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)

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.
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PMID:CD11c (LEU-M5) expression characterizes a B-cell chronic lymphoproliferative disorder with features of both chronic lymphocytic leukemia and hairy cell leukemia. 137 51

Hairy cell leukemia (HCL) is a lymphoproliferative disorder of B-lymphocytes, with pathological manifestations usually including splenomegaly and pancytopenia. Naturally occurring and recombinant interferons (IFNs), specifically of the alpha subtype, have shown a significant anti-tumor effect in HCL patients, with improvement of hematologic parameters within the first few months of treatment. The mechanisms responsible for the beneficial action of IFN-alpha in HCL patients are unclear, but several hypotheses have been suggested. Recently, a continuous line of cells (Eskol) from a patient diagnosed with hairy cell leukemia was established and shown to have several properties of a leukemic hairy cell. In the present study, we investigated the direct effect of IFN-alpha and interleukin (IL-2) on the Eskol cell line, and lymphokine regulation of natural killing (NK) activity against these cells. It was found that IFN-alpha has a direct antiproliferative effect on Eskol cells. Furthermore, Eskol cells were found to be completely resistant to NK-cell mediated cytotoxicity (CMC) but were somewhat sensitive to either IFN-alpha-primed NK or lymphokine-activated killer (LAK) cells-CMC. The resistance of Eskol cells to NK-CMC is due to a low binding ability to effector cells. Moreover, it was found that like IFN, IL-2 can protect Eskol cells from activated NK-CMC. Both cytokines reduced the ability of Eskol cells to induce NK-cytotoxic factor (NKCF) release from NK cells following conjugate formation between Eskol cells and effector cells. Moreover, cycloheximide treatment abolished the protective effect against NK-CMC induced by IFN-alpha or by IL-2. Therefore, it seems that the protective effect against NK-CMC induced by both cytokines is mediated via the same mechanism.
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PMID:Cytokine and natural killing regulation of growth of a hairy cell leukemia-like cell line: the role of interferon-alpha and interleukin-2. 173 47

Nine consecutive patients with HCL seen over a period of five years were reviewed. Male: Female ratio was 8:1. Median age at diagnosis was 49 years. Weakness and fatigue (66%) were the commonest presenting symptoms and splenomegaly (66%) was the commonest physical findings. Varying degrees of pancytopenia was the consistent feature in majority of cases. Diagnosis was made on the basis of bone marrow biopsy and characteristic EM picture. Forty-four percent of cases developed serious infection during their clinical course. Gram negative bacilli and fungi were the most frequently isolated organisms. Major sites of infections were pneumonia and septicemia. Splenectomy was carried out in four cases. Rapid recovery of haematological parameters without any significant complication was observed in all these cases. Two patients were treated with alfa-interferon. In both the cases recovery of haematological parameters was slow compared to those under going splenectomy. One patient treated with alfa-interferon died due to infection related complications while the other went into remission.
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PMID:Hairy cell leukaemia. A review of nine cases. 178 82

Clinicopathological findings of six cases of Hairy cell leukaemia are presented. All the patients were males, the age ranged between 32-57 years. Complications of anaemia and neutropenia were common modes of presentation. Hepatomegaly and splenomegaly were present in all the cases whereas only 2 patients had lymphadenopathy. Severe pancytopenia was detected in 3 cases and circulating hairy cells were present in all the cases. Trephine biopsy done in all six patients was found to be diagnostic. Tartrate resistant acid phosphatase was detected in the hairy cells of 2 cases.
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PMID:A clinico-pathological study of six cases of hairy cell leukaemia. 179 14

Hairy cell leukemia (HCL) expressing both surface monocytoid antigen and IgM (kappa) was reported. A 62-year-old male was admitted to our hospital in September 21, 1989 because of leukocytosis. Physical examinations showed axillary and inguinal lymphadenopathy but no hepato-splenomegaly. The leukocyte count was 12,600/microliters with 73% of abnormal cells like large lymphocytes which had abundant cytoplasm and hairy appearance under phase microscopy. They had ruffles with microvilli under electron microscope. Bone marrow puncture showed normocellular marrow with 71.2% of abnormal cells similar to the peripheral blood. Surface markers were CD11b+, CD21+, HLA-DR+, Tac- and IgM (kappa). They were positive for ++acid phosphatase staining, but negative for peroxidase and tartrate-resistant acid phosphatase staining. He was diagnosed as Japanese type HCL. HCL expressing both surface monocytoid antigen and IgM is rare and the clinical features of our case are compared with those reported in Japan.
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PMID:[Hairy cell leukemia expressing SIgM+, SIgG-, CD11b+ and CD21+ and accompanying lymphadenopathy without splenomegaly]. 182 8

The experience at the National Cancer Institute from 1955 to 1988 with 46 cases of splenectomy for massive splenomegaly (greater than or equal to 1,500 grams) was reviewed to assess the indications, pathology, operative, and postoperative course for this procedure. The median age was 51 years. Thirty-one splenectomies (67.4%) were performed for malignancy (chronic lymphocytic leukemia, 11; chronic myelogenous leukemia, 10; lymphoma, 9; hairy cell leukemia, 1), 11 for myeloid metaplasia, and four for other nonmalignant conditions. Indications for splenectomy included hypersplenism (32 patients), symptoms (6), diagnosis (3), and splenic rupture (3). A midline incision (30 patients) was most commonly used. Median operative time was 2 hours, 50 minutes. Median operative blood loss was 1,300 ml (range, 100 ml-60 units). The splenic artery was ligated initially in 16 patients (34.8%) but did not correlate with blood loss or operating time. The median splenic weight was 2,030 grams (range, 1500-5320 gm). The postoperative complication rate was 39.1 per cent (21 complications in 18 patients). This included infection in 10 patients, bleeding in six patients. Six patients required reoperation (bleeding, 4; abscess, 1; small bowel obstruction, 1 patient). The 30-day operative mortality was 19.6 per cent (9 patients). Excluding operative deaths, 35 patients were available for follow-up evaluation. Twenty-nine patients had improvement in parameters for which splenectomy was indicated. Six patients had no change in their course after splenectomy. These findings indicate that many patients with massive splenomegaly benefit from splenectomy, however, the procedure is associated with a high risk for postoperative morbidity and mortality.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Splenectomy for the massively enlarged spleen. 199 65

Splenic B cell lymphoma with circulating villous lymphocytes (SLVL) is a lymphoproliferative disorder characterized by the presence in the peripheral blood of atypical B-lymphocytes with hairy appearance. Although the clinical features with massive splenomegaly, absence of peripheral lymphadenopathy and blood cytopenia may mimic hairy cell leukemia (HCL), precise analysis of the morphologic and immunologic features allow differential diagnosis between these two entities. Bone marrow and spleen histology resemble the pattern in chronic lymphocytic leukemia (CLL). We studied 8 patients with this entity illustrating the difficulty of diagnosis between SLVL and HCL.
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PMID:Splenic B-cell lymphoma with villous lymphocytes (SLVL). A lymphocytic lymphoma simulating hairy cell leukemia. A study of 8 cases. 210 74

In this report the clinical, morphologic, histologic and immunologic findings of 41 patients with hairy lymphoid cells in peripheral blood and/or bone marrow are analyzed. In 27 patients the diagnosis of hairy cell leukemia was established. 14 patients had other variants of lymphoproliferative disorders: malignant lymphoma with hairy cells--7, chronic lymphocytic leukemia with hairy cells--5, and T cell lymphoproliferative disorder with hairy cells--2 patients, respectively. Several variants of malignant lymphoma with hairy cells were defined: lymphocytic, centrocytic and lymphoplasmacytic. The importance of combined use of bone marrow biopsy and immunophenotyping for the correct diagnosis of hairy cell leukemia and other "hairy-cell" lymphoproliferative disorders is stressed. The obtained data suggest relationship between characteristic clinical manifestation (isolated splenomegaly), presence of hairy cells and CD11c-antigen expression.
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PMID:Hairy cell leukemia and other "hairy-cell" lymphoproliferative disorders (LPD-HC): the significance of morphologic, histologic and immunologic studies. 223 4

Hairy cell leukemia (HCL), a well-recognized chronic lymphoproliferative disorder, is frequently characterized by pancytopenia, monocytopenia, splenomegaly and marrow fibrosis, which typically leads to an unsuccessful bone marrow aspiration (dry tap). Patients with a high white cell count without neutropenia and/or monocytopenia, with an aspirable and hypercellular marrow, splenomegaly and neoplastic cells with hairy cell features have been recently recognized and classified as HCL variants. We report here the clinical, hematological and immunological features of 7 such cases. All patients presented splenomegaly with a high leukocyte count; 2 were anemic and only 1 thrombocytopenic. Five patients were treated with alpha-Interferon (alpha-IFN) but 4 failed to achieve any significant response; two of these were subsequently splenectomized and successfully treated with Chlorambucil. Splenectomy, followed by Chlorambucil, was performed at diagnosis in the remaining 2 cases, both of which achieved a partial response and are alive and well. Six out of the 7 patients are still alive. The recognition of these peculiar patients is also important because they most often do not respond to alpha-IFN, while splenectomy, followed by Chlorambucil, may be a reasonable therapeutic option for them.
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PMID:Hairy cell leukemia variant: a morphologic, immunologic and clinical study of 7 cases. 233 88

We describe the clinical and laboratory features of 17 adult patients with a variant form of hairy cell leukemia (HCL-V) studied over the last 7 years. The main findings were: splenomegaly, moderate anemia, thrombocytopenia, and a raised white blood cell count (median 116 x 10(9)/L; range 15 to 482). The circulating lymphoid cells had abundant villous cytoplasm and a round, occasionally bilobed nucleus, with a prominent nucleolus. Monocytopenia, a feature of typical HCL, was not seen; neither was tartrate-resistant acid phosphatase demonstrated in eight cases tested. HCL-V cells had a mature B-cell phenotype: CD19+, CD20+, CD22+, FMC7+, CD11c+, CD10-, CD5-, with light chain isotope restriction in 15 cases. In contrast to typical hairy cells, HCL-V cells were negative with the monoclonal antibodies anti-HC2 and anti-TAC (CD25). Immunoglobulin (Ig) was not detected in two cases and IgG was expressed in the cell membrane of 73% of cases. Bone marrow histology was different from HCL, showing interstitial infiltration by cells clumped together and a moderate amount of reticulin, but the spleen showed the typical red pulp expansion of HCL. HCL-V patients did not respond to splenectomy (5 of 7) or alpha-interferon (7 of 7); 2 of 3 patients had a partial response to 2'deoxycoformycin. The clinical course was benign with 15 patients alive with a median survival greater than 4 years. We confirm that HCL-V is a distinct clinico-pathologic entity with intermediate features between HCL and B-prolymphocytic leukemia.
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PMID:A variant form of hairy cell leukemia resistant to alpha-interferon: clinical and phenotypic characteristics of 17 patients. 236 67


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