Hairy cell leukemia and allied chronic lymphoid leukemias: current knowledge and new therapeutic options. 1997

A Polliack
Department of Hematology, Hadassah University Hospital, Jerusalem, Israel.

This review deals mainly with the essentials of hairy cell leukemia (HCL) detailing clinical aspects, laboratory findings and morphology. Rare manifestations of HCL are listed. Newer aspects relating to cytokines, soluble interleukin receptors and TNF are reviewed. Differential diagnosis including HCL-variant, SLVL, PLL and CLL/PLL are discussed. Prognostic factors and in particular therapeutic aspects are detailed with particular emphasis on the new purine analogues Pentostatin and 2-CdA. A list of suggested reading is offered.

UI MeSH Term Description Entries
D007943 Leukemia, Hairy Cell A neoplastic disease of the lymphoreticular cells which is considered to be a rare type of chronic leukemia; it is characterized by an insidious onset, splenomegaly, anemia, granulocytopenia, thrombocytopenia, little or no lymphadenopathy, and the presence of "hairy" or "flagellated" cells in the blood and bone marrow. Hairy Cell Leukemia,Leukemic Reticuloendotheliosis,Reticuloendotheliosis, Leukemic,Hairy Cell Leukemias,Leukemias, Hairy Cell,Leukemic Reticuloendothelioses,Reticuloendothelioses, Leukemic
D008228 Lymphoma, Non-Hodgkin Any of a group of malignant tumors of lymphoid tissue that differ from HODGKIN DISEASE, being more heterogeneous with respect to malignant cell lineage, clinical course, prognosis, and therapy. The only common feature among these tumors is the absence of giant REED-STERNBERG CELLS, a characteristic of Hodgkin's disease. Non-Hodgkin Lymphoma,Diffuse Mixed Small and Large Cell Lymphoma,Diffuse Mixed-Cell Lymphoma,Diffuse Small Cleaved-Cell Lymphoma,Diffuse Undifferentiated Lymphoma,Lymphatic Sarcoma,Lymphoma, Atypical Diffuse Small Lymphoid,Lymphoma, Diffuse,Lymphoma, Diffuse, Mixed Lymphocytic-Histiocytic,Lymphoma, High-Grade,Lymphoma, Intermediate-Grade,Lymphoma, Low-Grade,Lymphoma, Mixed,Lymphoma, Mixed Cell, Diffuse,Lymphoma, Mixed Lymphocytic-Histiocytic,Lymphoma, Mixed Small and Large Cell, Diffuse,Lymphoma, Mixed-Cell,Lymphoma, Mixed-Cell, Diffuse,Lymphoma, Non-Hodgkin's,Lymphoma, Non-Hodgkin, Familial,Lymphoma, Non-Hodgkins,Lymphoma, Nonhodgkin's,Lymphoma, Nonhodgkins,Lymphoma, Pleomorphic,Lymphoma, Small Cleaved Cell, Diffuse,Lymphoma, Small Cleaved-Cell, Diffuse,Lymphoma, Small Non-Cleaved-Cell,Lymphoma, Small Noncleaved-Cell,Lymphoma, Small and Large Cleaved-Cell, Diffuse,Lymphoma, Undifferentiated,Lymphoma, Undifferentiated, Diffuse,Lymphosarcoma,Mixed Small and Large Cell Lymphoma, Diffuse,Mixed-Cell Lymphoma,Mixed-Cell Lymphoma, Diffuse,Non-Hodgkin's Lymphoma,Reticulosarcoma,Reticulum Cell Sarcoma,Reticulum-Cell Sarcoma,Sarcoma, Lymphatic,Sarcoma, Reticulum-Cell,Small Cleaved-Cell Lymphoma, Diffuse,Small Non-Cleaved-Cell Lymphoma,Small Noncleaved-Cell Lymphoma,Undifferentiated Lymphoma,Diffuse Lymphoma,Diffuse Lymphomas,Diffuse Mixed Cell Lymphoma,Diffuse Mixed-Cell Lymphomas,Diffuse Small Cleaved Cell Lymphoma,Diffuse Undifferentiated Lymphomas,High-Grade Lymphoma,High-Grade Lymphomas,Intermediate-Grade Lymphoma,Intermediate-Grade Lymphomas,Low-Grade Lymphoma,Low-Grade Lymphomas,Lymphatic Sarcomas,Lymphocytic-Histiocytic Lymphoma, Mixed,Lymphocytic-Histiocytic Lymphomas, Mixed,Lymphoma, Diffuse Mixed-Cell,Lymphoma, Diffuse Undifferentiated,Lymphoma, High Grade,Lymphoma, Intermediate Grade,Lymphoma, Low Grade,Lymphoma, Mixed Cell,Lymphoma, Mixed Lymphocytic Histiocytic,Lymphoma, Non Hodgkin,Lymphoma, Non Hodgkin's,Lymphoma, Non Hodgkins,Lymphoma, Nonhodgkin,Lymphoma, Small Non Cleaved Cell,Lymphoma, Small Noncleaved Cell,Lymphosarcomas,Mixed Cell Lymphoma,Mixed Cell Lymphoma, Diffuse,Mixed Lymphocytic-Histiocytic Lymphoma,Mixed Lymphocytic-Histiocytic Lymphomas,Mixed Lymphoma,Mixed Lymphomas,Mixed-Cell Lymphomas,Non Hodgkin Lymphoma,Non Hodgkin's Lymphoma,Non-Cleaved-Cell Lymphoma, Small,Non-Hodgkins Lymphoma,Noncleaved-Cell Lymphoma, Small,Nonhodgkin's Lymphoma,Nonhodgkins Lymphoma,Pleomorphic Lymphoma,Pleomorphic Lymphomas,Reticulosarcomas,Reticulum Cell Sarcomas,Reticulum-Cell Sarcomas,Sarcoma, Reticulum Cell,Small Cleaved Cell Lymphoma, Diffuse,Small Non Cleaved Cell Lymphoma,Small Non-Cleaved-Cell Lymphomas,Small Noncleaved Cell Lymphoma,Small Noncleaved-Cell Lymphomas,Undifferentiated Lymphoma, Diffuse,Undifferentiated Lymphomas
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000970 Antineoplastic Agents Substances that inhibit or prevent the proliferation of NEOPLASMS. Anticancer Agent,Antineoplastic,Antineoplastic Agent,Antineoplastic Drug,Antitumor Agent,Antitumor Drug,Cancer Chemotherapy Agent,Cancer Chemotherapy Drug,Anticancer Agents,Antineoplastic Drugs,Antineoplastics,Antitumor Agents,Antitumor Drugs,Cancer Chemotherapy Agents,Cancer Chemotherapy Drugs,Chemotherapeutic Anticancer Agents,Chemotherapeutic Anticancer Drug,Agent, Anticancer,Agent, Antineoplastic,Agent, Antitumor,Agent, Cancer Chemotherapy,Agents, Anticancer,Agents, Antineoplastic,Agents, Antitumor,Agents, Cancer Chemotherapy,Agents, Chemotherapeutic Anticancer,Chemotherapy Agent, Cancer,Chemotherapy Agents, Cancer,Chemotherapy Drug, Cancer,Chemotherapy Drugs, Cancer,Drug, Antineoplastic,Drug, Antitumor,Drug, Cancer Chemotherapy,Drug, Chemotherapeutic Anticancer,Drugs, Antineoplastic,Drugs, Antitumor,Drugs, Cancer Chemotherapy
D014408 Biomarkers, Tumor Molecular products metabolized and secreted by neoplastic tissue and characterized biochemically in cells or BODY FLUIDS. They are indicators of tumor stage and grade as well as useful for monitoring responses to treatment and predicting recurrence. Many chemical groups are represented including HORMONES; ANTIGENS; amino and NUCLEIC ACIDS; ENZYMES; POLYAMINES; and specific CELL MEMBRANE PROTEINS and LIPIDS. Biochemical Tumor Marker,Cancer Biomarker,Carcinogen Markers,Markers, Tumor,Metabolite Markers, Neoplasm,Tumor Biomarker,Tumor Marker,Tumor Markers, Biochemical,Tumor Markers, Biological,Biochemical Tumor Markers,Biological Tumor Marker,Biological Tumor Markers,Biomarkers, Cancer,Marker, Biochemical Tumor,Marker, Biologic Tumor,Marker, Biological Tumor,Marker, Neoplasm Metabolite,Marker, Tumor Metabolite,Markers, Biochemical Tumor,Markers, Biological Tumor,Markers, Neoplasm Metabolite,Markers, Tumor Metabolite,Metabolite Markers, Tumor,Neoplasm Metabolite Markers,Tumor Markers, Biologic,Tumor Metabolite Marker,Biologic Tumor Marker,Biologic Tumor Markers,Biomarker, Cancer,Biomarker, Tumor,Cancer Biomarkers,Marker, Tumor,Markers, Biologic Tumor,Markers, Carcinogen,Metabolite Marker, Neoplasm,Metabolite Marker, Tumor,Neoplasm Metabolite Marker,Tumor Biomarkers,Tumor Marker, Biochemical,Tumor Marker, Biologic,Tumor Marker, Biological,Tumor Markers,Tumor Metabolite Markers
D015451 Leukemia, Lymphocytic, Chronic, B-Cell A chronic leukemia characterized by abnormal B-lymphocytes and often generalized lymphadenopathy. In patients presenting predominately with blood and bone marrow involvement it is called chronic lymphocytic leukemia (CLL); in those predominately with enlarged lymph nodes it is called small lymphocytic lymphoma. These terms represent spectrums of the same disease. B-Cell Leukemia, Chronic,B-Lymphocytic Leukemia, Chronic,Chronic Lymphocytic Leukemia,Leukemia, B-Cell, Chronic,Leukemia, Lymphocytic, Chronic,Lymphocytic Leukemia, Chronic, B-Cell,Lymphoma, Small Lymphocytic,B-Cell Chronic Lymphocytic Leukemia,B-Cell Malignancy, Low-Grade,Diffuse Well-Differentiated Lymphocytic Lymphoma,Disrupted In B-Cell Malignancy,Leukemia, B Cell, Chronic,Leukemia, Chronic Lymphatic,Leukemia, Chronic Lymphocytic,Leukemia, Chronic Lymphocytic, B-Cell,Leukemia, Lymphoblastic, Chronic,Leukemia, Lymphocytic, Chronic, B Cell,Lymphoblastic Leukemia, Chronic,Lymphocytic Leukemia, Chronic,Lymphocytic Leukemia, Chronic, B Cell,Lymphocytic Lymphoma,Lymphocytic Lymphoma, Diffuse, Well Differentiated,Lymphocytic Lymphoma, Diffuse, Well-Differentiated,Lymphocytic Lymphoma, Well Differentiated,Lymphocytic Lymphoma, Well-Differentiated,Lymphoma, Lymphocytic,Lymphoma, Lymphocytic, Diffuse, Well Differentiated,Lymphoma, Lymphocytic, Diffuse, Well-Differentiated,Lymphoma, Lymphocytic, Well Differentiated,Lymphoma, Lymphocytic, Well-Differentiated,Lymphoma, Lymphoplasmacytoid, CLL,Lymphoma, Small Lymphocytic, Plasmacytoid,Lymphoma, Small-Cell,Lymphoplasmacytoid Lymphoma, CLL,Small-Cell Lymphoma,B Cell Chronic Lymphocytic Leukemia,B Cell Leukemia, Chronic,B Cell Malignancy, Low Grade,B Lymphocytic Leukemia, Chronic,B-Cell Leukemias, Chronic,B-Cell Malignancies, Low-Grade,B-Lymphocytic Leukemias, Chronic,CLL Lymphoplasmacytoid Lymphoma,CLL Lymphoplasmacytoid Lymphomas,Chronic B-Cell Leukemia,Chronic B-Cell Leukemias,Chronic B-Lymphocytic Leukemia,Chronic B-Lymphocytic Leukemias,Chronic Lymphatic Leukemia,Chronic Lymphatic Leukemias,Chronic Lymphoblastic Leukemia,Chronic Lymphoblastic Leukemias,Chronic Lymphocytic Leukemias,Diffuse Well Differentiated Lymphocytic Lymphoma,Disrupted In B Cell Malignancy,Leukemia, Chronic B-Cell,Leukemia, Chronic B-Lymphocytic,Leukemias, Chronic B-Cell,Leukemias, Chronic B-Lymphocytic,Leukemias, Chronic Lymphatic,Leukemias, Chronic Lymphoblastic,Low-Grade B-Cell Malignancies,Low-Grade B-Cell Malignancy,Lymphatic Leukemia, Chronic,Lymphatic Leukemias, Chronic,Lymphoblastic Leukemias, Chronic,Lymphocytic Leukemias, Chronic,Lymphocytic Lymphoma, Small,Lymphocytic Lymphomas,Lymphocytic Lymphomas, Small,Lymphocytic Lymphomas, Well-Differentiated,Lymphoma, CLL Lymphoplasmacytoid,Lymphoma, Small Cell,Lymphoma, Well-Differentiated Lymphocytic,Lymphomas, CLL Lymphoplasmacytoid,Lymphomas, Lymphocytic,Lymphomas, Small Lymphocytic,Lymphomas, Small-Cell,Lymphomas, Well-Differentiated Lymphocytic,Lymphoplasmacytoid Lymphomas, CLL,Malignancies, Low-Grade B-Cell,Malignancy, Low-Grade B-Cell,Small Cell Lymphoma,Small Lymphocytic Lymphoma,Small Lymphocytic Lymphomas,Small-Cell Lymphomas,Well-Differentiated Lymphocytic Lymphoma,Well-Differentiated Lymphocytic Lymphomas
D015463 Leukemia, Prolymphocytic A chronic leukemia characterized by a large number of circulating prolymphocytes. It can arise spontaneously or as a consequence of transformation of CHRONIC LYMPHOCYTIC LEUKEMIA. Prolymphocytic Leukemia,Leukemias, Prolymphocytic,Prolymphocytic Leukemias
D015649 Pentostatin A potent inhibitor of ADENOSINE DEAMINASE. The drug induces APOPTOSIS of LYMPHOCYTES, and is used in the treatment of many lymphoproliferative malignancies, particularly HAIRY CELL LEUKEMIA. It is also synergistic with some other antineoplastic agents and has immunosuppressive activity. 2'-Deoxycoformycin,CI-825,Co-Vidarabine,Deoxycoformycin,Imidazo(4,5-d)(1,3)diazepin-8-ol, 3-(2-deoxy-beta-D-erythro-pentofuranosyl)-3,4,7,8-tetrahydro-, (R)-,NSC-218321,Nipent,2' Deoxycoformycin,CI 825,CI825,NSC 218321,NSC218321

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