RESUMO
A case of typical Ph-positive chronic myelogenous leukemia developed in a 58-year old female with a 7-year history of chronic lymphoproliferative disease is described. With the second disease progression, the features of lymphoid proliferation disappeared almost completely. Possible causes of this rare combination of two diseases are discussed.
Assuntos
Leucemia Mielogênica Crônica BCR-ABL Positiva/etiologia , Linfoma/complicações , Doença Crônica , Feminino , Humanos , Pessoa de Meia-Idade , Prognóstico , Fatores de TempoAssuntos
Anemia/induzido quimicamente , Buformina/efeitos adversos , Complicações do Diabetes , Deficiência de Ácido Fólico/induzido quimicamente , Deficiência de Vitamina B 12/induzido quimicamente , Anemia/diagnóstico , Doença Crônica , Diabetes Mellitus/sangue , Diabetes Mellitus/tratamento farmacológico , Deficiência de Ácido Fólico/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Deficiência de Vitamina B 12/diagnósticoRESUMO
A total of 5 patients with a clinicohematological picture resembling hairy-cell leukemia (HCL) have been described. However, the morphological features of leukemic lymphocytes, the absence of acid phosphatase in them, the nodular character of the bone marrow lesion combined with an unusual phenotype have permitted the authors to distinguish these cases as a separate variant of B-cell malignant lymphoma. Paraprotein (M-class) was detected in the blood of two of the patients. A conclusion has been made that the combination of splenomegaly with hairy lymphocytes in the blood is characteristic of not only HCL, but it can be also observed in different variants of malignant lymphoma.
Assuntos
Leucemia de Células Pilosas/diagnóstico , Linfócitos/patologia , Linfoma/diagnóstico , Esplenomegalia/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Leucemia de Células Pilosas/sangue , Linfoma/sangue , Linfoma/classificação , Linfoma/complicações , Masculino , Pessoa de Meia-Idade , Esplenomegalia/sangue , Esplenomegalia/complicaçõesRESUMO
A patient has been described with an unusual variant of B-cell lymphoid leukemia. Specific morphology of leukemic cells (the presence of nuclear cleavage), initial and principal localization of the process in the bone marrow, as well as an original phenotype of malignant cells (CD5+, CD35+, CD37+ and CD38+) have given the grounds for the designation of this rare variant of leukemia as centrocytic.
Assuntos
Linfócitos B/patologia , Medula Óssea/patologia , Leucemia Linfocítica Crônica de Células B/diagnóstico , Terminologia como Assunto , Idoso , Humanos , Leucemia Linfocítica Crônica de Células B/sangue , Leucemia Linfocítica Crônica de Células B/classificação , MasculinoRESUMO
The data from literature on chromosome abnormalities in various subgroups of the myelodysplastic syndrome (according to FAB-classification) are presented. Special attention is paid to information about changes in the number and structure of chromosomes during the disease and their prognostic significance. The relation of definite chromosome aberrations to the clinical course and prognosis of the disease, in particular to its transformation into acute leukosis is under discussion.
Assuntos
Aberrações Cromossômicas/genética , Síndromes Mielodisplásicas/genética , Deleção Cromossômica , Transtornos Cromossômicos , Humanos , Cariotipagem , Prognóstico , Transformação GenéticaRESUMO
The authors have presented the current data on so-called Ph'-negative chronic myeloleukemia (CML). A detailed clinico-hematologic analysis has proved that most of patients with unchanged chromosome 22 have other myeloproliferative disease, most often it is chronic myelomonocytic leukemia. In some CML patients Ph'-chromosome is masked as a result of translocations, in other patients, although chromosome 22 is unchanged, the molecular-genetic marker of CML--chimera bcr/c-abl-gene, is detected on chromosome 22 or some other chromosome. It has been noted that only in rare cases of typical CML characteristic cytogenetic and molecular-genetic changes are absent. Further investigations should be conducted in the group of patients studied.
Assuntos
Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/genética , Cromossomos Humanos Par 22 , Cromossomos Humanos Par 9 , Doença Crônica , Humanos , Cariotipagem , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/sangue , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/diagnóstico , Cromossomo Filadélfia , Translocação GenéticaRESUMO
The bone marrow histological picture was studied in 13 patients with hairy-cell leukemia (HCL) who were followed up at the Hematological Centre in the Latvian SSR. The diagnosis was made or proved in all the cases, among them in 9 patients - on the basis of the proliferation type of leukemia cells in the bone marrow trephine biopsy that is characteristic of HCL. The analysis of the clinical and morphological features of the disease has permitted the authors to distinguish specific types of HCL: 1) splenic and bone-marrow variants predominantly were diagnosed clinically, 2) typical and atypical variants resembling malignant lymphoma or chronic lymphoid leukemia were diagnosed histomorphologically. The authors have recommended trephine biopsy+ for a wider use (as a more informative method as compared to sternal punction) in the diagnosis and differential diagnosis of HCL.
Assuntos
Medula Óssea/patologia , Células-Tronco Hematopoéticas/patologia , Leucemia de Células Pilosas/patologia , Adulto , Idoso , Contagem de Células Sanguíneas , Divisão Celular/fisiologia , Feminino , Humanos , Leucemia de Células Pilosas/sangue , Masculino , Pessoa de Meia-Idade , Pancitopenia/sangue , Pancitopenia/etiologia , Pancitopenia/patologiaAssuntos
Leucemia Mieloide Aguda/complicações , Neutrófilos/patologia , Anomalia de Pelger-Huët/diagnóstico , Aplasia Pura de Série Vermelha/complicações , Diferenciação Celular , Diagnóstico Diferencial , Humanos , Leucemia Mieloide Aguda/sangue , Masculino , Pessoa de Meia-Idade , Anomalia de Pelger-Huët/sangue , Aplasia Pura de Série Vermelha/sangueRESUMO
Splenectomy still remains the method of choice in treatment of patients with hairy cell leukemia (HCL) due to high efficiency and low percentage of complications. Five-year survival of patients with HCL after splenectomy was 74%. Because of the low percent of hairy cells in the patient's blood the diagnosis of HCL should widely include trephine biopsy of the bone marrow as well as immunological studies of the leukemic cells. In order to detect pathological processes in the spleen and estimate the topographo-anatomical alterations in the left quadrant of the abdomen before operation, it is expedient to use computed tomography, ultrasound examination, scintigraphy.
Assuntos
Leucemia de Células Pilosas/cirurgia , Esplenectomia , Adulto , Idoso , Medula Óssea/patologia , Feminino , Humanos , Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/mortalidade , Masculino , Pessoa de Meia-Idade , Cuidados Pré-Operatórios , Baço/patologiaRESUMO
Analysis of the disease course in 17 patients afflicted with hairy-cell leukemia enabled the authors to specify 3 clinico-hematologic variants of the disease: a typical or a splenic one (with a predominant injury to the spleen), an atypical or bone marrow one (with a predominant injury to bone marrow), and a hypoplastic one. The bone marrow variant was characterized by the most pronounced leukemic injuries to bone marrow, severe bacterial complications, and the most unfavourable prognosis. The remaining variants were marked by their own individual features and ran a more favourable course. The division of hairy-cell leukemia into 3 variants promotes the improvement of the disease diagnosis and the elaboration of the optimal treatment policy in each specific case as well as the disease prognosis. The pathogenetic heterogeneity of the hairy-cell leukemia variants is discussed.
Assuntos
Leucemia de Células Pilosas/diagnóstico , Adulto , Idoso , Biópsia , Medula Óssea/patologia , Doenças da Medula Óssea/sangue , Doenças da Medula Óssea/diagnóstico , Doenças da Medula Óssea/patologia , Diagnóstico Diferencial , Feminino , Humanos , Leucemia de Células Pilosas/sangue , Leucemia de Células Pilosas/patologia , Masculino , Pessoa de Meia-Idade , Esplenomegalia/sangue , Esplenomegalia/diagnóstico , Esplenomegalia/patologiaAssuntos
Síndromes Mielodisplásicas/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Refratária/sangue , Anemia Refratária/diagnóstico , Anemia Refratária/mortalidade , Anemia Refratária com Excesso de Blastos/sangue , Anemia Refratária com Excesso de Blastos/diagnóstico , Anemia Refratária com Excesso de Blastos/mortalidade , Medula Óssea/patologia , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/sangue , Síndromes Mielodisplásicas/mortalidade , PrognósticoAssuntos
Leucemia Eritroblástica Aguda/etiologia , Leucemia Monocítica Aguda/etiologia , Síndromes Mielodisplásicas/complicações , Adulto , Anemia Refratária/complicações , Anemia Refratária/diagnóstico , Humanos , Leucemia Eritroblástica Aguda/diagnóstico , Leucemia Monocítica Aguda/diagnóstico , Masculino , Síndromes Mielodisplásicas/diagnóstico , Remissão Espontânea , Fatores de TempoRESUMO
Improved cytogenetic techniques allow for the identification of subtle chromosomal rearrangements associated with particular subgroups of monocytic leukemias. A detailed assessment of both quantitative and structural chromosomal defects is presented and clinical and prognostic value of the karyotypic pattern is discussed.