A chronic lymphoproliferative disorder with distinctive features: a distinct variant of hairy-cell leukaemia
| Autor: | J. C. Cawley, Frank G. J. Hayhoe, G. F. Burns |
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| Rok vydání: | 1980 |
| Předmět: |
Cancer Research
Pathology medicine.medical_specialty Monocytopenia Neutropenia Lymphocyte Activation Peripheral blood mononuclear cell Monocytes Organomegaly Phagocytosis Bone Marrow hemic and lymphatic diseases Humans Medicine Aged Hairy Cell Leukemia Variant Leukemia Hairy Cell business.industry Hematology Middle Aged medicine.disease Microscopy Electron Haematopoiesis medicine.anatomical_structure Oncology Antigens Surface Immunology Red pulp Female Lymph Nodes Bone marrow medicine.symptom business Spleen |
| Zdroj: | Leukemia Research. 4:547-559 |
| ISSN: | 0145-2126 |
| DOI: | 10.1016/0145-2126(80)90066-1 |
| Popis: | Two patients with a unique combination of clinical, haematological, pathological and immunological features are described. These features included a chronic course without significant constitutional symptoms, substantial splenomegaly without other organomegaly, a high WBC count with many abnormal mononuclear cells but without neutropenia or monocytopenia, and an easily aspirable marrow with well-preserved haematopoiesis and a modest infiltrate of mononuclear cells. The splenic architecture was completely replaced by a diffuse infiltrate of mononuclear cells, with prominent involvement of the red pulp. The bone marrow was also diffusely involved, but increased reticulin fibres were not a feature. Neither the MCV nor the LAP score was elevated. The abnormal mononuclear cells possessed striking surface hairs and strong tartrate-resistant acid phosphatase, but differed markedly from the pathognomonic cells of hairy-cell leukaemia in their internal structure. The cells were phagocytic and their surface-marker phenotype was E−γFc+μFc+C3−Mo−SIgG+. These cases are compared and contrasted with similar clinical entities including hairy-cell leukaemia, chronic lymphocytic leukaemia, prolymphocytic leukaemia and certain non-Hodgkin's lymphomata and it is concluded that the features of their disease are sufficiently distinctive to justify their recognition as a distinct variant of hairy-cell leukaemia. It is proposed to term the entity Type II hairy-cell leukaemia as compared with the usual form of hairy-cell leukaemia, Type I. |
| Databáze: | OpenAIRE |
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