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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The splenic red cell volume has been measured directly by an isotope method with quantitative scanning in 10 patients with leukaemic reticuloendotheliosis (hairy cell leukaemia). The volume ranged between 211 and 726 ml (mean 410 ml, SD 158) and this constituted 15--48% (mean 28.1%, SD 9.5) of the total circulating red cell volume. This is an exceptionally large pool when compared with that found in myeloproliferative and lymphoproliferative disorders with the same degree of splenomegaly. It is consistent with the histological features which show marked red cell accumulation in the splenic cord areas. The red cell pooling in the spleen thus appears to be a significant factor in the anaemia and there was fairly good correlation between the percentage of improvement in the anaemia and the percentage of red cell volume contained in the spleen. By direct measurement of the splenic red cell pool, it is possible to predict the extent to which splenectomy will benefit the anaemia and this may also provide an indirect measure of the extent of bone marrow dysfunction in the causation of the anaemia.
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PMID:Splenic red cell pooling in hairy cell leukaemia. 87 2

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

Evaluation of the histologic changes of the bone marrow and clinical data of twelve male and two female patients with hairy cell leukamia before treatment. Mean age of the patients was 46 years, time from the onset of symptoms 5.5 months. 13 of the patients were anaemic, showing splenomegaly, 12 suffered from thrombopenia, 9 from granulocytopenia, and 8 from hepatomegaly. In all of the cases, the relative numbers of lymphocytes in the blood had been increased together with various amounts of characteristic hairy cells. All of the cases had a typical histologic picture of lymphocytic bone marrow infiltration, mostly of the diffuse type. In 50% of the cases not only the well known rod-like intracellular inclusions could be seen, but also ring-shaped figures whose significance is discussed. Decrease of the granulopoiesis, disintergration of the marrow sinusoids, and osteoporosis are the most important additional signs. The progression of the disease is marked by increasing bone marrow infiltration, by splenohepatomegaly, anaemia, thrombopenia, and increasing numbers of typical lymphocytes in the blood. The bone marrow being considered to be the origin of the disease for good reasons, the histobiopsy of this organ ranks among the diagnostic and prognostic measures to be taken at first sight.
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PMID:[Bone marrow diagnostic in hairy cell leukaemia (author's transl)]. 92 7

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

Tumour necrosis factor (TNF) can act as an autocrine growth factor for hairy cell leukaemia (HCL) cells. The TNF produced by the malignant clone may also inhibit normal haematopoiesis thereby contributing to the cytopenias observed in patients with the disease. We have studied the effects of infusing a murine monoclonal anti-TNF antibody in three patients with HCL. In two patients receiving 0.5 mg of antibody/kg on alternate days for 12 d, the drug was well tolerated. The third patient received 2 mg/kg on alternate days and developed symptoms of serum sickness by day 9. In two patients with severe B-lymphocytopenia, circulating CD19 and CD20 positive, B-cells were restored to normal, the majority of which were negative for the HCL-associated marker CD11c. B-lymphocyte recovery was associated with a rise in serum immunoreactive IL-6 and with an early rise in immunoreactive TNF. These short courses of anti-TNF MAb treatment had modest effect on the tumour burden, producing a reduction in splenomegaly in one patient. Exploration of the effects of more prolonged administration of higher dose anti-TNF antibody will only be feasible when less immunogenic MAbs are available.
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PMID:Effects of anti-TNF monoclonal antibody infusion in patients with hairy cell leukaemia. 137 67

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

We have followed for 33 months the changes that occurred in natural killer (NK) cell numbers and activity in a patient (A) with hairy cell leukaemia (HCL), using a single cell assay and a microcytotoxicity assay. The composition of the peripheral blood mononuclear cell population and malignant cell phenotype were also analysed. During this period he received treatment with interferon and his grossly enlarged spleen was removed. Four further patients were also studied, two were splenectomized and all had received treatment with interferon. In four of the five patients studied there was an apparent link between low NK activity and presence of a tumour-infiltrated spleen, and in the fifth patient, who was aleukemic and had no splenomegaly, NK function was related to disease activity. There was no correlation between NK activity and the number of target binding (TB) cells in these five patients. IFN had little direct effect on overall NK activity, but the proportion of killing cells among TB cells was increased. Three patients showed binding of several cells to a single target. Further analysis revealed that in the patients most of the TB cells were not CD56-positive NK cells, in contrast to TB cells from normal subjects. In patient A a large proportion (84%) of TB cells were identified as malignant cells and in patient E 15% of TB cells were malignant cells. The phenotype of the malignant cells was: CD19+, HLA-DR+ and CD25(Tac)+, except for patient A. In this patient the hairy cells were positive for the NK marker CD56 as well as the monocyte marker CD14. Furthermore, a change occurred in phenotype as only later samples carried CD25. It is concluded that the level of NK function correlates closely with disease activity in HCL and that competitive target cell binding by malignant cells may be one cause of depressed NK-cell function in hairy cell leukaemia.
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PMID:Natural killer cell function and malignant cell phenotype in hairy cell leukaemia. 153 17

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


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