, with extremely high white blood cell (WBC) counts, thus creating a dilemma regarding the best treatment in the second-line setting.
PubMed: 31700732
DOI: 10.7759/cureus.5629