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1.
Med Clin (Barc) ; 72(7): 265-71, 1979 Apr 10.
Article in Spanish | MEDLINE | ID: mdl-459593

ABSTRACT

The prognosis of patients with chronic lymphocytic leukemia is very difficult to evaluate. The classification system by stages, which at the present time is the most simple and useful method for the prognosis of this disease, recognizes five degrees of involvement: stage 0 (medullary and peripheral lymphocytosis); stage I (lymphocytosis + enlarged lymph nodes); stage II (lymphocyosis + hepato-and/or splenomegaly); stage III (lymphocytosis + anemia), and stage IV (lymphocytosis + thrombocytopenia). In the present report 95 controlled patients at the Farreras Valentí School of Hematology are analyzed using the classification by stages; there were 19 cases in stage 0; 16 in stage I; 30 in stage II; 21 in stage III, and 9 in stage IV. The mean survival rate in the global series was 63 months and the average 90.9 months. As in other series, it was observed that patients in stages 0 and I have a much better prognosis than those in stages III and IV. Stage II occupies an intermediate position in relation to prognosis, since the actuarial survival figure for this stage can practically be superimposed on that of the global series. The classification of chronic lymphocytic leukemia by stages permits the specification of the therapeutic indications for this condition; it also encourages the search for new modalities of treatment for stages with poor prognosis, since in these cases chronic lymphocytic leukemia behaves in the manner of an acute or subacute hemopathy.


Subject(s)
Leukemia, Lymphoid/mortality , Adult , Aged , Female , Humans , Leukemia, Lymphoid/diagnosis , Male , Middle Aged , Prognosis
2.
Nouv Rev Fr Hematol (1978) ; 21(3): 251-6, 1979.
Article in French | MEDLINE | ID: mdl-296376

ABSTRACT

The trephine bone marrow biopsies of 51 patients with myeloproliferative syndromes were revised searching for lymphoid follicles and lymphoplasmocytosis: 18 of these had idiopathic myelofibrosis and 33 chronic myelogenous leukaemia. Six of the 18 biopsies on patients with myelofibrosis showed lymphoid follicles but only one of the 33 with chronic meylogenous leukaemia did (P = 0.01, Fisher exact test). In addition, four of the six myelofibrosis having follicles had two or more of them. When the pathological pattern of myelofibrosis was considered according to the Lennert and al. classification we found significantly more follicles in the cellular phase of the disease than in the advanced phases (P = 00.4, Fisher exact test). These findings can be considered as a morphological argument supporting the idea of an immunological mechanism in the development of myelofibrosis.


Subject(s)
Bone Marrow/pathology , Leukemia, Myeloid/pathology , Lymphoid Tissue/pathology , Primary Myelofibrosis/pathology , Biopsy , Humans , Primary Myelofibrosis/etiology , Primary Myelofibrosis/immunology , Retrospective Studies
15.
Hum Genet ; 34(3): 285-92, 1976 Dec 15.
Article in English | MEDLINE | ID: mdl-187542

ABSTRACT

We report a hereditary hemolytic anemia associated with a severe erythrocyte pyrimidine 5'-nucleotidase deficiency in a Spanish family of five members in which the parents are first cousins. Both parents exhibited decreased nucleotidase activity without clinical or hematologic abnormalities. Two children (a male and a female) showed severe pyrimidine 5'-nucleotidase deficiency with hemolytic anemia. The remaining child (a male) showed no signs of the disease. The findings strongly suggest an autosomal recessive mode of inheritance in this enzymopathy. This seems to be the first report of pyrimidine 5'-nucleotidase deficiency in Spain.


Subject(s)
Anemia, Hemolytic/enzymology , Nucleotidases/deficiency , Adenosine Triphosphate/blood , Adolescent , Anemia, Hemolytic/genetics , Erythrocytes/enzymology , Female , Genes, Recessive , Glutathione/blood , Humans , Male , Pedigree , Pyrimidine Nucleotides , Ribose-Phosphate Pyrophosphokinase/deficiency , Spain , Spectrophotometry, Ultraviolet
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