scholarly journals Chronic Myelocytic Leukemia in Identical Twins and a Sibling

Blood ◽  
1968 ◽  
Vol 31 (2) ◽  
pp. 216-225 ◽  
Author(s):  
GEORGE K. TOKUHATA ◽  
CHARLES L. NEELY ◽  
DOROTHY L. WILLIAMS

Abstract Identical twins and their older brother have been studied: all diagnosed within a span of three months as having chronic myelocytic leukemia; both twins were symptomatic and the brother asymptomatic. Chromosome analyses were made on peripheral blood cells. The asymptomatic brother had never been treated. The Ph1 chromosome was present in each of the three siblings. A number of other chromosome abnormalities were found. Results were interpreted in terms of a probable genetic factor in the Philadelphia chromosome and susceptibility to chronic myelocytic leukemia.

2013 ◽  
Vol 2 (3) ◽  
pp. 141
Author(s):  
Muthia Rendra ◽  
Rismawati Yaswir ◽  
Akmal M Hanif

AbstrakLeukemia merupakan penyakit keganasan sel darah yang berasal dari sumsum tulang ditandai oleh proliferasi sel-sel darah putih, dengan manifestasi adanya sel-sel abnormal dalam darah tepi. Pada tahun 2006, leukemia berada pada urutan ke-5 dari keseluruhan penderita kanker di Indonesia. Leukemia kronik merupakan leukemia yang paling sering terjadi pada dewasa dan lanjut usia. Secara umum leukemia kronik diklasifikasikan atas Leukemia Granulositik Kronik (LGK) dan Leukemia Limfositik Kronik (LLK). Leukemia kronik yang perjalanannya lambat dan diiringi oleh gejala yang tidak khas, maka penelitian ini bertujuan untuk mengetahui gambaran laboratorium leukemia kronik di bagian Peyakit Dalam RSUP DR. M. Djamil Padang. Jenis penelitian ini adalah deskriptif retrospektif. Instrumen yang digunakan pada penelitian ini adalah data sekunder yang diperoleh dari Instalasi Rekam Medik RSUP Dr. M. Djamil Padang berupa data pasien leukemia kronik yang dirawat di Bagian Penyakit Dalam RSUP Dr. M. Djamil Padang sejak 1 Januari 2010 – 31 Desember 2012. Hasil penelitian ini menunjukkan bahwa dari 16 kasus leukemia granulositik kronik terdapat 37,5% pasien mengalami anemia sedang, 100% leukositosis, jumlah trombosit dapat menurun, normal, dan meningkat dengan presentase masing-masing 25%, 25%, dan 50%. Gambaran eritrosit sebagian besar normositik anisositosis. Separuh pemeriksaan darah tepi menunjukkan peningkatan persentasi mielosit, 31,25% menunjukkan peningkatan persentasi metamielosit dan eosinofil, serta sebagian besar menunjukkan presentasi blast. Sedangkan gambaran sumsum tulang hiperseluler, penekanan eritropoetik, mielopoetik hiperaktif, dan trombopoetik dalam batas normal. Leukemia limfositik kronik yang terdiri dari 1 kasus menunjukkan gambaran laboratorium berupa anemia sedang, leukositosis, trombositopenia, gambaran eritrosit nomokrom anisositosis, peningkatan jumlah leukosit, peningkatan jumlah limfosit, presentasi smudge cell, dan ditemukan presentasi blast pada darah tepi, tetapi selularitas tidak dapat dinilai.Kata kunci: leukemia kronik, darah tepi, BMPAbstractLeukemia is a malignant disease of blood cells derived from the bone marrow characterized by the proliferation of white blood cells, with the manifestation of the abnormal cells in the peripheral blood. In 2006, leukemia was ranked 5th of all cancer patients in Indonesia. Chronic leukemia is the most common leukemia in adult and the elderly. In general, chronic leukemia classified on chronic myelocytic leukemia (CML) and chronic lymphocytic leukemia (CLL). The onset of chronic leukemia is insidious and accompanied by symptoms that are not typical, this research aims to describe the laboratory findings of chronic leukemia patients treated at Internal Medicine Department of Dr. M. Djamil Hospital Padang.This research is a retrospective descriptive research. The instruments used in this research are the secondary data derived from the Medical Record Departement Dr. M. Djamil Hospital Padang in the form of leukemia chronic patients’ data who were treated in Internal Medicine Department of Dr. M. Djamil Hospital Padang since January 1st 2010 – December 31st 2012. The results of this research showed that of 16 cases of chronic myelocytic leukemia contained 37.5% of the patients had moderate anemia, leukocytosis 100%, platelet count can be decreased, normal, and increased the percentage of each 25%, 25%, and 50%. The morphology of erythrocytes mostly normocytic anisocytosis. Half of peripheral blood examination showed an increase in the percentage of myelocyte, 31.25% showed an increase in the percentage metamyelocyte and eosinophils, as well as most of the shows presentation blast. The bone marrow are hypercellular, compressing erythropoietic, myelopoietic hyperactivity and thrombopoietic mostly normal in number. Chronic lymphocytic leukemia consisting of 1 case shows the laboratory findings are moderate anemia, leukocytosis, thrombocytopenia, the morphology of erythrocyte is normochromic anisocytosis, leukocytes increase in number, increase in the number of lymphocytes, presentations smudge cells, and blast presentation is found in the peripheral blood, but the cellularity not be assessed.Keywords: chronic leukemia, peripheral blood, BMP


Blood ◽  
1994 ◽  
Vol 83 (12) ◽  
pp. 3629-3637 ◽  
Author(s):  
JQ Guo ◽  
JY Lian ◽  
YM Xian ◽  
MS Lee ◽  
AB Deisseroth ◽  
...  

Chronic myelogenous leukemia (CML) is a myeloproliferative disorder associated with the Philadelphia chromosome (Ph1) in more than 95% of these patients. The Ph1 and the resulting BCR-ABL fused genes are markers for this type of leukemia. In CML, the product of the fused BCR- ABL gene is typically a protein of approximately 2,000 amino acids termed P210 BCR-ABL. We have developed an assay for the BCR-ABL protein involving Western blotting of circulating white blood cells (WBC) with an anti-ABL monoclonal antibody that can detect P210 BCR-ABL and P145 ABL in peripheral blood cells from chronic phase Ph1-positive leukemia patients. This assay was used to analyze the BCR-ABL protein content of circulating WBC from CML patients before and after various treatments. In parallel to changes in percentages of Ph1-positive blood cells as determined by cytogenetic analyses of bone marrow samples, BCR-ABL protein expression in blood cells decreased or increased as patients entered remission or underwent relapse. Of interest, six Ph1-negative CML patients were BCR-ABL protein-positive. All except one had a rearrangement in the major breakpoint cluster region and that patient expressed P185 BCR-ABL and not P210. Our results indicate that the BCR- ABL Western blotting assay has clinical applications for both diagnosis and prospective evaluation of Ph1-positive and Ph1-negative CML patients.


Blood ◽  
1994 ◽  
Vol 83 (12) ◽  
pp. 3629-3637 ◽  
Author(s):  
JQ Guo ◽  
JY Lian ◽  
YM Xian ◽  
MS Lee ◽  
AB Deisseroth ◽  
...  

Abstract Chronic myelogenous leukemia (CML) is a myeloproliferative disorder associated with the Philadelphia chromosome (Ph1) in more than 95% of these patients. The Ph1 and the resulting BCR-ABL fused genes are markers for this type of leukemia. In CML, the product of the fused BCR- ABL gene is typically a protein of approximately 2,000 amino acids termed P210 BCR-ABL. We have developed an assay for the BCR-ABL protein involving Western blotting of circulating white blood cells (WBC) with an anti-ABL monoclonal antibody that can detect P210 BCR-ABL and P145 ABL in peripheral blood cells from chronic phase Ph1-positive leukemia patients. This assay was used to analyze the BCR-ABL protein content of circulating WBC from CML patients before and after various treatments. In parallel to changes in percentages of Ph1-positive blood cells as determined by cytogenetic analyses of bone marrow samples, BCR-ABL protein expression in blood cells decreased or increased as patients entered remission or underwent relapse. Of interest, six Ph1-negative CML patients were BCR-ABL protein-positive. All except one had a rearrangement in the major breakpoint cluster region and that patient expressed P185 BCR-ABL and not P210. Our results indicate that the BCR- ABL Western blotting assay has clinical applications for both diagnosis and prospective evaluation of Ph1-positive and Ph1-negative CML patients.


Blood ◽  
1973 ◽  
Vol 42 (5) ◽  
pp. 799-804 ◽  
Author(s):  
Dorothy Warburton ◽  
Avrum Bluming

Abstract A small acrocentric chromosome, identical in appearance to the Philadelphia chromosome of chronic myelocytic leukemia, was identified in all karyotyped marrow cell metaphases prepared from a 74-yr-old male with refractory dysplastic anemia. The abnormal chromosome was shown by quinacrine fluorescent staining to be derived from a Y chromosome which has lost the characteristic brilliantly fluorescent material of its long arms. A normal Y chromosome was consistently observed in karyotypes of cultured peripheral blood lymphocytes from this patient.


1987 ◽  
Vol 58 (03) ◽  
pp. 936-942 ◽  
Author(s):  
Lindsey A Miles ◽  
Edward F Plow

SummaryGlu-plasminogen binds to platelets; the monocytoid line, U937, and the human fetal fibroblast line, GM1380 bind both plasminogen and its activator, urokinase. This study assesses the interaction of these fibrinolytic proteins with circulating human blood cells. Plasminogen bound minimally to red cells but bound saturably and reversibly to monocytes, granulocytes and lymphocytes with apparent Kd values of 0.9-1.4 μM. The interactions were of high capacity with 1.6 to 49 × 105 sites/cell and involved the lysine binding sites of plasminogen. Both T cells and non-rosetting lymphocytes and two B cell lines saturably bound plasminogen. Urokinase bound saturably to gianulocytes, monocytes, non-rosetting lymphocytes and a B cell line, but minimally to T cells, platelets and red cells. Therefore, plasminogen binding sites of high capacity, of similar affinities, and with common recognition specificities are expressed by many peripheral blood cells. Urokinase receptors are also widely distributed, but less so than plasminogen binding sites. The binding ol plasminogen and/ or urokinase to these cells may lead to generation of cell- associated proteolytic activity which contributes to a variety of cellular functions.


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