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At least 19 recordsLinked to original sources
PubMed1990

Purine metabolism enzymes and immunological phenotype in chronic B-cell malignancies: chronic lymphocytic leukemia, prolymphocytic leukemia and hairy cell leukemia.

The chronic lymphocytic leukemia, the prolymphocytic leukemia and the hairy cell leukemia of B-cell immunophenotype are closely related disorders, but differ in their cytomorphological and clinical features. In an attempt to differentiate further among these forms of leukemia some immunological and cytochemical markers were studied. Simultaneously we measured adenosine deaminase and purine nucleosidephosphorylase activities by a method of paper radiochromatography in peripheral blood/bone marrow leukemic cells from 23 patients with chronic lymphocytic leukemia, 5 patients with prolymphocytic leukemia, one with prolymphocytoid transformation of chronic lymphocytic leukemia and 15 patients with hairy cell leukemia. The mosaic of immunological and cytochemical markers based on the sum of positive and negative features allowed for the correct diagnosis in a majority of cases. From the number of 43 investigated cases we found the typical enzyme patterns in 39 of them. On the basis of purine enzyme activity we were able to differentiate between chronic lymphocytic leukemia versus prolymphocytic and hairy cell leukemia. In one patient with chronic lymphocytic leukemia we could detect very early stage of prolymphocytoid transformation by increased activity of purine nucleosidephosphorylase activity. There were only two patients with chronic lymphocytic leukemia who were assigned to the prolymphocytic leukemia on the basis of purine nucleosidephosphorylase activity and two patients with hairy cell leukemia with atypical enzyme pattern attributable to the nonrepresentative number of pathological cells in the peripheral blood. Our study showed that purine nucleosidephosphorylase activity in leukemia cells may be useful as an additional parameter in the distinction of prolymphocytic from lymphocytic leukemia and that it may represent an enzymatic marker for early detection of prolymphocytoid transformation of chronic lymphocytic leukemia. Characteristic purine enzyme pattern was found also for diagnostic confirmation of hairy cell leukemia.

Adenosine Deaminase↗
PubMed1994

Similar patterns of V kappa gene usage but different degrees of somatic mutation in hairy cell leukemia, prolymphocytic leukemia, Waldenstrom's macroglobulinemia, and myeloma.

To compare V kappa gene usage and the amount of somatic mutation in rearranged Ig genes from patients with lymphoproliferative disorders, we have polymerase chain reaction-amplified and sequenced a total of 26 V kappa genes from a total of 55 cases. Six sequences were obtained both from six cases of prolymphocytic leukemia (PLL) and from nine cases of hairy cell leukemia (HCL). Seven sequences were obtained both from 11 cases of Waldenström's macroglobulinemia (WM) and 29 cases of multiple myeloma (MM). Eleven different germline genes have been used in this series, indicating a wide but nonrandom usage of germline Ig gene rearrangements in these disorders. Comparison of the nucleotide sequences of V kappa genes obtained from B-cell malignancies with germline V kappa genes shows that somatic mutation is rare in PLL and HCL and common in WM and MM. Analysis of the pattern of mutations suggests that WM and MM are derived from B cells that have been selected by antigen at a relatively late stage of differentiation.

Amino Acid Sequence↗
PubMed1988

Cytogenetic studies in chronic lymphocytic leukemia, prolymphocytic leukemia and hairy cell leukemia: a progress report.

We studied the B lymphocyte metaphases in 78 of 94 cases of CLL, 24 of our 28 hairy cell leukaemias, 4 CLL's which transformed into prolymphocytic leukaemia and 8 original prolymphocytic leukaemias. Twelve CLL's were carriers of trisomy 12, 13 of this trisomy 12 with other abnormalities and 15 had other abnormalities without trisomy 12. Trisomy 12 seemed to be more frequent in patients with CLL or prolymphocytic leukaemia with a monoclonal gammopathy. In contrast to our previous results, the isolated finding of a trisomy 12 had an influence on the median survival.

Chromosomes, Human, Pair 12↗
PubMed1991

[Prolymphocytic leukemia].

Prolymphocytic leukemia is an entity characterized by morphologic, immunologic, clinical and behavioral features which allows its keeping apart from chronic lymphoid leukemia. Careful analysis of blood smear is the main step for its diagnosis. Our purpose is to remind this particular chronic lymphoproliferative syndrome which requires energetic initial treatment. Prolymphocytic leukemia de novo as well as a possible transformation of chronic lymphoid leukemia to prolymphocytic leukemia are discussed.

Adult↗
PubMed2005

Prolymphocytic leukemia.

Prolymphocytic leukemia is a rare chronic lymphoproliferative disorder that includes two subtypes, B cell and T cell, each with its own distinct clinical, laboratory and pathological features. T-cell prolymphocytic leukemia has an aggressive course with short median survival and poor response to chemotherapy. With the use of the purine analogue pentostatin more than half of patients will have a major response and a minority will have a complete remission, usually lasting months. With the introduction of alemtuzumab, most patients who progressed despite treatment with pentostatin had a major response with a complete remission rate higher than that obtained with pentostatin when used as a first line. Unfortunately, progression still follows shortly. We recommend alemtuzumab as initial therapy and offer stem cell transplant (SCT) to selected young, healthy patients who respond. Although B-cell prolymphocytic leukemia is also a progressive disease, some patients can achieve a prolonged progression-free-survival with fludarabine. Patients presenting with massive splenomegaly may be effectively palliated with splenic irradiation or splenectomy. Rituximab is a promising agent and further investigations are warranted to better define its role in treatment of this disorder.

Alemtuzumab↗
PubMed1985

Enzymatic differences between chronic lymphocytic leukemia and prolymphocytic leukemia.

Chronic lymphocytic leukemia and prolymphocytic leukemia of the B-cell immunophenotype are closely related disorders, but differ in their cytomorphologic and clinical features. In an attempt to differentiate further between these two forms of leukemia, we measured adenosine deaminase and purine nucleoside phosphorylase activities by using a linked-enzyme spectrophotometric assay on peripheral-blood leukemic cells from seven patients with chronic lymphocytic leukemia, three patients with prolymphocytic leukemia, and one patient with prolymphocytoid transformation of chronic lymphocytic leukemia. By using discriminant analysis, we were able to distinguish the two groups only on the basis of purine nucleoside phosphorylase activity (F1,9; p less than 0.001). The purine nucleoside phosphorylase activity in leukemic cells with prolymphocytic cytomorphology was significantly elevated (mean = 58.6 nM/min/mg protein) compared to the activity in leukemic cells with lymphocytic cytomorphology (mean = 25.6 nM/min/mg protein). There was only one patient with chronic lymphocytic leukemia who was assigned to the prolymphocytic leukemia group on the basis of her purine nucleoside phosphorylase activity. Our study suggests that purine nucleoside phosphorylase activity in leukemic cells may be useful in the distinction of prolymphocytic leukemia from chronic lymphocytic leukemia, and that it may be an enzymatic marker for the early detection of prolymphocytoid transformation of chronic lymphocytic leukemia.

Adenosine Deaminase↗
PubMed1987

Acute tumor lysis syndrome in prolymphocytic leukemia.

Prolymphocytic leukemia is characterized by a low response rate to agents that are effective in the treatment of chronic lymphocytic leukemia. A 75-year-old man with prolymphocytic leukemia was followed up over a period of 69 months. During that time, he was treated with high-dose chlorambucil plus prednisone; cyclophosphamide administered daily; combinations of vincristine sulfate, prednisone, and cyclophosphamide; irradiation to the spleen; and alpha-interferon without substantial antitumor effect. The patient had a brief response after splenectomy. When he received low-dose vincristine therapy daily with high-dose prednisone therapy given on alternate days, a fatal tumor lysis syndrome occurred. Although rare, this complication should be kept in mind, particularly in patients who had rapid reduction of tumor cells.

Acute Disease↗
PubMed1996

High frequency of somatic mutations in the VH genes expressed in prolymphocytic leukemia.

Prolymphocytic leukemia (PLL) is a chronic lymphoproliferative disorder, characterized by prominent splenomegaly, prolymphocytes accounting for more than 55% of circulating lymphocytes, and short-term survival. To better characterize the nature of the cellular origin in this disease, we analyzed lg heavy chain variable region (VH) genes in eleven cases of de novo PLL Leukemic cells expressed a skewed repertoire characterized by predominant use of the V3 family members (73%), with preferential use of the V3-23 gene (50% of the VH3 genes). All sequences from expressed VH genes diverged from their putative germline counterpart, and in eight cases the divergence was greater than 5%. In seven cases, which expressed the V3-23 gene and VH4 family members, nucleotide substitutions could be confidently attributed to somatic mutations. The type and distribution of these mutations clearly indicated that in three cases the cells had been subjected to an antigen selection process. Taken together, these results suggest that B-PLL cells display a skewed repertoire of lg VH regions and probably represent, at least in some instances, expansion of postgerminal center cells that have undergone antigen driven selection.

Adult↗
PubMed1986

Induction of features characteristic of hairy cell leukemia in chronic lymphocytic leukemia and prolymphocytic leukemia cells.

In vitro maturation was induced with 12-O-tetradecanoylphorbol-13-acetate in leukemic cell samples from patients with chronic lymphocytic leukemia (n = 10) and prolymphocytic leukemia (n = 4). The cells were studied for morphology, for immunological markers using the fluorescence activated cell sorter, and for acid phosphatase isoenzymes using both cytochemistry and isoelectrofocusing. Morphologically the induced changes included appearance of cells with an excentric nucleus and basophilic cytoplasm and eventually of cells with many fine cytoplasmic projections ("hairs"). Analysis of immunological markers by flow cytometry revealed that the monoclonal antibody defined cell surface molecule HD6 (CD22), which is strongly expressed on hairy cell leukemia (HCL) but absent from plasmacytoma and plasma cells, can be induced or enhanced in the leukemic samples. In the study of acid phosphatase isoenzymes using cytochemistry we observed the induction of the tartrate resistant isoenzyme. Further, using isoelectrofocusing we could demonstrate the induction of the same band of tartrate resistant acid phosphatase with an isoelectric point of 9.0-9.7 as detected also in HCL. This particular isoenzyme is considered characteristic of HCL but is absent in plasmacytoma. Our data demonstrate that chronic lymphocytic leukemia and prolymphocytic leukemia cells can be induced to realize a common genetic program which bears characteristics of HCL, indicating that these three entities are much more closely related than previously thought.

Acid Phosphatase↗
PubMed1982

Treatment of prolymphocytic leukemia.

Prolymphocytic leukemia is characterized by marked splenomegaly, distinctive cellular morphologic characteristics, and a poor clinical course. Five patients with typical PL were treated systematically with vincristine/prednisone, chlorambucil/prednisone, splenic irradiation, splenectomy, and other chemotherapy regimens. No patient responded to vincristine/prednisone. Two patients responded to chlorambucil/prednisone, and four patients had brief responses to splenic irradiation. Two patients underwent splenectomy, one of whom had a prolonged clinical remission. There were no complete remissions. No other chemotherapy combinations were of value. The median survival was 33 months. Recommendations are made to use chlorambucil/prednisone or splenic irradiation as initial treatment. Splenectomy should be considered in patients refractory to these modalities. The course of PL may be more protracted than originally reported.

Aged↗
PubMed2003

Phase II study of cladribine and cyclophosphamide in patients with chronic lymphocytic leukemia and prolymphocytic leukemia.

BACKGROUND: One of the mechanisms of action of cladribine is the inhibition of DNA repair of damage caused by radiation, alkylating agents, or other drugs. To determine its antitumor activity in combination with cyclophosphamide, we initiated a Phase II trial of the two agents in patients with advanced chronic lymphocytic leukemia (CLL) or prolymphocytic leukemia (PLL). METHODS: Twenty-nine patients with refractory or recurrent CLL or PLL received cladribine 4 mg/m(2)/day and cyclophosphamide 350 mg/m(2)/day (both administered intravenously) for 3 days every 4 weeks. RESULTS: Eleven patients (38%), nine with CLL and two with PLL, had a response. The median duration of response was 12 months. Severe extrahematologic toxicity (National Cancer Institute Grade 3-4) occurred in two patients, consisting of skin rash in one patient and progressive multifocal leukoencephalopathy in the other. The most common form of hematologic toxicity was severe neutropenia, which developed after 25% of the 84 courses was administered. Severe thrombocytopenia and anemia developed after 12% and 7% of the courses, respectively, and five episodes of anemia were immunomediated. In addition, three major infections resulted in the death of one patient. CONCLUSIONS: Although inferior to the combination fludarabine plus cyclophosphamide, this regimen showed interesting activity in patients with advanced CLL or PLL. Myelosuppression was the major dose-limiting toxic effect.

Adult↗
PubMed2002

Alemtuzumab in relapsed or refractory chronic lymphocytic leukemia and prolymphocytic leukemia.

Twenty-three adult patients with relapsed or refractory chronic lymphocytic leukemia (CLL) or prolymphocytic leukemia (PLL) were treated for up to 12 weeks with the anti-CD52 monoclonal antibody alemtuzumab. Patients were a median of six years from diagnosis and had been treated with a median of four chemotherapy regimens (median of 24 total cycles) prior to enrollment. Fourteen patients (61%) had received prior monoclonal antibody therapy with rituximab. Adverse symptoms were primarily mild to moderate fever, rigor/chills, nausea/vomiting, or fatigue/malaise in up to 86% of patients. Patients with low blood counts at the initiation of alemtuzumab tolerated therapy well. A total of 17 patients were evaluable for disease response. Nine patients (53%) responded with complete remissions in the peripheral blood. Of these nine, five were evaluated by bone marrow biopsy with four complete responses (CR) and one partial response. Six of the nine presented with nodal disease at the start of alemtuzumab therapy with three CRs and three partial responses. Alemtuzumab is a monoclonal antibody that offers effective treatment for chemotherapy refractory CLL and PLL and is generally well tolerated in the outpatient setting.

Aged↗
PubMed2002

Cytomegalovirus viremia during Campath-1H therapy for relapsed and refractory chronic lymphocytic leukemia and prolymphocytic leukemia.

Campath-1H is effective therapy for patients with relapsed and refractory chronic lymphocytic leukemia (CLL) and prolymphocytic leukemia (PLL), but it is associated with profound lymphopenia and deficiencies in cell-mediated immunity. We report the incidence of cytomegalovirus (CMV) viremia in 34 patients treated with Campath-1H for relapsed or refractory CLL and PLL. All patients received infection prophylaxis during therapy and continuing for at least 2 months following Campath-1H. Five patients (15%) developed CMV viremia at a median of 28 days (range, 20-30 days) after the first dose of Campath-1H. The median CMV viral load was 860/mL (range, 420-2100/mL), as determined by quantitative plasma polymerase chain reaction (PCR). All 5 patients had a temperature > 38.5 degrees C, normal chest radiographs, normal liver function tests, and negative bacterial blood cultures with no clinical evidence of CMV disease at the time of presentation with CMV viremia. The median absolute neutrophil count (ANC) was 740/ microL (range, 340-1600/ microL), and the median absolute lymphocyte count (ALC) was 16/microL (range, 11-169/ microL) for the 5 patients at the time of CMV viremia. All 5 patients received ganciclovir therapy followed by prompt fever resolution and clearance of CMV viremia by plasma PCR. By univariate regression analysis, the following were not risk factors for CMV viremia: age, number of prior regimens, prior rituximab therapy, prior splenectomy, modified Rai stage at Campath-1H therapy (low/intermediate vs. high), ANC, and ALC; although, there was a trend towards significance for prior rituximab therapy (P = 0.07). Cytomegalovirus viremia may be a significant infectious complication during Campath-1H therapy and should be investigated further in future studies.

Adult↗
PubMed1992

[Immunophenotype of chronic lymphocytic leukemia and prolymphocytic leukemia].

The immunophenotype of chronic lymphocytic leukemia (CLL) and prolymphocytic leukemia (PLL) is described, mainly according to the FAB classification. CLL and related diseases have been recognized as highly variable diseases and their classification is still confusing. Is there true T-CLL? How should we deal with non-T, non-B-CLL such as NK-GLL? What about the patient with borderline manifestations? Is the concept "MLUS" appropriate? Further examination and more detailed analysis is required in order to clarify the disease entity of CLL and related disorders.

Antigens, CD↗
PubMed1987

Cytogenetic studies on prolymphocytic leukemia. II. T cell prolymphocytic leukemia.

We report chromosome abnormalities in 15 cases of T cell prolymphocytic leukemia (T-PLL). All cases were characterized by clinical, morphological, and membrane marker analysis. The most frequent abnormality was an inv(14)(q11q32) observed in nine cases. The T cell receptor (TCR) alpha chain gene is localized to 14q11 and the immunoglobulin heavy-chain gene to region 14q32. Four cases also had translocations involving 14q11. Trisomy or multisomy for 8q resulting from an i(8q) or from rearrangements with 8p12 as the breakpoint was observed in nine cases, and a deletion of 6q was found in four cases. Trisomy or partial trisomy for 7q was observed in four cases, of which two had abnormalities of band 7q35 to which the TCR beta chain gene is mapped. The expression of Tac antigen, investigated in 27 cases of human T cell leukemia virus I-negative chronic T cell leukemia, which included the 15 cases of T-PLL, showed a good correlation with abnormalities of 7q35. Our studies on chronic T leukemias suggest that inv(14)(q11q32) and trisomy for 8q are abnormalities characteristic of T-PLL.

Antibody Diversity↗
PubMed1987

Cytogenetic studies on prolymphocytic leukemia. 1. B-cell prolymphocytic leukemia.

We describe clonal chromosome abnormalities in 13 new cases of B-cell type prolymphocytic leukemia (B-PLL) investigated using pokeweed, lipopolysaccharide B, TPA (phorbol ester), and Epstein-Barr virus (EBV) as mitogens. B-PLL showed a much better response to all four mitogens when compared with B-cell chronic lymphocytic leukemia (B-CLL). A 14q+ was the most frequent abnormality and was observed in 7 of the 13 cases. A t(11;14)(q13;q32) was observed in 2 patients in this series and in 2 cases from a previous series of 9 patients studied in this laboratory, giving an incidence in B-PLL of 18% for this abnormality. The more frequent rearrangement of both IgH genes in B-PLL when compared to B-CLL may predispose to a higher incidence of 14q+ in B-PLL. Trisomy 12 which is a feature of B-CLL was observed in one case in the present series. Other abnormalities of chromosome 12 included 12p-(p12-13) in 2 cases and t(12;14)(q22;q32) in 1 case. The t(6;12) previously described as a specific abnormality in B-PLL was not observed in the 22 cases (13 present series, 9 previous series) studied in our laboratory.

Aged↗
PubMed1987

Chronic lymphocytic leukemia and prolymphocytic leukemia: a clinicopathological reappraisal.

A series of 300 cases of chronic B-cell leukemia was studied in relation to clinical and laboratory features, and three groups were identified on the basis of the percentage of circulating prolymphocytes (%PROL): typical CLL less than or equal to 10% PROL, 174 cases; PLL greater than 55% PROL, 42 cases; and an intermediate group CLL/PL (11%-55% PROL), 84 cases. Some features of the CLL/PL group resemble those of PLL, such as a disproportionate splenomegaly in relation to the degree of lymphnode involvement. However, membrane markers suggested a closer affinity of CLL/PL with CLL [high percentage of M rosettes, expression of the P67 (T1) antigen, and low reactivity with the McAb FMC7], although high-density SmIg was found in one-third of CLL/PL, as well as in the majority of the PLL cases. Cells volume measurements demonstrated that the prolymphocytes of both PLL and CLL/PL are significantly larger than the homogeneous population of small lymphocytes of typical CLL. Followup studies of the PB picture in CLL and CLL/PL showed that the majority of patients maintain a relatively stable percentage of PROL, but a progressive prolymphocytoid transformation to a PLL-like disease may occur in some cases. On univariate analysis of survival, seven features of disease had a high prognostic values for the whole group of patients: %PROL, absolute number of PROL (ABS PROL), WBC, spleen size, M rosettes, SmIg intensity, and age. However, only ABS PROL (greater than 15 X 10(9)/l) and spleen size (greater than 8 cm) were shown to be independent prognostic features on a multivariate regression analysis. The median survival time of patients with PLL (3 years) was significantly shorter than the median of 8 years for patients with CLL. Within the heterogeneous CLL/PL group, patients with ABS PROL greater than 15 X 10(9)/l (two-thirds) had a median survival time as bad as for PLL patients, whereas the median has not been reached for those with ABS PROL less than 15 X 10(9)/l.

Humans↗