Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have investigated the incidence and significance of abdominal lymphadenopathy in hairy cell leukemia (HCL) by routinely CT scanning 88 patients. These included 70 men and 18 women with a median age of 51 years (range 25-83). Abdominal CT scans were performed at diagnosis in 29 patients and in 59 during the course of the disease. Abdominal lymphadenopathy was documented in 25 patients (28%) overall; the incidence of abdominal lymphadenopathy was higher in relapse (56%) than at diagnosis (17%). All patients with lymphadenopathy had bone marrow disease. There is no association with age or sex but lymphadenopathy tends to be more common in patients with long-standing disease (median duration of disease 6 years v one year in those without nodes) and in patients with bulky disease, particularly in the relapse group (splenomegaly/splenectomy in 95% vs 40%). The presence of abdominal nodes is also associated with relative resistance to treatment, with more treatment failures and fewer complete responses seen in this group. Most patients with lymphadenopathy had large, immature-looking hairy cells present in both the bone marrow and lymph nodes, when these were examined. Abdominal lymphadenopathy in HCL is more common than previously recognised, particularly in relapsed patients, and is always associated with active disease. The presence of large hairy cells and the relative resistance to treatment suggest that this phenomenon represents a form of transformation of the disease. Longer follow up is required to confirm this.
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PMID:Incidence and significance of abdominal lymphadenopathy in hairy cell leukaemia. 782 58

Splenic marginal zone (MRZ) cell lymphoma is a recently described neoplasm arising in a unique compartment of splenic white pulp, producing massive splenomegaly and spreading to bone marrow and distant lymph nodes. We report three cases of splenic lymphoma that morphologically and immunohistochemically appear to originate from MRZ cells that presented as indolent neoplasms involving the spleen but with no or only moderate enlargement of the organ, presumably representing an early clinical stage of this disorder. Despite the evidence of involvement of the liver in one case, lymph nodes and bone marrow proved to be uninvolved. Histologically, the three spleens showed similar features, being characterized by the involvement of white pulp follicles and periarteriolar lymphoid sheaths by medium-sized lymphoid cells with slightly irregular nuclei and ample cytoplasm. Immunohistochemically, all the specimens expressed a series of B-lineage markers that, in contrast to specimens of monocytoid B cell lymphoma (MBCL) and hairy cell leukemia (HCL) studied for comparison, did not react with KiB3, LN1, and DBA.44 monoclonal antibodies.
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PMID:Splenic marginal zone cell lymphoma: report of an indolent variant without massive splenomegaly presumably representing an early phase of the disease. 782 15

Hairy cell leukemia is a rare chronic B-lymphocyte malignancy characterized by pancytopenia, splenomegaly, immunological abnormalities and morphologically typical neoplastic mononuclear cells in blood and bone marrow. Until recently the disorder was treated with splenectomia and/or alpha-interferon. Intravenous infusion of 2-chloro-deoxy-adenosine (2-CdA) is a new and efficacious treatment principle making hairy cell leukemia a potentially curable disease. A Nordic cooperative study is currently examining the effects of 2-CdA given subcutaneously for seven days. 19 Norwegian patients are included so far, with encouraging results. The long-term effect of 2-CdA is not yet known. The high remission frequency, the short duration of the treatment and the few side effects make this drug a natural first choice in treating hairy cell leukemia.
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PMID:[Progress in the treatment of hairy cell leukemia. Subcutaneous administration of 2-chloro-deoxy-adenosine for 7 days]. 791 11

Hairy cell leukaemia is a rare chronic lymphoproliferative disease, characterized by splenomegaly, pancytopenia and recurrent infection. The characteristic 'hairy cells', present in the peripheral blood and bone marrow, are the hallmark of this leukaemia. The disease has a chronic, progressive course, and the majority of patients afflicted by it require therapy. The most common reason to initiate treatment is neutropenia with or without associated infectious complications, or the development of severe thrombocytopenia. Therapeutic options in hairy cell leukaemia include splenectomy, interferon administration, or the use of chemotherapeutic agents such as pentostatin (2'-deoxycoformycin) and 2-chlorodeoxyadenosine. Splenectomy is still indicated in the treatment of young patients with significant splenomegaly and only minimal bone marrow involvement. Interferon treatment induces remission in approximately 90% of patients with hairy cell leukaemia, but complete remission is obtained in only 5-10%. The development of antibodies against interferon was initially considered a major problem, but longer follow-up of patients who developed antibodies has shown that it is transient and does not have a significant impact on the overall response to treatment. Pentostatin induces complete remission in 60-70% of patients and partial remission in 20-40%. 2-Chlorodeoxyadenosine is a very promising drug in the treatment of this rare leukaemia, inducing long-lasting complete remission in approximately 80% of patients. While interferon does not cure the disease, it is possible that a subset of patients treated with pentostatin or 2-chlorodeoxyadenosine are cured. Longer follow-up of these patients will determine whether this is true.
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PMID:Hairy cell leukaemia. 791 37

Peripheral blood mononuclear cells (PBLs) from 14 patients with low grade non-Hodgkin's B-cell lymphomas with predominant splenomegaly were studied by means of scanning (SEM) and transmission electron microscopy (TEM). All patients had peripheral blood and bone marrow involvement, the absence of lymphoadenopathy, and, except in one case, immunophenotypic features of a malignant proliferation of mature spleen B-cells arising from outside the germinal center, but not consistent with CLL or HCL. Several distinctive cytological features were observed in PBLs of the different subgroups. The SEM surface features of PBLs in patients with intermediate differentiation lymphocytic lymphoma (IDL) (five cases), lymphoplasmacytoid immunocytoma (LP-IC) (two cases), and mixed small and large cells malignant lymphoma (one case) were characterized by the presence of numerous well-developed microvilli. Some distinctive TEM ultrastructural features were also seen in the different cases. In the two cases of splenic lymphoma with villous lymphocytes (SLVL), SEM revealed large and elongated surface microvilli generally arising from two or three poles of the cells. This surface morphology, confirmed by TEM analysis, may be pathognomonic of this disease. Four additional cases, tentatively classified as small lymphocytic lymphoma on the basis of immunophenotypic data, were extremely heterogeneous at both SEM and TEM analysis. The ultrastructural features revealed by SEM and TEM may be useful for the more precise characterization of this heterogeneous group of diseases, which is generally difficult to define even when immunophenotypic and molecular approaches are used.
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PMID:Scanning and transmission electron microscopy of clonal peripheral blood lymphocytes in low grade non-Hodgkin's B-cell lymphomas with predominant splenomegaly. 791 21

Monocytoid B cell lymphoma (MBCL) is a newly recognized low-grade lymphoma. The malignant cells of MBCL seems to be the counterpart of reactive monocytoid B cells. The MBCL cells show a resemblance to the cells of hairy cell leukemia (HCL). The cell of MBCL exhibits immunoglobulin light chain restriction and markers of terminal BG cell differentiation. On the other hand, the HCL lack expression of CD 25 and are tartrate resistant, acid phosphatase-negative. The MBCL has prediction for elderly females. At the time of diagnosis, it is usually present in stage I or II. It may be difficult to differentiate the clinical and morphological expression of MBCL from that of any other indolent lymphoproliferative diseases, especially HCL and small lymphocytic lymphoma. Unlike other low grade lymphomas in MBCL, however, splenomegaly and bone marrow involvement are uncommon, while leukemic conversion is very rare. The MBCL transformation or association to high grade lymphomas is very unusual. In this case, it isn't clear if MBCL undergoes progression to higher grade lymphomas or if there is a "composite lymphoma" at the onset, in any case the prognosis is worse. In our patient, the involvement seemed to be only at the spleen and caeliac nodes, there was not bone marrow involvement. The fast evolution suggests that the MBCL underwent a transformation to a higher grade lymphoma. It is a common behavior in the low grade lymphoma.
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PMID:[Monocytoid lymphoma evolving into large-cell lymphoma]. 817 84

An infectious episode is the presenting manifestation of hairy cell leukemia (HCL) in approximately 30% of cases. Most often this is bacterial and only rare cases of opportunistic fungal infection are described. We report a patient who presented with sporotrichal involvement of multiple cutaneous sites and lymph nodes. The lesions resolved following antifungal therapy, but persisting pancytopenia and splenomegaly necessitated further hematological evaluation. A diagnosis of HCL was suspected based on morphologically characteristic hairy cells in the peripheral blood that contained tartrate resistant acid phosphatase. A bone marrow biopsy specimen had a normocellular marrow with an increase in interstitial lymphoid cells that stained with L26, MB2, and LN2 antibodies. On flow cytometry these cells were positive for the leukocyte common antigen, B cell markers, and the CD11c antigen confirming the diagnosis of HCL. We believe that this is the first report of sporotrichosis infection as a presenting manifestation of HCL.
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PMID:Sporotrichosis as a presenting manifestation of hairy cell leukemia. 817 80

Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new entity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic studies to further characterize this lymphoma, which is frequently confused with hairy cell leukemia and other low-grade B-cell lymphoid neoplasms. The diagnostic criteria we used include 1) prominent splenomegaly, 2) insignificant or no lymphadenopathy, 3) lymphocytosis without leukopenia, 4) presence of circulating villous lymphocytes, 5) characteristic cytologic and histologic features, and 6) specific phenotypic and cytochemical findings. Our studies show that SLVL does not represent a pure entity but rather a morphologically heterogeneous group of low-grade lymphomas with various cytologic and histologic features. Although immunophenotyping is helpful in differential diagnosis, multiparameter studies are necessary to confirm the diagnosis. In our series, only two patients died of SLVL, who probably developed transformation to a higher-grade lymphoma.
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PMID:Splenic lymphoma with circulating villous lymphocytes: report of seven cases and review of the literature. 825 10

Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder that is characterized clinically by splenomegaly, pancytopenia, and the presence of malignant tartrate-resistant acid phosphatase (TRAP)-positive lymphocytes displaying unique morphological features in the peripheral blood and bone marrow. In the past, splenectomy has been the mainstay of treatment, resulting in symptomatic palliation and hematological improvement in 90 percent of patients. However, most patients undergoing splenectomy eventually require additional therapy; hematologic parameters and the degree of bone marrow cellularity are now utilized to identify to identify subsets of patients who might derive long-term benefit from the procedure. 2'-Deoxycoformycin (DCF) and 2-chlorodeoxyadenosine (2-cda) are experimental agents that have demonstrated curative potential in HCL; however, unresolved concerns about the long-term toxicity of DCF currently preclude its routine use, and further studies are required to define the toxicity and duration of responses obtained with 2-cda. As a result, alpha-interferon (alpha IFN) presently represents the treatment of choice for most patients with HCL. While alpha IFN has not been shown to cure HCL, its advantages include a high rate of response, a favorable side effect profile, its ability to be used safely in patients with active infections, and its ability to re-induce remission in relapsing patients. The mechanisms underlying alpha IFN's effectiveness seem to involve its immunomodulatory actions, which decrease the frequency of infectious complications in the early stages of therapy, and its ability to induce hairy cells to differentiate into more mature cells less responsive to proliferative signals.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Role of alpha-interferon in the treatment of hairy cell leukemia]. 830 2

We have reported the case of a woman aged 76 years suffering from pancytopenia, splenomegaly, relative lymphocytosis, lymphocytes with villous projection in the peripheral blood. On the basis of membrane phenotype, cytochemistry, ultrastructural examination of blood and marrow lymphocytes, differential diagnosis was between chronic lymphatic leukemia (CLL), CLL of mixed cell type, hairy cell leukemia (HCL), HCL variant, splenic lymphoma with circulating villous lymphocytes (SLVL), Non-Hodgkin lymphoma of mantle zone (NHL). The application of morphologic and immunological methods has reinforced the value of cytomorphology and has proved advantageous in increasing our understanding of the heterogeneity of B CLL itself. A new classification of chronic B leukemias has been proposed on the basis of clinical, cytomorphological, histological, cytochemical, immunological criteria: CLL; CLL of mixed cell type including cases with more than 10% and less than 55% prolymphocytes (CLL/P) and a less well defined form with pleomorphic lymphocytes (CLL/P) and a less well defined form with pleomorphic lymphocytes but less than 10% prolymphocytes; prolymphocytic leukemia (PLL); hairy cell leukemia (HCL); HCL variant; splenic lymphoma with circulating villous lymphocytes (SLVL); leukemic phase of NHL (follicular lymphoma, intermediate or mantle zone lymphoma, and others); lymphoplasmacytic lymphoma; plasma cell leukemia. Even if the application of cytomorphological and immunological criteria has increased our knowledge of the heterogeneity of chronic B leukemias, in some cases, as in the one reported, exact classification may be very difficult. However, on the basis of clinical and laboratory criteria this case can be classified as SLVL, notwithstanding some discrepancies of the immunophenotype.
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PMID:[Question of differential diagnosis in a case of chronic B-cell lymphopathy]. 833 25


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