Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 20-year old patient is presented with generalized lymphadenopathy, splenomegaly, hyperleukocytosis and a bone marrow biopsy showing panmyelosis with predominance of immature granulocytes. Lymph node biopsy showed a histopathological feature that was diagnosed as a chronic granulocytic leukemia in blast crisis. The cell surface phenotype of these blast cells showed predominance of immature CD1+, CD7+ T lymphocytes. The T cell lineage was confirmed by DNA rearrangement studies. In addition, the patient showed erythrocytosis, arterial O2 saturation of 92% and thrombocytosis, characteristics of polycythemia vera. After chemotherapy, the patient relapsed with similar symptoms and lymph node cells of similar immature T phenotype. With a revised diagnosis of immature T cell lymphoma associated to a myeloproliferative disorder and polyglobulia, the patient received a combined treatment of Cyclophosphamide-Adriamycin-Vincristine-VM26-Prednisone. Two months later, the patient relapsed again. He received the first phase of induction of the BFM protocol, with partial clinical remission. Five months later, the patient returned with fever, polyadenopathy and splenomegaly. Lymph node cells showed again immature T cell phenotype. The patient was next treated with the m-BACOD scheme, with no response and progression of the disease and he died few days later due to massive bleeding and cardiorespiratory failure.
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PMID:[T lymphoma of immature phenotype associated with polycythemia vera]. 182 May 2

Five patients with lymphoid blastic transformation of chronic myeloid leukemia have been treated with IL2 associated with Vincristine (VCR) plus Prednisone (PDN). Our study indicates that IL2 may be employed in the management of this disease without excessive toxicity at the higher doses in hospitalized patients and at the lower doses as outpatients. Concerning the therapeutic efficacy, our preliminary results indicate that IL2 might be useful in enhancing the chemosensitivity of the leukemic blasts. It remains to be seen if this will result in a rapid return to the CP and in a prolongation of survival.
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PMID:IL-2 in the treatment of chronic myeloid leukemia after lymphoid blast crisis: a pilot study. 209 90

The blastic cell phenotypes of 26 cases of CGL in blastic phase were estimated and the patients were treated with different schemes. The following methods of typisation of the blast cells were used: cytochemical stainings (POX, Sudan B, PAS, nonspecific esterase), estimation of TdT activity, and in 11 patients the testing with monoclonal antibodies of VI series. Using these methods 10 patients (38%) with lymphoid form of the blastic phase, 11 (43%) with the myeloid type and 5 patients (19%) with undifferentiated type were diagnosed. In the group of lymphoblastic type a longer survival time and complete remissions were observed. High TdT activity in blastic cells did correspond with favourable response to Vincristin and Prednison. The introduction of TdT assessment into the diagnosis of CGL allows the cells to be classified more precisely, thus helping in defining the prognosis and in the choice of treatment programme.
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PMID:Diagnostic and prognostic value of the terminal deoxynucleotidyl transferase (TdT) activity in treating the blastic phase (bp) of chronic granulocytic leukaemia (CGL). 241 15

Between 1969 and 1983, 51 (35 men and 16 women) new cases of acute lymphoblastic leukemia (ALL) were diagnosed in patients aged 15 to 85 years (mean 21 yrs.). All patients received a "total therapy" which included: 1st induction (PRD, VCR, DBR, and/or L-ASN); 2nd, Central Nervous System profilaxis (craneal TCT and/or intrathecal MTx); 3rd, maintenance (6MP and MTx) and 4th, reinductions every 3 months (PRD, VCR, and DRB). This treatment lasted for at least 3 years. Complete Remission (CR) was achieved in 45 patients (88.2%): 3 of these patients were referred to other centers to continue treatment, 1 patient developed an early "metamorphosis" to hemophagocytic hystiocytosis and another patient developed a late chronic granulocytic leukemia (Ph +) dying a few months later after an acute myeloblastic worsening. During treatment 16 patients relapsed (9 in bone marrow and 7 in Central Nervous System). Treatment was discontinued in 24 patients with complete remission of which 5 relapsed in bone marrow 17 to 61 months after treatment). In one of the latter (ALL Ph +) an allogenic bone marrow transplant was performed and CR was achieved and maintained 46 months later. The post diagnosis acutarial curve of the 51 patients gave a mean survival of 6 years with a plateau at 43% of the patients after 11 years. The duration of the first uninterrupted CR was of 6.5 years and a plateau was reached at 46% of the patients after 10.5 years. At the present time, 20 patients are in CR (46 to 129 months) without treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Possibilities of remission of acute lymphoblastic leukemia in adults: retrospective study of 51 patients subjected to a "total therapy" protocol in the period 1969-1983]. 277 23

PMN elastase is a useful additional parameter in the differential diagnosis of the leukaemias. In all patients with myelocytic leukaemias there were elevated levels of elastase-alpha 1-proteinase inhibitor (E-alpha 1PI), while in the lymphatic leukaemias complexed elastase levels were decreased. The highest values were found in the peripheral blood plasma and bone marrow plasma of patients with CML. Despite high E-alpha 1PI concentrations there were no signs of bleeding or consumption of plasmatic coagulation factors. In AML a wide range of E-alpha 1PI levels was observed, extending from slightly elevated to four hundred-fold increased. In myeloblastic leukaemias without maturation (FAB M 1) the concentrations of complexed elastase remained below 150 ng/ml. In myeloblastic leukaemias with maturation (FAB M2) the E-alpha 1PI values ranged between 214 ng/ml and 850 ng/ml (means = 402 +/- 69), and in myelo-monoblastic leukaemias (FAB M4) between 450 ng/ml and 720 ng/ml (means = 663 +/- 72). The only case of promyelocytic leukaemia (FAB M 3) exhibited an extremely high value of 4,550 ng/ml, while a monocytic leukaemia (FAB M5) showed an extremely low value of 5 ng/ml. During cytostatic therapy there was a rapid decrease in levels of complexed elastase, with E-alpha 1PI values returning to normal in remission. In recidivating cases there was an increase of E-alpha 1PI levels in AML and a decrease in ALL. There was a correlation between the E-alpha 1PI concentrations in peripheral plasma and leukaemic bone marrow infiltration, so providing a good basis for monitoring remission from leukaemia and indicating relapse. It was also interesting to observe an extremely low E-alpha 1PI level (5 ng/ml) in patients with myelodysplasia. Under Decortin/Plenastril therapy the concentration rose to 50 ng/ml. An E-alpha 1PI level of 10 ng per ml was observed in one case of Ranitidine agranulocytosis. Under corticoid therapy the value returned to normal within eight days.
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PMID:The importance of granulocyte elastase in haematological diagnosis. 316 79