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)

Three cases of malignant histiocytosis occurring in children aged 2 months, 10 months and 14 years, are described. In all children the diagnosis was based on anaemia, granulocytopenia or thrombocytopenia, splenomegaly and marked erythrophagocytosis by bone marrow and lymph node atypical histiocytes. Two children aged 10 months and 14 years, underwent splenectomy after which combined chemotherapy with cyclophosphamide, vincristine and prednisone (COP) was started. In the older child a complete remission was achieved. The younger child died soon after the onset of the treatment. The youngest child was treated with bleomycin, adriamycin, cyclophosphamide, vincristine and prednisone (BACOP). He died of pneumonia and sepsis two months after the start of the treatment.
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PMID:Malignant histiocytosis. Histiocytic medullary reticulosis. 7 57

Hairy cell leukemia is a chronic but steadily progressive malignancy usually of older males. Clinically, patients present with splenomegaly and/or cytopenia. The diagnosis is made by demonstration of the hairy cell in Wright's-stained peripheral blood and in bone marrow and by the characteristic histologic findings in the bone marrow biopsy and spleen sections. Infection is the most significant problem complicating the course of patients with hairy cell leukemia and the role of granulocytopenia and/or monocytopenia is undoubtedly substantial. Splenectomy has produced an objective response in improving hematologic parameters in a large number of patients and may prolong survival in those patients who respond. The precise nature of hairy cells remains unknown. The cells exhibit features of both monocytes and B-lymphocytes in morphologic, cytochemical, immunologic and functional studies. A more complete understanding of the monocytic and lymphocytic stem cells and their maturation may provide insight into the origin of the hairy cell.
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PMID:Hairy cell leukemia: a review. 8 94

We have investigated the mechanism of splenic irradiation-induced granulocytopenia in two patients with myelofibrosis and marked splenomegaly. Serial assays were performed for circulating granulocyte-monocyte progenitors capable of colony formation in vitro (CFU-C). For comparison, similar studies were performed on two patients receiving whole brain irradiation for glioma. Splenic irradiation caused a significant decrease in circulating CFU-C in the myelofibrosis patients. There was no decrease in circulating CFU-C in the brain-irradiated patients. No radiation-induced humoral inhibitor of granulopoiesis and no increased CFU-C radiosensitivity could be demonstrated in the myelofibrosis patients. These observations, taken together with previous data on splenic blood flow and pooling, suggest that the major mechanism of irradiation-induced granulocytopenia in myelofibrosis is destruction of proliferating precursor cells in the splenic tissue and sinusoids.
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PMID:Splenic irradiation in myelofibrosis: effect on circulating myeloid progenitor cells. 50 23

Alcohol-related disturbances are seen against the three blood cell systems. They appear after important alcohol consumption within few days and are independent from the existence of liver cirrhosis with splenomegaly. They are promptly and completely reversible after interruption of alcohol supply. Disturbances in erythropoiesis are manifested in bone marrow with megaloblasts, ring sideroblasts, and vacuoles in cytoplasma and nucleus of nucleated red cells. They are caused by folate deficiency and by perturbations of iron utilization, which is perhaps connected with impaired heme synthesis following pyridoxal phosphate deficiency. Serum iron generally increases during alcohol consumption and decreases in the following alcohol-free period. The anemia may be macrocytic and normochromic or dimorphic with hypochromic microcytes. Anemias of hard alcohol drinkers are observed also as consequence of bleeding or hemolysis of different causes. The lability against infections of drinkers is associated with changes in granulopoiesis. The most important findings are granulocytopenia, vacuoles in the immature marrow cells, perturbations in granulopoietic maturation, and decrease of marrow response. Frequently, alcohol drinkers demonstrate thrombocytopenia which is caused by ineffective thrombopoiesis and by shortened life span of platelets as direct effect of ethanol. Functional impairments of thrombocytes have been published, too.
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PMID:[Alcohol-related disturbances in haematopoiesis (author's transl)]. 64 97

We studied the clinical and laboratory characteristics of 71 patients with hairy cell leukemia to ascertain whether prognostic factors important for the therapy of the disease existed. Granulocytopenia of less than 500/mu 1 was associated with the occurrence of a serious infection in 46% of the patients, compared to 19% of patients whose absolute granulocyte count was greater than 500/mu 1 (P less than 0.05). In most patients with symptoms of thrombocytopenia or leukopenia, splenectomy led to improved blood counts. Patients treated with splenectomy only had a significantly longer survival than those treated with steroids only (P less than 0.05). A group of patients who were slightly older and had minimal splenomegaly and few hairy cells in the peripheral blood did well without therapeutic intervention. Twenty-four patients have died, 16 of an infectious process. The actuarial survival for the group is 51% at 4 years, and there has been no change subsequently.
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PMID:Hairy cell leukemia: a clinical review based on 71 cases. 71 40

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

A quantitative antiglobulin consumption technique was used to measure immunoglobulin G (IgG) present on human granulocytes. Granulocytes from 50 normal subjects had less than 20 X 10(-14) g IgG per cell. Patients with granulocytopenia due to bone-marrow failure, patients with IgG multiple myeloma, patients with splenomegaly, and patients with rheumatoid arthritis without granulocytopenia had granulocyte-bound IgG within the range of normal. Four patients with rheumatoid arthritis, splenomegaly, and severe granulocytopenia (Felty's syndrome) had granulocyte-bound IgG between 30 and 220 X 10(-14) g IgG per cell. One of these patients underwent splenectomy, after which his granulocyte-bound IgG fell to normal. Seven additional patients with Felty's syndrome who had previously undergone splenectomy had normal levels of granulocyte-bound IgG. Thus quantitation of granulocyte-bound IgG appears to be useful in defining patients with immunologically mediated granulocytopenia. Studies of patients with Felty's syndrome who have undergone splenectomy suggest that the spleen may produce this neutrophile-bound immunoglobulin.
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PMID:Felty's syndrome: granulocyte-bound immunoglobulin G and splenectomy. 97 Jul 68

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

A 1-month-old girl was admitted because of staphylococcal cellulitis of the buttock and the shoulder, and peripheral agranulocytosis. CBC on admission showed 10,100/microliters of WBC with 1% mature neutrophils, 5% monocytes, 6% eosinophils, 2% basophils and 86% lymphocytes. Bone marrow aspiration revealed maturation arrest of neutrophil precursors at the level of myelocyte. We treated the patient with subcutaneous rhG-CSF (Kirin-Amgen, Tokyo) for sequential 7-day course at the starting dose of 3 micrograms/kg, and increased weekly. The dose was escalated at the level of 18 micrograms/kg for 2 weeks subcutaneously, 8 days after effective dose of 18 micrograms/kg, the absolute neutrophil counts more than 1,000/microliters was attained, and bone marrow aspiration showed an increase of neutrophil precursors beyond the myelocyte level with maturation. Our case proved that both WBC and absolute neutrophil counts were increased parallel with the dose escalation of rhG-CSF. Shortly after the cessation of rhG-CSF, WBC and absolute neutrophil counts were decreased. No side effect was detected except for mild splenomegaly which was resolved after cessation of rhG-CSF. In methylcellulose culture with PHA-LCM, marrow cell of patient produced normal number of CFU-GM, and myeloid precursors could proliferate and differentiate to normal polymorphonuclear neutrophils, but rhG-CSF produced only small number of CFU-GM. Our case confirms that rhG-CSF is a new approach to control the life-threatening infection of congenital agranulocytosis.
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PMID:[A successful case of congenital agranulocytosis treated with recombinant human granulocyte colony-stimulating factor]. 170 2

Hairy cell leukemia (HCL) is a rare chronic lymphoproliferative disorder which has been extensively studied over the past decade. Much has been learned regarding the diagnosis, natural history, biology, and treatment of this unique neoplasm. The disease most commonly affects middle aged men and characteristic clinical features include splenomegaly, cytopenias, and usually the presence in the peripheral blood of distinctive 'hairy cells' with irregular cytoplasmic projections. Diagnosis can usually be confirmed by bone marrow biopsy. Although the natural history can be extremely variable among patients, complications are usually referable to the cytopenias, with anemia and infection being most frequent. In addition to pyogenic infections, patients are susceptible to unusual organisms including atypical mycobacterium, legionella, and fungi. The requirement of red blood cell transfusion, severe granulocytopenia or thrombocytopenia, frequent infections, or painful splenomegaly are all indications for treatment. Splenectomy is the standard initial treatment of choice. However, in the past few years there have been exciting major advances in the therapeutic modalities for HCL. Recombinant alpha-interferon is highly effective, with beneficial responses occurring in close to 90% of patients. The Food and Drug Administration has recently approved the use of interferon for HCL. This represents the first time a biological response modifier has been approved for the treatment of human disease. In addition, preliminary results with the adenosine deaminase inhibitor, 2'deoxycoformycin (dcf), have been encouraging. Further clinical trials are required in order to determine the optimal sequential treatment strategy for HCL. The exact mechanisms of action of both interferon and dcf in HCL remain to be elucidated. A better understanding of the unusual features of the hairy cell and the underlying biological effect of these two agents in HCL may have important applications in other hematologic and non-hematologic malignancies.
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PMID:Hairy cell leukemia: clinical features and therapeutic advances. 244 91


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