Small B cell lymphomas and leukemias including hairy cell leukemia
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Introduction 1123 Chronic lymphocytic leukemia 1123 Epidemiology 1124 Diagnosis 1124 Clinical features 1124 Clinical staging and prognostic markers 1125 Clinical staging systems 1125 Traditional prognostic markers 1125 New prognostic markers 1126 Treatment 1127 Pretreatment evaluation 1127 Criteria for response and minimal residual disease 1128 First-line therapy 1129 Treatment at relapse 1132 Stem cell transplantation 1134 New therapies 1135Richter large cell transformation 1135 Management of complications 1136 B cell prolymphocytic leukemia 1137 Immunophenotype and cytogenetics 1137 Clinical course and management 1137 Hairy cell leukemia 1137 Morphology and immunophenotype 1138 Treatment 1138 Splenectomy 1138 Interferon alpha 1138 Pentostatin 1138 Cladribine 1138 Treatment at relapse 1138 Hairy cell leukemia variant 1139 Key points 1139 References 1140Small B cell lymphoproliferative disorders comprise a variety of disease entities, the commonest of which is chronic lymphocytic leukemia (CLL). Rarer primary B cell leukemias include B prolymphocytic leukemia (PLL) and hairy cell leukemia (HCL). In addition, clonal B cells may appear in the peripheral blood originating from a primary non-Hodgkin lymphoma (NHL).Bone marrow aspiration specimens of 5 different leukemia patients were electron microscopically observed. The results showed that the both as acute premyelocyte leukemia diagnosed cases (case 1 and 2) revealed many abnormal premyelocytes which contained cytoplasmic Auer bodies with markedly different structures. The case 3 and 4 were diagnosed as different types of hairy cell leukemia, namely typical hairy cell leukemia (type Ⅰ) and atypical hairy cell leukemia (type Ⅱ). The morphological difference of both type Ⅰ,Ⅱ cells were expressed mainly in the different lengths and numbers of the hairy processes on the cell surface and these differences can only be observed electron microscopically. The case 5 was a partly differentiated type of acute myelocytic leukemia and the cytoplasm of the premyelocyte contained only a few electron dense granules. The electron microscopy of these cells showed a marked superiority in its resolving power than the light microscopy and suggested its significance also in the diagnosis of leukemia.
Hairy Cell
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In response to DNA damage, p53 undergoes post-translational modifications (including acetylation) that are critical for its transcriptional activity. However, the mechanism by which p53 acetylation is regulated is still unclear. Here, we describe an essential role for HLA-B-associated transcript 3 (Bat3)/Scythe in controlling the acetylation of p53 required for DNA damage responses. Depletion of Bat3 from human and mouse cells markedly impairs p53-mediated transactivation of its target genes Puma and p21 . Although DNA damage-induced phosphorylation, stabilization, and nuclear accumulation of p53 are not significantly affected by Bat3 depletion, p53 acetylation is almost completely abolished. Bat3 forms a complex with p300, and an increased amount of Bat3 enhances the recruitment of p53 to p300 and facilitates subsequent p53 acetylation. In contrast, Bat3-depleted cells show reduced p53–p300 complex formation and decreased p53 acetylation. Furthermore, consistent with our in vitro findings, thymocytes from Bat3-deficient mice exhibit reduced induction of puma and p21, and are resistant to DNA damage-induced apoptosis in vivo. Our data indicate that Bat3 is a novel and essential regulator of p53-mediated responses to genotoxic stress, and that Bat3 controls DNA damage-induced acetylation of p53.
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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.
Prolymphocytic leukemia
Chronic leukemia
Hairy Cell
Immunophenotyping
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Deltaretrovirus
Lymphocytosis
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Hairy Cell
Pathogenesis
Deltaretrovirus
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Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal B lymphocytes. it's always classified as a sub-type of leukemia (CLL). Hairy cell leukemia makes up approximately 2% of all leukemias, with fewer than 2,000 new cases diagnosed annually in North America and Western Europe combined.
Hairy Cell
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Holcus lanatus
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HLA-B-associated transcript 3 (BAT3) was originally identified as one of the genes located within human major histocompatibility complex. It encodes a large proline-rich protein with unknown function. In this study, we found that a fragment of the BAT3 gene product interacts with a candidate tumor suppressor, DAN, in the yeast-based two-hybrid system. We cloned the full-length rat BAT3 cDNA from a fibroblast 3Y1 cDNA library. Our sequence analysis has demonstrated that rat BAT3 cDNA is 3617 nucleotides in length and encodes a full-length BAT3 (1098 amino acids) with an estimated molecular mass of 114,801 daltons, which displays an 87.4% identity with human BAT3. The deletion experiment revealed that the N-terminal region (amino acid residues 1-80) of DAN was required for the interaction with BAT3. Green fluorescent protein-tagged BAT3 was largely localized in the cytoplasm of COS cells. Northern hybridization showed that BAT3 mRNA was expressed in all the adult rat tissues examined but predominantly in testis. In addition, the level of BAT3 mRNA expression was more downregulated in some of the transformed cells, including v-mos- and v-Ha-ras-transformed 3Y1 cells, than in the parental cells.
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SUMMARY A hitherto unrecorded virus having flexible rod‐shaped particles about 740–760 × 13 nm was isolated from Anthoxanthum odoratwn L. It was transmitted by sap inoculation, but not by several species of insect, seed or soil to 18 species of Gramineae including wheat, oats and barley. In susceptible species the virus normally produced a mosaic mottling of the leaves which was sometimes followed by a necrotic streaking or striping.
Mosaic virus
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Chronic leukemia
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