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

We report a unique case of de novo composite lymphoma in the tibia of a 35-year-old man who presented with increasingly frequent and intense pain in the right upper leg. He was otherwise healthy without significant medical history. A plain radiograph of the right leg showed a permeative lesion with alternating areas of radiolucency and radiodensity in the upper third of the tibia. Magnetic resonance imaging showed a large, heterogeneous enhancing lesion involving the medullary and cortical bone of the proximal tibia with cortical disruption and extension into the adjacent soft tissue. A biopsy showed sheets and clusters of large cells, punctuated by clusters of small, irregular lymphocytes. Flow cytometry and immunohistochemical analysis showed composite lymphoma: diffuse large B-cell lymphoma (DLBCL) and peripheral T-cell non-Hodgkin lymphoma with predominantly small cell morphologic features. The DLBCL expressed CD19, CD20, CD79a, CD5, CD10, CD23, CD38, CD117, bcl-2, and bcl-6, with monotypic expression of immunoglobulin kappa light chain. The T cells expressed CD2, CD3, CD5, CD7, and CD8, with partial loss of CD4. Clonal rearrangement of T-cell receptor gamma chain gene was found. Neither the large B cells nor the small T cells expressed Epstein-Barr virus-encoded RNA. Physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other mass lesions in the body. No peripheral lymphocytosis or bone marrow involvement was present.
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PMID:Composite B-cell and T-cell non-Hodgkin lymphoma of the tibia. 1584 45

A 41-year-old Caucasian female was diagnosed with a CD3(+) CD4(+) CD8(+) variant of lymphoproliferative disorder of granular lymphocytes (LDGL) in the third year of remission following treatment of stage III-B Hodgkin lymphoma (HL). The patient was asymptomatic at diagnosis, without clinical evidence of immune disorder or recurrence of HL. Diagnosis was made incidentally, secondary to lymphocytosis discovered on a routine follow-up post HL therapy. Clonal chromosomal abnormalities were seen in 20% of peripheral blood lymphocytes with a karyotype 46, XX, t(2;6;2;11) (p13;q23;q24;q23). The breakpoint on 11q23 is distal to the MLL gene as shown by fluorescence in situ hybridization (FISH) analysis. To our knowledge, this is the first report of variant LDGL in association with HL treatment with documented clonal chromosomal abnormalities.
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PMID:Variant lymphoproliferative disorder of granular lymphocytes (LDGL) following Hodgkin lymphoma. 1592 4

Parameters of the erythroid, granulocytic, and megakaryocytic hemopoietic stems were compared in 87 patients with aggressive and indolent non-Hodgkin's lymphomas before and 6 months after the start cytostatic therapy. Before chemotherapy anemia was detected in 46% patients with aggressive and 49% patients with indolent lymphomas. Hemoglobin content, peripheral blood erythrocyte count, and total count of erythroid cells in the bone marrow increased during chemotherapy in the indolent lymphoma group. Increased count of erythroid cells in the myelogram was due to decreased count of lymphoid cells in the bone marrow, which was associated with complete or partial remission. In aggressive lymphoma chemotherapy decreased the mean level of hemoglobin and mean erythrocyte count in the peripheral blood, but the total count of erythroid cells in the bone marrow increased; no relationship was detected between lymphocyte count in the bone marrow and erythropoiesis characteristics. Lymphocytosis >50% in the myelogram before chemotherapy was less frequent in this group in comparison with indolent non-Hodgkin's lymphomas.
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PMID:Erythropoiesis in patients with aggressive and indolent non-Hodgkin's lymphomas. 1613 22

Parameters of hemogram and myelogram were studied in patients with aggressive and indolent non-Hodgkin's lymphomas: the relationships between the parameters recorded before treatment and during remission or progress 6 months after chemotherapy were studied by multifactorial analysis. The progress of indolent non-Hodgkin's lymphomas was associated with changes caused by tumor infiltration of the bone marrow; lymphocytosis in the myelogram or hemogram was associated with a relative decrease in the count of granulocytic hemopoietic stem cells. A sign associated with the absence of remission in aggressive non-Hodgkin's lymphomas was decreased level of hemoglobin and erythroid cells. Changes in myelogram attesting to anemia and suppressed erythropoiesis before chemotherapy are additional prognostic factors indicating obligatory intensification of chemotherapy for patients with aggressive non-Hodgkin's lymphomas.
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PMID:Hemogram and myelogram in progressing non-Hodgkin's lymphomas. 1625 28

Systemic mastocytosis is a disease characterized by multifocal mast cell proliferation in the bone marrow or other extracutaneous organs. Because of loosely scattered and hypo-/agranular mast cells, the diagnosis is sometimes very difficult. In the bone marrow, mast cell infiltration may be associated with prominent lymphoid infiltration leading to a misdiagnosis of a low grade non-Hodgkin lymphoma. A 49-year-old woman presented with right arm and leg pain, psychiatric symptoms, and diarrhea for four years. Physical examination and laboratory investigation revealed hepatosplenomegaly, anemia, mild thrombocytosis, mild leucocytosis and lymphocytosis. In the bone marrow biopsy, there was a prominent B lymphocyte proliferation reminiscent of a low grade non-Hodgkin lymphoma/leukemia and there were some spindle cells aggregates in paratrabecular location. The consecutive bone marrow biopsies were similar to the first. The subsequent splenectomy specimen exhibited striking fibrosis. In the lymph node sections, there was marginal zone hyperplasia. Multifocal accumulations of mast cells were strongly positive with mast cell tryptase and CD117 on immunohistochemical staining, though no metachromasia was identified in Giemsa and Toluidine Blue stained aspirates and tissue sections, probably due to hypo-/agranulation of mast cells. The case was presented to emphasize the importance of the antibody to mast cell tryptase in the diagnosis of mastocytosis and to discuss problems of differential diagnosis of systemic mastocytosis.
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PMID:Systemic mastocytosis presenting with a prominent B lymphocyte proliferation in the bone marrow and extensive fibrosis of the spleen. 1747 86

Splenic lymphoma with circulating villous lymphocytes (SLVL) is a special kind of lymphoproliferative disease characterized by specific clinical, haematological, histomorphological and immunophenotypic features that make it different from hairy cell leukaemia, prolymphocytic leukaemia and non-Hodgkin lymphoma. Four patients suffering from SLVL, 3 men and 1 woman, 62 years in average (range 55-67 years), are presented. All patients had anaemia and splenomegaly. One patient had huge pseudocyst of the spleen, two had mild lymph-adenopathy, while 3 patients had thrombocytopenia. The number of WBC ranged from 13-29 x 10(9)/l with lymphocytes ranging from 62-80%. Monoclonal IgM paraprotein was found in 3 patients. Immunophenotyping showed cells with features of mature B lymphocytes. Splenectomy was carried out in three patients. They all recovered and stayed symptomfree. One patient refused surgery. In our opinion SLVL is not a rare disease as it was thought; it should especially be taken into diagnostic consideration in elder patients, particularly in males with splenomegaly and lymphocytosis.
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PMID:[Splenic lymphoma with circulating villous lymphocytes]. 1797 22

The simultaneous occurrence of Hodgkin lymphoma with a variety of B-cell Non-Hodgkin lymphomas (composite lymphoma) has been described. We report the first case of composite Hodgkin lymphoma and splenic marginal zone lymphoma occurring simultaneously in the same lymph node of a 64-year-old man who presented with cervical and axillary lymphadenopathy and massive splenomegaly. He had a peripheral blood lymphocytosis and a bone marrow infiltrated by small lymphocytes. However, cervical lymph node biopsy showed classic Hodgkin lymphoma. His splenomegaly showed only a partial response to six cycles of ABVD chemotherapy so he underwent splenectomy with biopsy of remaining nodes. Histology of the spleen and nodes showed splenic marginal zone lymphoma. Review of the original biopsy confirmed the presence of both diseases in the original lymph node.
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PMID:Composite splenic marginal zone lymphoma and classic Hodgkin lymphoma -- an unusual combination. 1798 2

Families with multiple individuals affected with chronic lymphocytic leukaemia (CLL) and other related B-cell tumours have been described in the literature. Familial CLL does not appear to differ from sporadic CLL in terms of prognostic markers and clinical outcome. Large, population-based case-control and cohort studies have also shown significant familial aggregation of CLL and related conditions, including non-Hodgkin and Hodgkin lymphoma. Monoclonal B-cell lymphocytosis also aggregates in CLL families. However, the clinical implication of familial aggregation is minimal given the overall rarity of CLL. Linkage studies have been conducted in high-risk CLL families to screen the whole genome for loci that contribute to susceptibility but no gene mutations have yet been identified by this method. Association studies of candidate genes have implicated immune function and other genes but more studies are needed to verify these findings. The ability to conduct large scale genomic studies will play an important role in detecting susceptibility genes for CLL over the next few years and thereby help to delineate aetiological pathways.
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PMID:Family studies in chronic lymphocytic leukaemia and other lymphoproliferative tumours. 1802 Oct 91

Families with multiple individuals affected with chronic lymphocytic leukemia (CLL) and other related B-cell tumors have been described in the literature. Familial CLL does not appear to differ from sporadic CLL in terms of prognostic markers and clinical outcome. While some environmental factors (such as farming-related exposures and occupational chemicals) may increase risk of CLL, results of epidemiologic studies have been generally inconsistent. Rates of CLL in the population show significant international variation, with the highest rates in the U.S. and Europe and the lowest rates in Asia. Migrants from Asia to the U.S. also have low rates of CLL, which supports a greater role for genetic compared with environmental risk factors. Large, population-based case-control and cohort studies have also shown significant familial aggregation of CLL and related conditions including non-Hodgkin and Hodgkin lymphoma. Monoclonal B-cell lymphocytosis also aggregates in families with CLL. However, the clinical implication of familial aggregation is minimal given the overall rarity of CLL. Linkage studies have been conducted in high-risk CLL families to screen the whole genome for loci that contribute to susceptibility, but no gene mutations have yet been identified by this method. Association studies of candidate genes have implicated immune function and other genes, but more studies are needed to verify these findings. The ability to conduct large-scale genomic studies will play an important role in detecting susceptibility genes for CLL over the next few years and thereby help to delineate etiologic pathways.
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PMID:Familial CLL: genes and environment. 1802 49

Though B-chronic lymphocytic leukemia (CLL) is known to be a heterogeneous disease, only recently has the familial component of CLL been more thoroughly investigated. This entity is seen in approximately 5%-10% of all patients with CLL and can be associated with earlier age of diagnosis, higher female prevalence, and increased incidence of other lymphoproliferative disorders (LPDs), such as non-Hodgkin lymphoma and the more recently described monoclonal B-cell lymphocytosis CLL in family members. The prognostic parameters and clinical course of familial CLL is not clearly distinguishable from that of sporadic disease. In addition, it is not clear that the treatment responses for progressive disease has any discernible difference in familial versus sporadic CLL. The genetic etiology of CLL is unknown, and early work on familial CLL has not yet uncovered any obvious gene or group of genes that can be clearly related to the pathophysiology of CLL. However, the detailed genetic study of familial CLL is likely to be critical in uncovering relevant genes. At present it is best to indicate to concerned CLL patients that their relatives are at relatively low risk of developing CLL or other LPDs.
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PMID:Familial chronic lymphocytic leukemia: what does it mean to me? 1977 40


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