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)

Hairy-cell leukemia (HCL) is an unusual malignant hematologic disorder involving splenomegaly, pancytopenia, and circulating mononuclear cells with prominent cytoplasmic projections. As in most forms of leukemia, the risk of pulmonary infection by normal pathogens and opportunistic invaders alike is high. HCL may be associated with granulomatous infections of the lung, especially mycobacterioses. Of the authors' series of 33 patients, 9 had a fungal or mycobacterial infection, including 5 atypical mycobacterial species. Five of the 6 patients with mycobacterioses and 1 of the 3 with fungal pneumonia survived the infection with appropriate therapy. Granulomatous infections, particularly mycobacterioses, should be seriously considered in the differential diagnosis of pulmonary parenchymal disease in a patient with HCl.
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PMID:Pneumonia in hairy-cell leukemia. 720 21

To study the effect of splenectomy in hairy cell leukemia, we initiated a retrospective multicenter analysis. A series of 391 patients, contributed by 22 centers (median: 15 cases per center, range 2--130), was analyzed. Since comparison of the data from these centers showed few significant differences, the data were pooled. Factors that proved to have prognostic value at the time of diagnosis was significantly longer in the splenectomy group (n = 225) than in the nonsplenectomy group (n = 166) (P less than 0.0001). When subgroups were analyzed, no difference in survival time was found between splenectomized and splenectomized patients having an age greater than 60 years, symptoms greater than 12 months, spleens less than 4 cm under costal margin, hemoglobin levels greater than 12 g/dl, neutrophils greater than 500/mm3, or platelets greater than 100,000/mm3. After the operation, the platelet count usually increased to acceptable values, whereas the neutrophil count increased less often. Patients with Hb greater than 11 g/dl, neutrophils greater than 1,000/mm3, and platelets greater than 100,000/mm3 after splenectomy, survived longer than patients with a less complete response to the operation (P less than 0.01). In patients with definite splenomegaly and pancytopenia, splenectomy seems to be beneficial. However, in patients without distinctly enlarged spleens, other therapeutical modalities should be considered.
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PMID:Splenectomy in hairy cell leukemia: a retrospective multicenter analysis. 722 99

A patient with primary splenic hairy cell leukemia is reported. This patient presented with massive splenomegaly and pancytopenia due to hypersplenism. Exploratory laparotomy failed to demonstrate any disease outside the spleen and splenic hilar lymph nodes. Splenectomy was the only form of treatment. During the following 21 years, no recurrent hairy cell leukemia has been found. This case allows speculation that hairy cell leukemia may originate in the spleen and that prolonged survival or cure of the disease after splenectomy alone may be due to removal of stem cells in the spleen.
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PMID:Primary splenic hairy cell leukemia--remission for 21 years following splenectomy. 731 43

A 59-year-old man presented with lymphocytosis with huge splenomegaly. The abnormal lymphocytes had a high nucleoplasm:cytoplasm ratio, a prominent nucleolus and hairy cytoplasmic projections. Immunophenotyping revealed B-cell leukemia with negative reactions to CD5 and CD25. Cytogenetic study showed 46,XY,der(5)t(5;6)(q35;p21), del(7)(p13)/46,idem,add(22)(q13). The patient did not respond to chlorambucil and a combination of cyclophosphamide, vincristine and prednisolone. Splenic irradiation induced partial remission. He developed progressive anemia and thrombocytopenia and died of Escherichia coli septicemia 33 months after the diagnosis of hairy cell leukemia variant.
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PMID:Hairy cell leukemia variant. 748 10

The Italian Registry for hairy cell leukemia (HCL) has recorded 725 patients with HCL diagnosed over 25 years. We analysed this large series of patients with the aim of providing an evaluation of changes in clinical presentation, impact of initial therapy and modifications in prognostic factors over the period of two decades. Over time, a progressive down-staging of the disease at the onset, along with a reduction of patients with severe anemia and marked splenomegaly, has been observed. A second malignancy was found in 3.7% of patients, mostly detected several years after the onset of HCL. A striking improvement of survival rates has been observed, from 58.9% survival at five years for patients diagnosed before 1985 to 87.5% at five years for patients diagnosed after 1985 (p < 0.0001). Before 1985 hemoglobin alone provided prognostic information, whereas after 1985, clinical stage and the number of leukocytes correlated better with patient outcome. Survivals at 5 and 10 years were 34.4% and 29.6% respectively for untreated patients, 58.8% and 44.1% for patients receiving chemotherapy, steroids or other drugs, 64.1% and 56.1% for splenectomized patients and 88.9% (at 5 years) for alpha interferon (IFN)-treated patients (p < 0.0001). Our findings suggest that IFN has improved the prognosis of HCL, and that it must be considered a good initial treatment for patients with HCL.
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PMID:Hairy cell leukemia: a clinical review based on 725 cases of the Italian Cooperative Group (ICGHCL). Italian Cooperative Group for Hairy Cell Leukemia. 751 10

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are differentiated B-cell leukemias with well-described clinical, morphologic, and immunologic characteristics. We encountered two patients with indolent chronic B-cell leukemia showing overlapping features of these malignancies. The patients had progressive splenomegaly, minimal lymphadenopathy, and abnormal lymphoid cells with abundant cytoplasm and villi, which were strongly positive for surface antigens CD22 and CD11c, features associated with HCL. However, blood counts showed lymphocytosis without neutropenia and monocytopenia, and the bone marrow biopsies demonstrated tightly aggregated nodules of lymphocytes. In addition, the lymphoid cells were dual positive for CD19 and CD5, displaying weak-to-moderately positive monoclonal surface immunoglobulin, findings strongly suggestive of CLL. One patient failed to respond to therapy with chlorambucil and prednisone. The second patient showed a partial response to treatment with 2-chlorodeoxyadenosine. We compare our patients with similar variants of differentiated B-cell leukemias reported in the literature, including disorders described as hairy cell variant (HCL-V) or splenic lymphoma with villous lymphocytes (SLVL).
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PMID:CD5+ chronic B-cell leukemia with features intermediate to chronic lymphocytic leukemia and hairy cell leukemia. 752 21

2-Chlorodeoxyadenosine (2-CdA) yields high complete remission (CR) rates in patients with hairy cell leukemia (HCL) Two approaches were used to detect minimal residual disease. We studied two B-lineage antibodies, L26 and MB2, and a T-lineage antibody, UCHL-1, in fixed marrow core biopsies from 34 patients with HCL before and after 2-CdA to detect minimal residual in the marrow. In addition, the splenic index was calculated before and after treatment to detect residual splenomegaly. Prior to therapy, hairy cells exhibited intense cytoplasmic membrane reactivity with L26 and strong intracytoplasmic reactivity with MB2. UCHL-1 did not react with hairy cells. Thirty-one patients were assessable 3 months after therapy. Five of 24 (21%) patients in CR by routine evaluation had residual HCL detected by immunostaining. Four of these 5 patients have been reevaluated at 1 year. One patient relapsed by routine evaluation, 2 remained positive by immunostaining alone, and 1 patient became negative by immunostaining. A total of 19 patients have been evaluated at 1 year and 17 remain in CR. Three of these 17 were positive by immunostaining, 2 of whom had been positive at 3 months and 1 additional patient who became positive by immunostaining at 1 year. Of 9 patients evaluated at 2 years, an additional 2 of 3 patients with minimal residual disease detected previously by immunostaining at 3 months relapsed by routine morphology and 1 had persistent positive immunostaining. Only 1 patient in remission by morphology and immunostaining has relapsed.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Assessment of complete remission after 2-chlorodeoxyadenosine for hairy cell leukemia: utility of marrow immunostaining and measurement of splenic index. 752 9

Hairy cell leukemia (HCL), a rare haematological disorder of B-cell origin, mainly presents with bone marrow infiltration, haematopoietic insufficiency, and splenomegaly. In some cases, osteolytic lesions can be observed. Many of these clinical features, especially haematopoietic insufficiency and osteolytic lesions are likely to be caused by soluble factors, such as cytokines. There is evidence that these factors are produced by the malignant hairy cells themselves, suggesting a paracrine pathway. The importance of autocrine as well as paracrine growth loops in growth regulation of HCL-cells is supported by a series of excellent studies, performed within the last few years. It could be clearly shown that cytokines are involved in this autocrine and paracrine regulatory process. The most important cytokines which should be mentioned in this respect are tumor necrosis factor alpha, (TNF alpha). Interleukin-2 (IL-2), Interleukin-4 (IL-4), Interleukin-6 (IL-6) and B-cell-growth factor (BCGF). The role of other factors such as viruses and oncogenes remains rather unclear. Nevertheless, recent data suggest that the c-fms, which encodes for the macrophage colony stimulating factor (M-CSF) may be involved in the pathophysiological control of HCL growth. In this review, we summarise the important data and studies performed recently which shed light on the complex network of autocrine and paracrine growth regulation of HCL.
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PMID:Autocrine and paracrine regulation of neoplastic cell growth in hairy cell leukemia. 754 30

The first case of patient affected both by gastric carcinoma and hairy cell leukemia (HCL) is reported. From a clinical standpoint, this 54-year old man presented with striking leukopenia without splenomegaly. From a morphological point of view, the infiltration by leukemic cells occurred in the hypoplasic areas of the bone marrow biopsy (BMB) without increase in reticulin fibers. From these observations the authors deduce that: 1) BMB in many cases is the only morphologic tool for diagnosis and prognosis of HCL; 2) probably the "hypoplasic" variant of HCL will become more frequent, because of the increasing indication to BMB in course of pancytopenia, even in absence of splenomegaly; 3) our case is probably related more to an early phase of the disease than to a distinct variant. In addition, we propose that the co-existence of an aggressive solid tumor and HCL could be related to the immunosuppressive action by hairy cells.
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PMID:Hairy cell leukemia without splenomegaly nor myelofibrosis in a patient with gastric adenocarcinoma: early phase of the disease or a variant? 756 77

In 1985, Cancer and Leukemia Group B initiated a multi-institutional study to define the role of interferon alpha in therapy of previously untreated active hairy cell leukemia (HCL). This is a long-term follow-up report of the study. Fifty-five evaluable patients were treated with recombinant interferon-2b 2 million units/m2 subcutaneously three times a week for 1 year. Treatment was well tolerated; toxicity mainly consisted of flu-like syndrome and pancytopenia, both of a transient nature. Seventy-three percent of patients had objective beneficial responses with 8.3 months median time to achieve at least a partial response (PR). After 1 year of therapy, the patients have been observed for a median of 5 years. There was a continual trend towards relapse throughout this period but 28% have remained in remission beyond 6 years. Forty-six patients (83%) are alive at 6 years. Among the 40 patients who achieved at least a PR, there were 28 with splenomegaly at the beginning of study: the spleen size was reduced in all with interferon alpha therapy and none required splenectomy. This study confirms the results of other investigators, and demonstrates that recombinant alpha interferon-2b is an effective agent for treatment of hairy cell leukemia.
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PMID:Recombinant alpha-2b-interferon in therapy of previously untreated hairy cell leukemia: long-term follow-up results of study by Cancer and Leukemia Group B. 763 Jan 81


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