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)

We observed a case of hairy cell leukaemia without splenomegaly and without hairy cells in blood that was difficult to diagnose. Typical hairy cells with cytoplasmic projections were found in hypocellular bone marrow smears rarely whereas atypical mononuclear cells of hairy cell type constituted 70% and 1.5% of them exhibited ring-shaped nuclei. These cells were in 8% positive for tartrate-resistant acid phosphatase. Bone marrow biopsy revealed diffuse infiltration with hairy cells. We reviewed bone marrow smears of 11 other patients with hairy cell leukaemia and we found hairy cells with ring-shaped nuclei in one of them only with a frequency of one promile.
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PMID:[A diagnostically difficult case of hairy cell leukemia with ring-shaped nuclei]. 204 40

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

From a total number of 221 patients with leukaemic lymphoproliferative syndromes studied at the Hospital dos Capuchos, in Lisbon, seven patients whose cell morphology differed from that of "classical" lymphoproliferative syndromes were separated; marked splenomegaly without lymph node enlargement was present in all of them. Immunophenotypic studies confirmed B-cell origin of the lymphoproliferation in the seven patients. Small lymphocytes with mature appearance predominated in three of these cases, presenting as: a) the only cell population (with immunophenotype RR+, FMC7-, CD5+), b) along with a significant amount of prolymphocytes (RR+, CD5+, FMC7+), c) accompanying a population of cells with lymphoplasmacytoid differentiation (RR-, CD5-, CD38+, associated to serum monoclonal IgM). Those data strongly suggested that these three lymphoproliferative syndromes corresponded, respectively, to a classic B-cell chronic lymphocytic leukaemia, a chronic lymphocytic leukaemia with prolymphocytes, and an immunocytoma. In three other cases the morphology of the proliferating cells was intermediate between prolymphocytes and hairy cells (i.e., variant hairy cells) and they strongly reacted with monoclonal antibodies FMC7 and LeuM5 (CD11c), showing low positivity with antigens CD5 and CD25, in the absence of receptors for mouse red blood cells. The remaining B-cell lymphoproliferative syndrome studied had small centrocytes in peripheral blood, their phenotype being RR-, CD5+, FMC7+/-, CD10+ and CD38+, which suggested a centrofollicular lymphoma with leukaemic expression. In summary, the present study seems to confirm the heterogeneity of the chronic lymphoproliferative syndromes showing splenomegaly as an outstanding clinical feature. Immunophenotype along with cell morphology are important in the differential diagnosis, especially whenever splenectomy cannot be carried out, in order to choose the appropriate therapy.
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PMID:[Usefulness of the immunologic phenotype in the diagnosis of chronic lymphoproliferative syndromes with splenomegaly as the dominant clinical manifestation]. 229 Nov 46

A 43-year-old man with hairy-cell leukemia and marked splenomegaly developed severe hypophosphatemia which improved after splenectomy. Since splenic tissue phosphorus was significantly elevated, and since serum phosphorus returned to normal levels immediately after the operation, it is postulated that excessive uptake of phosphorus by the rapidly dividing leukemic cells might have caused the transient decrease in serum phosphorus.
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PMID:[Transient hypophosphatemia associated with hairy-cell leukemia]. 231 8

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

A 55-year-old woman was first seen in October 1986, because of splenomegaly, moderate anemia and leukocytosis. The hemoglobin was 8.8 g/dl, platelet count 24.4 X 10(4)/microliters, and the white cell count 23,800/microliters with 73% atypical lymphoid cells. The bone marrow nucleated cell count was 99,000/microliters with 36% lymphoid cells. These atypical lymphoid cells showed hairy appearance under phase-contrast microscopy, and were positive for tartrate-resistant acid phosphatase. These cells showed the surface phenotype of CD10, CD19, CD20, Leu M5, HCM, and IgG K. Biochemical data revealed marked polyclonal hypergammagloburinemia (PHG) of IgG type (IgG 8756 mg/dl). To elucidate the mechanism of the PHG, we investigated whether hairy cells produce interleukin 6 (IL-6) and express IL-6 receptor. The culture supernatant of these hairy cells increased 3H-thymidine uptake of a IL-6 dependent hybridoma clone (MH60) in a dose-dependent manner. These cells were stained with anti-IL-6 antibody using immuno-cytochemical technique. Our results suggested that these hairy cells produce and secrete IL-6. Immunocytochemical staining with anti IL-6 receptor antibody and the binding assay with 125I-labelled recombinant IL-6 revealed that these cells express little or no receptors for IL-6. It was therefore suggested that IL-6 produced by hairy cells in this case is not an autocrine growth factor for these cells but may play a role in development of PHG by stimulating normal B lymphocytes to produce an excessive amount of immunoglobulin.
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PMID:[Production and secretion of BSF2/IL6 in a case of hairy cell leukemia with polyclonal hypergammaglobulinemia]. 238 10

A total of 5 patients with a clinicohematological picture resembling hairy-cell leukemia (HCL) have been described. However, the morphological features of leukemic lymphocytes, the absence of acid phosphatase in them, the nodular character of the bone marrow lesion combined with an unusual phenotype have permitted the authors to distinguish these cases as a separate variant of B-cell malignant lymphoma. Paraprotein (M-class) was detected in the blood of two of the patients. A conclusion has been made that the combination of splenomegaly with hairy lymphocytes in the blood is characteristic of not only HCL, but it can be also observed in different variants of malignant lymphoma.
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PMID:[Malignant lymphoma with "hairy" lymphocytes in the blood and splenomegaly--a new variant of lymphoproliferative diseases]. 239 62

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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