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)

Seven spleens and two peripheral blood specimens from eight patients with hairy cell leukemia were examined with enzyme cytochemical and histochemical methods. Hairy cells consistently exhibited acid phosphatase and tartrate-resistant acid phosphatase. However, nonspecific esterases characteristic of monocytes and histiocytes were consistently absent or very weak. beta-glucuronidase and cytoplasmic membrane-bound ATPase were positive in four cases, suggesting a possible relationship to the B-lymphocytic series. Fundamental splenic changes were accumulation of hairy cells and benign macrophages within the pulp cords, with resulting extreme expansion of the cords. Abnormally well developed ellipsoids were identified around the sheathed arteries within the cords. Sinuses, specifically delineated with the NASDA reaction, were atrophic and often destroyed. No cytogeneologic relationship was found between sinus endothelial cells and hairy cells. The pulp cords are the primary site of involvement of the spleen in hairy cell leukemia. A simultaneous proliferation of neoplastic cells, histiocytes and reticulum fibers accounts for the splenomegaly and clinical hypersplenism characteristic of the disease.
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PMID:Hairy cell leukemia. Enzyme histochemical characterization, with special reference to splenic stromal changes. 87 31

On account of 2 own observations, main clinical and diagnostic features of Hairy cell leukemia (HCL) will be discussed. HCL is a rare, unusual type of chronic leukemia and is predominantly particular of middle-aged men. The occurrence of middle-sized lymphoid cells having a hairy cytoplasmic edge, and a tartrate-resistant acid PHOsphatase isoenzyme are the characteristic criteria of the HCL. The diffuse infiltration by hairy cells affecting primarily the spleen and bone marrow results in anaemia, granulocytopenia, thrombocytopenia and splenomegaly. Differential diagnosis have to be made in relation to other lymphatic leukemias, leukemic malignant lymphomas and monoclonal gammopathies as well as lymphotropic viral infections. Immunologic behaviour of hairy cells is like that of B-lymphocytes. Therefore, the term "leukemic reticuloendotheliosis" should no longer be applied.
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PMID:[Diagnosis and differential diagnosis of hairy cell leukemia]. 107 83

Seven surgically removed spleens from patients with hairy cell leukemia and hypersplenism were examined ultrastructurally. In all spleens the pulp cords were diffusely and compactly infiltrated by hairy cells. Numerous hairy cells were also evident in the often distended sinuses. The hairy projections were readily visible in electron micrographs and tended to interdigitate to form syncytium-like aggregates. Compression of hairy cells within the cords flattened the projections against the cell bodies and may account for the surface alterations reported by scanning electron microscopic studies. Controversy over the cytogenesis of hairy cells has not been resolved by ultrastructural studies. Although all seven patients had hypersplenism, the hairy cells showed no evidence of phagocytic activity. However, active phagocytosis by cordal macrophages was observed and there is a probable absolute increase in their number contributing to the splenomegaly. The dense infiltrate of hairy cells causes marked widening of the cords and retards the passage of formed elements of the blood through the red pulp. Prolonged sojourn of these elements in a metabolically unfavorable environment results in cellular damage, increased exposure to cordal macrophages, and premature destruction with the evolution of a hypersplenic syndrome.
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PMID:Hairy cell leukemia (leukemic reticuloendotheliosis) II. Ultrastructure of the spleen. 126 Jul 14

Eleven patients with hairy cell leukemia (HCL) were treated with YK-176 (2'-deoxycoformycin) at a dose of 5 mg/m2 by intravenous injection every week or every other week. Patients received a median of eight (range 4-19) injections of YK-176. Five patients had previously been untreated, four of whom had massive splenomegaly. Six patients had previously been treated, four with interferon-alpha (IFN-alpha) or IFN-alpha and chemotherapy and two with prednisolone. Two patients had had splenectomies. Five patients achieved complete remission (CR) and six, partial remission (PR) according to WHO criteria (remission rate 100%, 95% confidence interval (CI) 74-100%). All six neutropenic patients recovered > 1,500/microliters neutrophils, six of seven anemic patients recovered > 12.0 g/dl hemoglobin and five of nine thrombocytopenic patients recovered > 100,000/microliters platelets following the treatment. According to the response criteria for HLC, five patients achieved CR, two PR and four minor response. The overall remission (CR + PR) rate was 64% (95% CI 35-85%). The CR and PR have lasted from > 30 to > 718 days (median, > 281 days) so far with no relapses. Of four patients previously treated with IFN-alpha, two achieved CR and one, PR. All patients were alive with a median survival time of > 290 days from treatment (range > 50- > 763 days). The treatment was generally well tolerated. Mild to moderate nausea, vomiting, appetite loss and general fatigue were experienced in two patients, skin rash in one and a transient fever in three. YK-176 was a highly active agent in the treatment of HCL.
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PMID:Treatment of hairy cell leukemia with deoxycoformycin (YK-176). The Deoxycoformycin (YK-176) Study Group. 129 57

A female patient simultaneously developed hematologic evidence of hairy cell leukemia and marked but short-lived inflammatory involvement of a number of joints. Both these groups of symptoms resolved simultaneously and rapidly under alpha-2 interferon therapy. This course suggests that the arthritis was a rheumatologic manifestation of the hematologic disease. The concomitant occurrence in this patient of arthritis, splenomegaly and leukopenia was suggestive of Felty syndrome: these two conditions need to be differentiated.
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PMID:[Polyarthritis revealing hairy cell leukemia]. 130 95

Twenty-six patients with hairy cell leukemia (HCL) were treated with 2-chlorodeoxyadenosine (2-CdA), a purine analogue resistant to adenosine deaminase, at 0.1 mg/kg/d for 7 days by continuous intravenous infusion. Fifteen patients were previously untreated, while 11 patients had received prior treatment with splenectomy alone (three patients), interferon alpha alone (four), splenectomy, then interferon alpha (two), or splenectomy, interferon alpha, then 2-deoxycoformycin (2-DCF) (two). Sixteen (80%) of 20 patients evaluable at 3 months achieved complete remission (CR), and four (20%) achieved partial remission (PR) following a single cycle of therapy. All four patients in PR had complete recovery of their peripheral blood counts (except one patient whose platelet count remained 84,000/microL), but had residual HCL in the bone marrow (three patients) or residual splenomegaly (one). Patients with bulky adenopathy, massive splenomegaly, and severe pancytopenia responded as well as those with only modest marrow involvement. The three patients with residual marrow disease received a second cycle of 2-CdA, and two have attained CR. Therefore, 18 of 20 (90%) achieved CR with either one or two cycles of therapy. No patient achieving CR has relapsed at a median follow-up of 12 (+/- 2.1) months. Toxicities included myelosuppression and culture-negative fever. A community-acquired pneumonia was the only infectious complication. Since a single cycle of 2-CdA induces sustained CR in the vast majority of patients with minimal toxicity, this agent is emerging as the treatment of choice for all patients with HCL.
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PMID:A single cycle of 2-chlorodeoxyadenosine results in complete remission in the majority of patients with hairy cell leukemia. 135 62

A 45-year-old male was hospitalized on September 2, 1989 with chief complaints of general fatigue and fever. Physical examination revealed hepatomegaly and massive splenomegaly. Laboratory tests on admission showed Hb of 7.5g/dl, PLT 4.8 x 10(4)/microliters and WBC 9,610/microliters with 81% hairy cells. Bone marrow aspirate demonstrated 55.1% hairy cells and moderate myelofibrosis. Cytochemically, hairy cells were positive for tartrate-resistant acid phosphatase (TRAP). Surface markers were SmIg G+ A+ kappa +, CD11b+, CD11c+, CD19+, CD20+, CD21-, CD25+, HC2+, HLA-DR+. From these findings, a diagnosis of hairy cell leukemia (HCL) was made. After administration of deoxycoformycin (DCF) at a dose of 5.0mg/m2 1-2 times monthly, splenomegaly disappeared, as did hairy cells from the peripheral blood. Hematological level returned to within normal range except for the presence of 1.2% hairy cells and mild myelofibrosis in bone marrow aspirates. DCF has so far been effective for this patient. While DCF has been reported to be effective in the treatment of HCL in the West, it has not been determined in Japanese patients with HCL, who have different hematologic features from those of HCL patients in the West.
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PMID:[Hairy cell leukemia successfully treated with deoxycoformycin]. 146 84

A 61-year-old man was admitted to our hospital in April 18, 1988, with dyspnea and gingival bleeding. Physical examination revealed marked splenomegaly, and peripheral blood showed severe pancytopenia with 38% abnormal mononuclear cells. The abnormal cells were characterized by a hairy appearance under a phase contrast microscopy, and strong tartrate-resistant acid phosphatase activity. These cells reacted with CD19, CD25 and CD11c monoclonal antibodies by the immunostaining method. Bone marrow aspiration failed and bone marrow biopsy revealed diffuse proliferation of hairy cells (HC) with moderate fibrosis. In addition, the staining pattern of HC peroxidase is similar to that found in megakaryocyte series. He was diagnosed as HCL of the European-American type based on these findings. Interferon (IFN)-alpha was administered at a daily dosage of 3 x 10(6) IU by intramuscular injection. Although splenomegaly and hematological conditions improved gradually, he received splenectomy because of his incomplete hematological improvement. Normalization of peripheral blood cell counts and a marked decrease of HC in bone marrow were obtained. Tubuloreticular structure and tubular confronting cisternae were seen in peripheral mononuclear cells during IFN therapy.
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PMID:[Improvement with interferon-alpha therapy and splenectomy in hairy cell leukemia of European-American type]. 157 41

A 34-year-old man was admitted in May, 1989 because of severe left upper abdominal pain, which was caused by advanced splenomegaly. On initial examinations, peripheral blood showed leukocytosis (13,400/ml) including 60% hairy cell which also infiltrated in bone marrow (64%). The patient was diagnosed as having hairy cell leukemia (Japanese type) of B-cell lineage. Splenectomy was performed as an initial treatment. The effect of splenectomy was only palliative and transient leukocytosis progressed thereafter. alpha-interferon and bestrabucil (KM 2210) were then adopted for 4 months respectively. The effects were, however, unsatisfactory. Subsequently the patient was treated successfully with 2'-deoxycoformycin (DCF). Complete remission was attained following 12 injections of 7.5 mg/body of DCF during 5 months and durable remission persists for more than 6 months without maintenance therapy. The side effect was minimum.
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PMID:[Hairy cell leukemia successfully treated with 2'-deoxycoformycin following refractoriness to splenectomy and alpha-interferon therapy]. 163 72

Hairy cell leukemia is a malignant B-cell disorder characterized by splenomegaly and pancytopenia. The malignant cell is morphologically unique and characterized by fine cytoplasmic projections. Although studies of the cell have revealed important information about its proliferative capacity, cell surface, and membrane composition, less is known about the metabolic characteristics of the cell. We have previously investigated the oxidative metabolism of the hairy cell and have suggested that hairy cells might have a unique glucose metabolism compared to normal lymphocytes. This is indicated by a high rate of [6-14C]glucose oxidation in short-term culture consistent with an active Kreb's cycle and a high ratio of [6-14C]glucose oxidation to [1-14C] glucose oxidation. In this study, we evaluated an additional group of patients with hairy cell leukemia prior to or after treatment with the experimental drug 2'-deoxycoformycin (dCF). We found that in seven of eight patients the leukemic cells had a pattern similar to that previously described and that all of these seven patients had a significant response to therapy. The cells of the eighth patient had minimal Kreb's cycle activity, and at the time of study the patient was resistant to therapy with dCF. The metabolic activity of hairy cells may distinguish them from other lymphoid populations and may be a marker for sensitivity to dCF.
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PMID:Glucose metabolism of hairy cells. 164 Jul 37


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