Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chromosome abnormalities were demonstrated in 50-100% of Giemsa-banded metaphases from nine cases of B-cell prolymphocytic leukemia (B-PLL). Mitoses were obtained with pokeweed mitogen following pretreatment of peripheral blood (PB) prolymphocytes with neuraminidase-galactose oxidase. Chromosome 14 was abnormal in eight of the nine cases: a marker 14q+, with breakpoint at band q32 in seven and trisomy 14 in one. In four cases the abnormal No. 14 was one of several primary abnormalities and in four others it was seen in secondary clones. The origin of the translocated material was unknown in three cases, in two it resulted from t(11;14), later becoming t(11;14;21) in one of them, t(1;14) in another, progressing later to t(1;14;17); in yet another patient, the 14q+ was the result of a complex rearrangement t(6;14;17). Abnormalities of chromosome 6 were seen in six cases: 6q- as the primary abnormality in three; trisomy 6 was part of secondary changes in one case. Structural abnormalities of chromosome 1 were seen in six cases: 1q- in four (in one as the only abnormality), 1q+ in one case, and 1p- in another, both in the main clone. Trisomy 12 was demonstrated in three cases but not as the primary change. Spleen cells in two patients showed a higher frequency of abnormalities than in the PB, supporting the concept of the spleen being the organ primarily involved in B-PLL. Evidence of karyotypic evolution was demonstrated in six patients, in some clearly associated with clinical progression of the disease. The type and frequency of the abnormalities observed in B-PLL resemble those seen in non-Hodgkin's lymphomas and suggest major differences from B-CLL, although a relationship with the latter can not be completely ruled out at present.
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PMID:Chromosome abnormalities in B-cell prolymphocytic leukemia: a study of nine cases. 660 59

Although translocations of the BCL2 gene are frequent in B-cell non-Hodgkin's lymphomas (B-NHL) the incidence, nature, and prognostic significance of similar translocations in the phenotypically related chronic leukemias of mature B cells are unknown. Therefore, we examined 170 cases of B-cell chronic lymphocytic leukemia (B-CLL), 7 cases of B-cell prolymphocytic leukemia (B-PLL), 25 cases of hairy cell leukemia (HCL) and 22 cases of splenic lymphoma with villous lymphocytes (SLVL) with defined cytogenetic abnormalities by DNA blot using both 5' and 3' BCL2 probes to search for rearrangement of the BCL2 locus. Translocation t(14;18) (q32.3;q21.3) was detected cytogenetically in 3 cases of B-CLL. All had breakpoints in the 3' region of BCL2, mapping between the major breakpoint region (MBR) and the minor cluster region (mcr), the breakpoint clusters commonly detected in B-NHL. In 2 of the 3 cases, the breakpoint within BCL2 was mapped to a 1.0-kb EcoRI-HindIII fragment indicating a clustering of breakpoints. Two cases of B-CLL had cytogenetically detectable t(2;18)(p11;q21.3) or t(18;22)(q21.3;q11). Both had rearranged the 5' region of the BCL2 gene to the corresponding lg light-chain gene. Molecular cloning of the t(18;22)(q21.3;q11) showed that the translocation disrupted the BCL2 promoter region and the first untranslated BCL2 exon. Nevertheless, high levels of BCL2 protein were seen in this case. Only 2 other cases in whom cytogenetic analysis was not successful showed rearrangement of the 5' region of BCL2, an overall incidence of 2.3%. No cases of B-PLL, HCL, or SLVL showed either 5' or 3' BCL2 rearrangement. These data confirm the cytogenetic observations that translocations involving the BCL2 locus in all forms of leukemia of mature B cells are rare, and limited to a minor subset of B-CLL. BCL2 translocations in B-CLL involve hot spots of recombination of both the 5' and 3' regions of the BCL2 gene, which are distinct from those commonly seen in B-NHL, suggesting distinct pathogenic mechanisms.
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PMID:BCL2 translocations in leukemias of mature B cells. 820 92

Between February, 1970 and September, 1991, we performed splenectomies on 70 patients with chronic lymphoproliferative disorders including primary leukemias: 19 B-cell chronic lymphocytic leukemia, 1 B-cell prolymphocytic leukemia, 22 hairy cell leukemias, 4 large granular lymphocytic leukemias, 1 T-cell prolymphocytic leukemia, and non-Hodgkin's lymphomas (NHL): 10 splenic lymphomas with villous lymphocytes, 4 follicular lymphomas, 5 mantle cell lymphomas, 3 lymphoplasmacytic and 1 large cell NHL. The primary indications for surgery in this series were therapy-resistant disease (40%) and therapeutic splenectomy (38%). Postsplenectomy, 70% of patients had a complete hematological response, 23% had a partial response, and 7% were nonresponsive. Median treatment-free survival correlated with the hematologic response postsplenectomy and the underlying diagnosis. Better treatment-free survivals were seen in patients with lesser degrees of anemia and thrombocytopenia. Overall, improvements were more pronounced in the B-cell than in the T-cell disorders. Indications for further therapy, postoperative morbidity and mortality, and survival times are discussed along with a review of the literature. These findings advocate a continuing role for splenectomy in symptomatic lymphoid malignancies running with splenomegaly and hypersplenism.
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PMID:Splenectomy in lymphoproliferative disorders: a report on 70 cases and review of the literature. 822 Jan 25

The evolution of combination chemotherapy regimens, combined with improvements in supportive care, has incrementally improved survival outcomes for patients with non-Hodgkin's lymphomas (NHL). Although 40-60% of younger patients with diffuse large cell lymphoma can now expect to be cured, significant numbers will either fail to achieve a remission or relapse after attaining a remission. In addition, certain histological subtypes are associated with particularly poor prognoses with combination chemotherapy alone (e.g. mantle cell lymphoma, B-cell prolymphocytic leukaemia). Relatively few of these patients can achieve long-term responses. Other NHL subtypes, whilst associated with more favourable prognoses in terms of overall survival, are rarely, if ever, cured (e.g. most low grade NHL including follicular lymphoma, chronic lymphocytic leukaemia and small lymphocytic lymphoma). For these reasons dose escalation and allogeneic transplantation have been investigated as potential ways of improving outcome, although this has mainly been in the setting of advanced disease. Any possible benefits have frequently been out-weighed by procedural morbidity and mortality. The parallel development of transplantation approaches that limit procedural toxicity along with advances in supportive care require that the role of allogeneic haematopoietic stem cell transplantation in the management of lymphoma be re-evaluated.
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PMID:The role of allogeneic transplantation in non-Hodgkin's lymphoma. 1563 49