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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The five-year experience with 75 consecutive splenectomies has been reviewed. Special detail was given to eight critically ill hypersplenic patients. Their diagnoses included Hodgkin's disease, lymphoma, leukemia, myelofibrosis and Felty's syndrome. Three presented with sepsis, two with anemia not responsive to transfusion, three had pathologic bleeding and two could not receive additional needed therapy of underlying disease because of low counts. All cases responded to splenectomy favorably. Hypersplenism is primarily a loss of balance between the splenic destruction-sequestration and bone marrow production. The demonstrated rapid consumption of transfused cells and some degree of functional reserve of the bone marrow is the prerequisite and clue for splenectomy response in critically ill patients.
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PMID:Splenectomy for hypersplenism. 70 6

After 1940, the number of splenectomies performed in the United States and elsewhere increased rapidly. Splenectomy for Banti's disease and malaria decreased gradually into disrepute. Removal of the spleen for idiopathic thrombocytopenic purpura, congenital spherocytic anemia and acquired hemolytic anemia became accepted practice. However, debate still continues regarding the proper indications for splenectomy in Gaucher's disease, Felty's syndrome and leukemia.
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PMID:The spleen and splenectomy. 194 96

To investigate the possibility that the recently recognised syndrome, leukaemia of large granular lymphocytes, could be associated with human T-cell leukaemia/lymphoma virus type I (HTLV-I), sera from 12 patients with this type of leukaemia were tested by the use of western-blot techniques for IgG antibodies to proteins related to human T-cell leukaemia/lymphoma virus type I (HTLV-I). Sera from 6 patients, including 2 patients with rheumatoid arthritis, reacted with p19 or p24 retroviral proteins or both. In contrast, no sample from 32 patients with uncomplicated rheumatoid arthritis, 27 with Felty's syndrome, 11 with other connective tissue disorders, or 21 normal individuals reacted with HTLV-I. The results suggest that leukaemia of large granular lymphocytes may be associated with a retrovirus related to HTLV-I.
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PMID:Serum reactivity to human T-cell leukaemia/lymphoma virus type I proteins in patients with large granular lymphocytic leukaemia. 288 Nov 34

Felty's syndrome (FS) refers to the occurrence of rheumatoid arthritis, splenomegaly, and neutropenia. A subset of these patients has recently been described with a chronic T cell leukemia of large granular lymphocytes (LGCL). To examine the spectrum of lymphocyte abnormalities in FS and LGCL, we examined phenotypic and genotypic properties of lymphocytes from eight FS patients. In two of these FS patients, we observed an elevated proportion of T cells with an unusual phenotype (CD3+/Leu-7+/Leu-8-/CR3+) (46 +/- 5% of mononuclear cells). The FS lymphocytes had large granular morphology on Wright-Giemsa stain and were active in antibody-dependent cellular cytotoxic activity. This phenotype, morphology, and activity was similar to LGCL patients except that the latter T cells additionally expressed the Fc-IgG receptor recognized by monoclonal antibody Leu-11 (CD 15). In the remaining six FS patients, the proportion of CD3+/Leu-7+/CR 3+ T cells was only 10 +/- 8%, which was not significantly different from age-matched normal subjects (6.6 +/- 2.2%). To determine the clonality of T lymphocytes in FS and LGCL, we examined DNA for rearrangements of the T cell antigen receptor beta-chain (Ti beta) and gamma-chain (Ti gamma) genes by using Southern blotting techniques. We found a clonal rearrangement of the Ti beta 1 and Ti gamma genes in both LGCL patients. In contrast, no clonal rearrangements of Ti beta or Ti gamma genes were detected in lymphocytes from the FS patients. These results indicate that FS patients are heterogeneous in their phenotype and that one subset exhibits polyclonal expansion of an unusual lymphocyte subset.
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PMID:Comparison of T cell receptor gene rearrangements in patients with large granular T cell leukemia and Felty's syndrome. 310 95

Large cell granulocytic leukemia (LCGL) or proliferative lymphocyte T gamma disease, characterized cytologically by the presence of lymphocytes with intracytoplasmic azurophil granules, raises the problem of whether or not it is monoclonal in character. However, although it may resemble a chronic lymphoid T leukemia or Felty's syndrome, it differs by the constant finding of infiltration of the splenic red pulp by large granular lymphocytes. Studies of their immunologic phenotype and functional activity produce heterogeneous results. The disease course varies considerably: the serious nature of the infections, knowledge of the physiopathologic mechanism of the neutropenia and the importance of the tumoral syndrome could represent therapeutic indications the modalities of which have still to be defined.
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PMID:[Heterogeneity of large granular lymphocyte leukemia. 2 cases]. 320 33

Twenty-one patients are described with a proliferation of morphologically mature T lymphocytes. The clinical course was chronic in most, and splenic enlargement the main clinical finding; skin involvement and lymphadenopathy were rare. The mean lymphocyte count at presentation was 8 X 10(9)/1 (range 0.75-24 X 10(9)/1). Nineteen of these patients showed some form of cytopenia (18 neutropenia, two red cell aplasia, eight thrombocytopenia) and one had hypogammaglobulinaemia. Seven patients had long-standing arthropathy serologically proven to be rheumatoid arthritis and these had previously been considered to have Felty's syndrome. Five of the group have died (three with an aggressive course), but most have remained stable for prolonged periods with a slow increase in peripheral lymphocyte count and marrow infiltration. Spontaneous regression was never observed but in two patients a prolonged remission was achieved by chemotherapy. The lymphocytes were morphologically and phenotypically homogeneous at presentation and remained so post-splenectomy; they contained azurophilic granules, stained with acid phosphatase but weakly or not at all with alpha napthyl acetate esterase. Membrane phenotyping shows the majority of the cells to be E+, Fc gamma+, OKT3+, OKT8+. Most cells do not stain with OKT1-like reagents and a significant number express HLA-Dr. From these and other reported cases it is clear that this condition represents a distinct entity resulting from the expansion of a subset of cytotoxic/suppressor T cells--the question of the benign or neoplastic nature of the disease remains open. Using T cell-specific antisera and E-rosetting techniques, a small percentage of CLL cases have been shown to be of T-cell origin (TCLL) (Dickler et al, 1973; Lille et al, 1973). Estimates of the percentage vary but in most series T-CLL has been diagnosed in less than 5% (Brouet & Seligmann, 1981), and this is supported by date from the M.R.C. Leukaemia Unit which found T-CLL in only 1.5% of 600 cases of CLL examined by marker studies (D. Catovsky, unpublished). Amongst the published reports of T-CLL a variety of clinical and morphological entities have been described including T prolymphocytic leukaemia (TPLL) (Brouet et al. 1975) and adult T cell disease in Japanese (Uchiyama et al, 1977) and West Indian Caribbean groups (ATLL) (Catovsky et al, 1982). In the original series of Brouet & Seligmann (1981) the group was defined as presenting in middle age with marked hepatosplenomegaly, some lymphadenopathy, skin involvement and with an aggressive disease course; peripheral blood and marrow lymphocytosis were variable.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic T cell lymphocytosis: a review of 21 cases. 633 88

In a retrospective study of 39 splenectomies, patients with increased blood cell breakdown (13 cases of idiopathic thrombocytopenic purpura (ITP), 5 cases of hereditary spherocytosis, 2 of Felty's syndrome and 2 of autoimmune hemolytic anemia) were compared with those patients also presenting decreased blood cell production [14 cases of myelofibrosis (MF) with splenomegaly and 3 cases of advanced chronic myelogeneous leukemia (CML)]. Platelet regeneration post-operatively was significantly (p less than 0.01) more rapid in the ITP than in the MF group. Only 1/22 patients in the ITP group had major post-operative complications as compared to 10/17 in the MF group. None of the patients in the ITP group died within 25 days of operation as compared to 5/17 in the MF group. Only 3/22 patients in the ITP group lost more than 800 ml of blood during the operation as compared to 8/17 with MF. No statistically significant higher blood loss was found in patients with less than 30 x 10(9) platelets/l preoperatively, compared to those with higher platelet counts. However, correlation between the splenic weights and amount of blood loss was statistically significant (p less than 0.01). Thus, splenectomy seems much better tolerated in patients with ITP, even if platelets are low, than in patients with myelofibrosis.
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PMID:[Splenectomy in idiopathic thrombopenic purpura and in myelofibrosis. A retrospective study of platelet increase, hemorrhagic complications and mortality]. 719 36

The authors report two patients with large granular lymphocyte (LGL) expansion associated with rheumatoid arthritis corresponding to pseudo Felty's syndrome. These cells have natural killer and T cell surface antigen markers. LGL are a heterogeneous population and expansion of these cells is responsible for leukemia, which is generally a monoclonal proliferation. It has been suggested that Epstein-Barr virus (EBV) is a putative agent in this leukemia. No EBV DNA was found with a polymerase chain reaction analysis in the lymphocyte DNA of our two patients. Some cases of pseudo Felty's syndrome have exhibited a monoclonal pattern on Southern blot analysis of the T cell receptor. On the contrary, our two cases showed a polyclonal pattern with TCR beta chain Southern blot analysis. This fact, associated with the mild course seen in both over more than twenty years, suggest that pseudo Felty's syndrome is a disease with a good prognosis.
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PMID:Pseudo Felty's syndrome. A polyclonal disease with a favorable prognosis. Report of two cases with Southern blot analysis of TCR. 829 49

The platelet-type thrombin receptor was expressed by large granular lymphocytes (LGLs) in a variety of proliferative diseases. Twenty patients with LGL proliferative disease were examined, including five T cell clones and a variety of polyclonal proliferations, some secondary to rheumatoid arthritis and Felty's syndrome; 17/20 showed high number of CD3+, CD8+, and CD57+ lymphocytes and 9/20 also had high numbers of CD16+ or CD 56+ positive lymphocytes. The thrombin receptor was present on more than 20% of the LGLs in 13/20 patients. The clonal T cell expansions showed the highest receptor expression with greater than 75% cells positive. Regression analysis of all 20 cases showed striking and highly statistically significant positive Spearman rank correlation between the proportion of thrombin receptor and CD57-positive LGLs (r = 0.56, P = 0.009). A negative correlation with CD56 was also found (r = -0.46, P= 0.043). Dual antibody flow cytometry showed the receptor was more often co-expressed with CD57 (64%) than with CD16 (19%) or CD56 (11%). The expression of the platelet-type thrombin receptor by LGLs of this phenotype raises the possibility of a functional role for thrombin in the pathogenesis of LGL proliferative diseases.
Leukemia 1996 Apr
PMID:Analysis of the platelet-type thrombin receptor in 20 cases of large granular lymphocyte proliferations. 861 48

A possible retroviral etiology for rheumatoid arthritis (RA) has been raised by results of recent studies. Therefore, we examined sera of patients with RA, including those with coexisting Felty's syndrome or leukemia of large granular lymphocytes, for the presence of antibodies to retroviral proteins of human T-lymphotrophic virus type I and type II (HTLV-I/II). Reactivity to recombinant HTLV-I envelope protein rgp21 alone was the primary pattern observed. Twenty-five percent of RA sera, 28% of Felty's syndrome sera, and 30% of large granular lymphocyte leukemia/RA sera reacted with rgp21, each significantly more than the 8% of normal sera (P less than 0.01). Removing rheumatoid factor did not abolish reactivity with rgp21 in any of six RA sera tested. Immunoreactivity to the authentic viral protein was confirmed by using purified rgp21 that was cleaved by CNBr to remove the bacterial fusion peptide, or by blocking sera with a synthetic peptide corresponding to the fusion peptide. Only one serum, from a patient with RA, showed definite evidence for prior infection with prototypic HTLV-II. These data indicate that 25% of RA sera have IgG antibodies to recombinant HTLV-I envelope protein rgp21, which is highly homologous to envelope protein gp21 of HTLV-II. These findings provide potentially novel clues regarding the pathogenesis of RA.
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PMID:Sera of patients with rheumatoid arthritis contain antibodies to recombinant human T-lymphotrophic virus type I/II envelope glycoprotein p21. 862 Jun 24


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