T-cell prolymphocytic leukemia. Clinical and immunologic characterization
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The clinical course of a patient with extensive skin involvement due to T-cell prolymphocytic leukemia is described. The malignant cells isolated from the patient's blood and skin were studied utilizing cytochemical analysis and multiple monoclonal antibodies directed against cell surface antigens. The leukemic cells displayed a surface antigen phenotype similar to that of normal post thymic suppressor T-cells. On the basis of this study together with the few published reports, it appears that T-prolymphocytic leukemia is derived from lymphocytes demonstrating either the suppressor or helper phenotype, and that extensive dermal infiltration may be independent of phenotypic classification. Although T-prolymphocytic leukemia shares certain morphologic, cytochemical, and immunologic features with chronic lymphocytic leukemia, it is an aggressive disease with an average survival of approximately 6 months and is best thought of as a distinct pathologic entity. Cancer 52:2049-2054, 1983.Keywords:
Prolymphocytic leukemia
Infiltration (HVAC)
Leukemic B-cell chronic lymphoproliferative disorders (B-CLPD) are a relatively heterogeneous group of diseases, all of which exhibit a clonal expansion of mature-appearing B-lymphoid cells in the peripheral blood (PB). Both primary leukemias and the leukemic phase of primary lymphomas are included in this category (1). Among the primary B-cell leukemias, chronic lymphocytic leukemia (CLL), prolymphocytic leukemia (PLL), and hairy cell leukemia (HCL) are usually considered; within the primary lymphomas, follicular lymphoma (FL), mantle cell lymphoma (MCL), marginal zone splenic lymphoma (MZSL), lymphoplasmacytic lymphoma (LPL), and the large B-cell lymphomas (LCLs) exhibit PB involvement more frequently (1).
Prolymphocytic leukemia
Lymphoplasmacytic Lymphoma
Lymphoproliferative Disorders
Follicular lymphoma
Mantle zone
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CD5
Lymphocytosis
Prolymphocytic leukemia
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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.
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The chronic lymphoid leukemias are a group of generally indolent B cell malignancies that include chronic lymphocytic leukemia (CLL), prolymphocytic leukemia, hairy cell leukemia, and large granular lymphocyte leukemia. The unique aspects of diagnosis and management for each condition are discussed separately, with the primary focus being on CLL, the most common form of leukemia in most Western countries. Charts show the percentages of CLL patients’ survival divided into Rai risk category, treatment-free survival, and overall survival, and an algorithm displays approaches to selecting therapy for CLL patients. Tables list how CLL and other B cell lymphoproliferative disorders can be distinguished using immunophenotyping, chromosome categories by fluorescence in situ hybridization for predicting CLL patient survival, criteria indications for the initiation of therapy from the International Workshop on CLL and the National Cancer Institute CLL Working Group, and criteria for high-risk CLL disease. This review contains 4 highly rendered figures, 4 tables, and 172 references.
Prolymphocytic leukemia
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Prolymphocytic leukemia
Follicular lymphoma
Lymphoproliferative Disorders
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Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in adults in the Western world, accounting for nearly 25% of all leukemias with an estimated annual age-adjusted incidence of 3 per 100,000 persons in the United States. The median age at diagnosis is approximately 70 years, with 81% of the patients diagnosed when aged ≥ 60 years. Under the World Health Organization (WHO) classification, CLL is a B-cell neoplasm and the entity T-cell CLL has been reclassified as T-cell prolymphocytic leukemia (PLL). Recent data from the Surveillance, Epidemiology, and End Results (SEER) cancer statistics indicate that 5-year survival of patients with CLL is 73%.
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The hematological malignancy chronic lymphocytic leukemia (CLL) is the commonest form of leukemia in adult humans in the Western hemisphere, with more than 15,000 cases recognized annually every year. The specific skin infiltrates in patients with B cell CLL (B-CLL) are varied and include localized or generalized erythematous papules, plaques, nodules, and large tumors. CD1d expression is quite variable in the setting of CLL. In most instances it is expressed at lower levels compared to certain other lymphoproliferative disorders. Polymerase chain reaction studies performed on paraffin-embedded tissues using appropriate consensus primers show immunoglobulin heavy chain gene rearrangement. Although T cell prolymphocytic leukemia was formerly considered to be the T cell counterpart of typically insidious B-CLL, the aggressive clinical course that defines most cases prompted reclassification by the World Health Organization (WHO). Skin involvement is common in the course of T cell prolymphocytic leukemia.
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Objective:To improve the understanding of chronic lymphocytic leukemia(CLL) who transformed to highly aggressive hematologic malignant tumors.Methods:Report two cases of CLL who transformed to Hodgkin's lymphoma and large B cell lymphoma respectively.Results:and Conclusion:CLL can transform to many kinds of highly aggressive malignant tumors.Richter's syndrome is the most common transformation of CLL,CLL can also transform to prolymphocytic leukemia,acute lymphocytic leukemia,Hodgkin's lymphoma,multiple myeloma and other none hematologic tumors.All these transformations have poorer prognosis and shorter survival expectance.
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B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias.B-PLL generally occurs in older people.It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts.The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival.Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation.Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD).The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study.The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia.There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis.Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders.The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.
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