Abstract
A review of the main features of prolymphocytic leukaemia, based on 80 cases studied by the author since 1974, is presented. Prolymphocytes have a B-cell membrane phenotype with strong expression of surface Ig in 81% of cases (B-PLL) whilst the rest have T-cell markers (T-PLL). Both forms of the disease have distinct laboratory and clinical findings. T-PLL has a more aggressive course with a median survival of 7 months (24 months in B-PLL). Ultrastructural analysis demonstrates larger lysosomal granules in T-PLL cells which results in a higher content of acid hydrolases. The intriguing relationship of B-PLL with B-CLL is discussed in the light of new data with immunological markers.
MeSH terms
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Aged
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Animals
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Antineoplastic Agents / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols*
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B-Lymphocytes / immunology
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Cyclophosphamide / administration & dosage
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Doxorubicin / administration & dosage
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Drug Therapy, Combination
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Humans
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Immunity, Cellular
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Karyotyping
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Leukapheresis
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Leukemia, Experimental / immunology
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Leukemia, Lymphoid / classification
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Leukemia, Lymphoid / immunology*
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Leukemia, Lymphoid / therapy
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Lymphocyte Activation
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Mice
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Middle Aged
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Phenotype
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Prednisone / administration & dosage
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Prognosis
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Splenectomy
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T-Lymphocytes / immunology
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Vincristine / administration & dosage
Substances
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Antineoplastic Agents
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Vincristine
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Doxorubicin
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Cyclophosphamide
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Prednisone