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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 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

Over a 4-year period 203 patients with various types of leukaemia were treated by the Haematology Unit at the Johannesburg Hospital. Ten of them were suffering from the condition known as hairy-cell leukaemia or leukaemic reticulo-endotheliosis. They were all men, and ranged in age from 29 to 67 years (mean 56 years). The majority presented with pancytopenia, and there was invariably splenomegaly, while lymphadenopathy was rare. Hairy cells were identified microscopically in the peripheral blood of 7 patients and in 5 the specific cytochemical marker, tartrate-resistant acid phosphatase, was present. In addition, in a further 2 patients this feature, which was not identified in the peripheral blood, was found in the splenic cells. The bone marrow trephine biopsy specimens characteristically showed extensive lymphoid infiltration associated with a dense disordered deposition of reticulin fibres. Electron microscopical and immunological studies proved to be of doubtful diagnostic value. Splenectomy was carried out on 9 patients, and there was tumour involvement in all the spleens removed. Two patients died from septicaemia, the one before splenectomy and the other 9 months after the operation. The 8 remaining patients have had their subjective symptoms alleviated and their peripheral blood indices have been improved by splenectomy, and none has required further treatment for periods now ranging from 7 to 41 months.
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PMID:The diagnosis and management of hairy-cell leukaemia. 8 70

Four cases of malignant histiocytosis with leukemic manifestations and chronic course were reported. Light microscopic, ultrastructural and ultrastructural cytochemical details of these atypical cells were demonstrated. Ultrastructurally these cells resembled hairy cells most closely among the known varieties of leukemic cells. However, ribosome-lamella complexes were not found and some atypical cells had a few short cytoplasmic projections. In addition, tartrate-resistant acid phosphatase was absent from these cells. We speculate that this leukemic reticuloendotheliosis with a chronic course seen in Japan seems to be analogous to malignant histiocytosis with massive splenomegaly reported by Vardiman et al.
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PMID:Malignant histiocytosis with chronic course. Ultrastructural and ultrastructural cytochemical studies. 14 14

A 64-year-old man who had a two year history of hairy cell leukemia became feverish with night sweats. Splenomegaly and enlarged nodes in the neck and in the anterior mediastinum were detected. Splenectomy was performed and multiple white nodules on a dark-red background were seen in the spleen. The white nodules represented a large cell malignant lymphoma; other areas of the spleen contained mononuclear cells typical in hairy cell leukemia. One of the hilar lymph nodes of the spleen was involved by malignant lymphoma, and the other eight lymph nodes were involved by hairy cell leukemia. The coincidence of the two diseases tends to favor the view that hairy cells are of lymphocytic origin.
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PMID:Coincidental hairy cell leukemia and large cell malignant lymphoma. 31 72

By lymphoid myelofibrosis or hairy cell leukaemia or tricholeukaemia is meant an unusual haemopathic condition known only for the past few years. It is characterized pathognomonically by the presence of lymphocyte type cells with villous extroflexions, hence the name "hairy cell". Clinically the disease presents as an involutive myelopathy associated with splenomegaly, generally without any particular lymph gland involvement. The attention of students today is concentrated on the nature of the hairy cells; while some are inclined to admit their monocyte or histiocyte derivation, others consider that they derive from B lymphocytes. Therapeutically, almost everybody agrees that splenectomy is the only valid step. A case of H.C.L., which was typical from the clinical and laboratory viewpoints is reported. It is probable that certain haemopathic pictures once classified among atypical leucoses and lymphomas, would today be more correctly classed as hairy cell leukaemia.
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PMID:[Lymphoid myelofibrosis or hairy cell leukemia]. 32 48

A laboratory and clinical evaluation of 24 patients with hairy cell leukemia was carried out over a 23-month period. Most patients had splenomegaly without adenopathy or pancyotpenia. Nine of the patients had undergone splenectomy prior to referral; their median WBC count was 6600/mm3. The median WBC count for the 14 patients who had no prior therapy was 3550/mm3, and their median platelet count was 80,500/mm3. Spleen weights ranged from 618 to 3780 g; there appeared to be no relationship between the size of the spleen and the response in the blood counts after splenectomy. Four patients in whom the majority of the WBC were hairy cells underwent splenectomy, which produced no real change in their WBC count; however, there was improvement in the platelet count in three. In contrast, the presence of leukopenia with a low percentage of hairy cells predicted a beneficial response to splenectomy. The study of surface immunoglobulins (SIg) in 16 patients demonstrated that resynthesis had occurred in each case. Phagocytosis of zymosan was studied in 15 patients; in 8 of these, 25% or more of the hair cells were capable of phagocytosis; in 6 others, 0--9%; and in one, 13%. The resynthesis of SIg is a feature usually associated with B-lymphocytes, but the phagocytosis of zymosan is not. Thus, the existence of either a spectrum of functional capabilities of hairy cells or several distinct subtypes is suggested by these data. Platelet aggregation with epinephrine was abnormal in 7 of 14 patients studied but there were no clinically significant bleeding problems. A chromosome abnormality was present in 2 of the 19 patients from whom adequate samples were obtained; the abnormality probably involved chromsome 12 in both patients as well as absent Y and was associated with a rapidly progressive clinical course. The presence of a predominant number of hairy cells with a normal or increased peripheral blood WBC count or of a chromosomal abnormality suggests that splenectomy might not be beneficial as the initial therapy and that chemotherapy should be considered.
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PMID:Hairy cell leukemia: an unusual lymphoproliferative disease: a study of 24 patients. 35 59

The experience of the University of Colorado Medical Center affiliated hospitals with leukemic reticuloendotheliosis, hairy cell leukemia, during the past two years has been reviewed. Eight instances were found. The majority of patients in this study presented with fatigue, pancytopenia and splenomegaly. Diagnosis was based upon finding characteristic hairy cells in the blood, bone marrow or spleen. Treatment, unlike other hematopoietic malignant conditions, was primarily surgical, with splenectomy being the treatment of choice, which may lead to prolonged remission in the majority of instances.
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PMID:Surgical leukemia. 68 93

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

Chronical course, splenomegaly and pancytopenia are characteristic for hairy cell leukemia. The main feature are so-called hairy cells in peripheral blood. This cells were investigated by cytological, cytochemical, histological and electron microscopical methods. Special cytoplasmic inculsions seem to be specific for this cell type.
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PMID:[Hairy cell leukemia]. 80 69

2 cases of hairy cell leukemia are reported. The disease is important for pathologists, because the splenomegaly is the leading clinical sign and often a splenectomy is performed with infomation about the true disease. The morphology of the spleen with infiltration of pulp cords and filling of sinuses by monomorphous blasts, with moderate fibrosis and atrophy of the white pulp is not convincing, because the hairy-like processes of the toumor-cell cytoplasm are not seen by usual histological examination. Hemocytomorphology and -cytochemistry are essential diagnostic procedures.
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PMID:[Hairy cell leukemia (author's transl)]. 84 70


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