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)

A 56-year-old man who had easy bruising, an enlarged left supraclavicular lymph node, and splenomegaly was diagnosed as having hairy-cell leukemia. Treatment consisted of splenectomy. Because of progressive lymphadenopathy in the following months, the patient required reevaluation. Examination of a lymph node biopsy specimen now revealed Hodgkin's disease, nodular sclerosis. This is the first pathologically confirmed case of hairy-cell leukemia coexisting with Hodgkin's disease.
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PMID:Hairy-cell leukemia associated with Hodgkin's disease: a case report. 397 51

A clinical and laboratory evaluation of 28 patients with hairy cells leukemia is performed. Twenty-two had splenomegaly and all but one had a pancytopenia with 5 to 70% of hairy cells in blood. A tartrate-resistant acid phosphatase activity was positive in the hairy cells of 11 patients of 14 studied. In all patients a myelofibrosis and a leukemic infiltration were found in a bone-marrow biopsy of iliac crest. Hemodilution by splenomegaly, mild hemolysis and dyshematopoiesis were observed in 10 patients by a 51Cr or 59Fe isotopic exploration. In seven cases an immunological study of the hairy cells was performed, a high percentage of the leukemic cells of these 7 patients had polyclonal surface Ig but without resynthesis of monoclonal S Ig which is a feature usually associated with B lymphocytes. In the blood of these patients normal T and B lymphocytes were decreased. A splenectomy was done in 12 patients (43%) always for severe pancytopenia Splenectomy was not randomised. Spleen weights ranged from 1 085 to 3 600 g. In splenectomised patients the level of hemoglobin, segmented cells and thrombocytes was significantly higher after surgery. The survival rate is better in the splenectomised group (median survival 57 months) than in the non-splenectomised group (median survival 19 months). Infectious diseases were frequent in all patients but less after splenectomy. Fourteen patients died, 8 owing to pancytopenia.
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PMID:[Hairy cell leukemia. I.--Clinical, biological and evolutive study on twenty-eight cases (author's transl)]. 627 Jul 94

Two men with advanced but previously untreated B cell hairy-cell leukemia were treated with low doses of pentostatin (2'-deoxycoformycin) in intermittent courses. There was prompt clearance of hairy cells from the blood, regression of splenomegaly and lymphadenopathy, and correction of anemia, thrombocytopenia, and granulocytopenia. Side effects were tolerable and myelosuppression was not observed. Both patients achieved complete remission documented by bone marrow aspiration and biopsy and radionuclide scans of liver and spleen. They remain in complete remission nine and six months, respectively, after their last treatment. Pentostatin (Warner-Lambert, Ann Arbor, Mich) is highly active in hairy-cell leukemia and merits more extensive evaluation in this disease. A woman with hairy-cell leukemia has begun treatment with pentostatin, and at ten weeks there is disappearance of gross splenomegaly and clearance of hairy cells from the blood. Bone marrow studies have not yet been repeated.
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PMID:Hairy-cell leukemia: induction of complete remission with pentostatin (2'-deoxycoformycin). 633 21

A syndrome which is known as plasma cell dyscrasia with polyneuropathy and various endocrine manifestations or plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, abnormal M protein and skin changes is very interesting because this syndrome has miscellaneous manifestations such as skin hyperpigmentation, hypertrichosis, polyneuropathy, M protein abnormality, plasma cell dyscrasia and endocrine disturbances. Miscellaneous endocrine abnormalities which have not been described so far are reported here. A 47 year old female was admitted with the chief complaints of edema and gait disturbance. Past and family histories were noncontributory. In April 1981, edema appeared in her face and legs. In June she noticed paresthesia in her legs. Edema increased gradually and she had difficulty walking. Her skin became pigmented and hairy. In October she was admitted because of polyneuropathy with increased cerebrospinal fluid protein without pleocytosis. Prednisolone was started. Walking improved slightly, but edema and paresthesia remained unchanged. Prednisolone was stopped at the end of the following March. In May 1982, she was admitted for further evaluation of edema and polyneuropathy. The patient was alert and cooperative. On standing the skin of her legs became cyanotic. There was hypertrichosis on the arms and legs. Her fingers were clubbed. A moderate swelling of the cervical lymph nodes was noted. There was mild hepatomegaly without splenomegaly. All tendon reflexes were lost. Plantar response was flexor. Muscular strength diminished mildly. She complained of paresthesia on the soles. Superficial sensation was normal. Vibratory sense decreased mildly. Cerebellar function and cranial nerves were normal. There was no sphincter disturbance. The examination of urine, stool and peripheral blood was normal.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Marked endocrine abnormalities in polyneuritis with skin hyperpigmentation, hypertrichosis, edema and increased lambda type immunoglobulin A: a case report]. 641 99

An unusual period of prolonged disease control after splenectomy without chemotherapy was achieved in 3 cases of B-cell lymphocytic lymphoma with pancytopenia, massive splenomegaly, minimal lymphadenopathy and circulating hairy cells. The finding of distinctive histopathological features in the bone marrow, lymph nodes and splenic tissue enabled this unusual condition to be characterized and differentiated from hairy cell leukaemia, which may have similar presenting features and in which response to splenectomy is generally good.
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PMID:Unusual response of lymphocytic lymphoma (not hairy cell leukaemia) to splenectomy without chemotherapy. 660 43

Clinical and morphologic characteristics of hairy cell leukemia at 11 patients have been analyzed. The frequency of this disorder is 2.5% from all leukemias. This disorder is often retrospectively diagnosed--at 7 cases from our study. the disorder is often retrospectively diagnosed--older age (78%). Splenomegaly is the main clinical manifestation, at all 11 patients. Pancytopenia is frequent finding but at 4 patients the leucocyte count was over 10 X 10(9)/1 in the beginning. Hairy cells, although not always with typical appearance, in 90% of the cases are found in peripheral blood over 10%. Bone marrow biopsy cytological and hystological findings at 9 patients were typical for diagnosis. Fibrosis was present in 6 specimens. At 7 patients diagnosis was confirmed with histological examinations of lymphocyte concentrates from peripheral blood on thin and ultrathin sections, as well as with electron microscopy characteristic appearance of hairy cells. Pneumonia as complication was registered in 24 occasions, gastro-intestinal infections at 9, haemorrhagic syndrome at 4 and skin carcinoma at 2 cases. Treatment was variable--2 patients were observed for more than 50 months, 2 were splenectomised, of which one with complete remission longer than a year, while from 8 treated with COP protocol, complete remission was obtained in 5 (62%) patients, and two treated with CHOP protocol entered complete remission for longer than 12 months. Average survival is 51 months (2--144). Three (28%) patients died.
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PMID:[Hairy cell leukemia. Personal experience with 11 cases]. 668 Mar 1

A patient with moderate splenomegaly and the leukemic phase of hairy cell leukemia presented with a white blood cell count (WBC) of 85,600/mm3 with 87% hairy cells and platelets of 125,000/mm3. Initial therapy consisted of chlorambucil alone at 4 mg each day. Within 6 months, the WBC decreased to 27,000/mm3 with 82% hairy cells, and the spleen was not palpable; platelets remained at 142,000/mm3. The patient has continued to do well for 2 years since the start of therapy, requiring no transfusions and having no problems secondary to organ infiltration. Selected hairy cell patients, those with a significant leukemic phase and with only mild thrombocytopenia, may benefit from initial chlorambucil therapy rather than immediate splenectomy.
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PMID:Initial alkylating agent chemotherapy in a leukemic hairy cell patient. 669 12

The authors reviewed the clinical course of 31 consecutive patients with hairy cell leukemia seen at the University of California Los Angeles. The clinical presentation included varying degrees of pancytopenia, splenomegaly, and bone marrow infiltration with hairy cells. Ten patients were identified as having an "atypical" disease, which is defined as absence of palpable splenomegaly and/or marrow cellularity of less than 45%. These atypical patients had clinically milder disease and significantly less anemia than the usual patient (mean hemoglobin, 12.1 g/dl versus 9.4 g/dl; P = 0.016), although neutropenia and thrombocytopenia were comparable. Mortality and infection rates were similar in both groups. Infections were common in all patients, but opportunistic infections and septicemia were rare in patients prior to initiation of therapy. Two thirds of the patients who received corticosteroids and/or cytotoxic agents had serious infections, with a 50% mortality rate. Nearly 70% of the neutropenic patients (leukocyte count less than 1000) who received any form of treatment had a serious infection. The most important factors predicting mortality were chemotherapy and an age older than 50 years. Patients who survived 2 years with their disease had an excellent prognosis, and four patients in this series are alive and well with their disease for more than 10 years.
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PMID:Hairy cell leukemia. Disease pattern and prognosis. 673 79

From 1972 to 1981 10 patients with hairy cell leukaemia were observed in the Medical University Clinic Cologne, this represents 1.8% of all leukaemias. Typical clinical signs are splenomegaly, no or only slightly enlarged lymph nodes and a moderate hepatomegaly. Almost in all cases an anaemia, thrombocytopenia and neutropenia with lymphocytosis was found, mostly combined as pancytopenia. The pathognomonic tartrate resistant acid phosphatase was found in the hairy cells to a differing amount besides a fibrosis and a lymphatic infiltration of the bone marrow. A normalization of the anaemia, the thrombocytopenia and the neutropenia was reached by splenectomy, but the increased susceptibility to infections could not be affected significantly.
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PMID:[Clinical aspects of hairy cell leukemia and its modification by splenectomy]. 676 22

The clinical and histological features of 16 cases of hairy cell leukaemia are described. The disease mainly affected middle aged and old men. All patients had clinically detected splenomegaly and more than half the patients had "dry" bone marrow aspirations. Most of the patients had pancytopenia but one patient had leucocytosis. The spleen was usually markedly enlarged; the mean weight of the excised spleens was 1700 g. The histological findings were typical. In the bone marrow there was monotonous proliferation of small mononuclear cells combined with an increase in reticulin. In the spleen numerous similar cells were seen in the red pulp with almost total replacement of the white pulp. Tumour cell infiltrates were also seen in the liver where they mainly involved the sinusoids, and in the lymph nodes where they caused partial obliteration of the lymph node structures. Four patients died of causes related to hairy cell leukaemia during the follow-up period. The immediate cause of death was infection in every case. Three of these patients had received chemotherapy as opposed to 3 of the 12 patients who did not die of hairy cell leukaemia.
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PMID:Hairy cell leukaemia. A clinico-pathologic study of 16 patients. 688 6


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