1/46. Chronic granulocytic leukemia, neutrophilic type, with paraproteinemia (IgA type K).A patient with chronic granulocytic leukemia, neutrophilic type, was followed for 28 months. A paraproteinemia, IgA type K, and Bence Jones proteinuria (K) appeared without prior chemotherapy with alkylating agents.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/46. Cryocrystalglobulinemia in hairy cell leukemia.A patient with hairy cell leukemia (leukemic reticuloendotheliosis) was noted to have a spurious leukocytosis caused by a spontaneously crystallizing cryoglobulin. The cryoprotein was identified as IgG lambda. An intracytoplasmic immunoglobulin demonstrable within the hairy cell was also IgG lambda. The cryoglobulin spontaneously disappeared over a four day period. A reliable automated count on the Coulter Model S could be obtained by prewarming the blood specimen to 37 C.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
3/46. Acquired von Willebrand disease in monoclonal gammapathies: effectiveness of high-dose intravenous gamma globulin.The reported underlying lymphoproliferative disorders associated with acquired von Willebrand disease (AvWD) include benign monoclonal gammapathy, multiple myeloma, Waldenstrom disease, chronic lymphocytic leukemia, non-Hodgkin lymphoma, and hairy cell leukemia. The AvWD in patients with a monoclonal gammapathy and/or a lymphoproliferative disorder is featured by a prolonged bleeding time, normal platelet count, and a decreased or absent ristocetine-induced platelet aggregation in combination with a prolonged aPTT and normal PT due to low levels of factor viii/von willebrand factor (vWF) parameters in the absence of a factor VIII inhibitor in the Bethesda assay. in vitro and vivo experiments consistently showed that the anti-vWF autoantibodies in monoclonal gammapathies cause a rapid clearance of the factor viii/vWF complex from the circulation after DDAVP and factor viii/vWF concentrate infusion. Multimeric analysis of the vWF usually show a type II-like AvWD due to the absence of large vWF multimers as the consequence of the rapid clearance of the anti-vWF-factor viii/vWF complex from the circulation. There is a poor response to intravenous DDAVP and factor viii/vWF concentrate infusion, but high dose intravenous gamma globulin (1 g/kg for 2 days) usually induces a transient correction of the factor viii/vWF parameters for 1 to a few weeks.- - - - - - - - - - ranking = 0.4keywords = leukemia (Clic here for more details about this article) |
4/46. A case of monoclonal gammopathy associated with acute myelomonocytic leukemia with eosinophilia suggested to be the result of lineage infidelity.Acute myelomonocytic leukemia (AMMoL) accompanied by monoclonal gammopathy is a rare condition, and its pathogenesis and the cytogenetic mechanism of such leukemogenesis have not been determined in detail. A case of AMMoL with eosinophilia accompanied by immunoglobulin g kappa monoclonal gammopathy is described. Immunophenotypic studies of the peripheral blood and bone marrow mononuclear cells revealed no evidence of abnormally proliferating cells of B-lineage. dna analyses of bone marrow mononuclear cells containing leukemic cells revealed rearrangement of the kappa-light chain (Igkappa) gene and c-myc and c-jun proto-oncogenes. The intensities of the rearranged bands for these genes on Southern blot analysis suggested the existence of a major population of leukemic cells with rearranged Igkappa gene and minor population(s) of leukemic cells with rearranged c-myc and/or c-jun proto-oncogene(s) in the patient's bone marrow and indicated the occurrence of genetic evolutionary changes in leukemic cells in this patient before starting chemotherapy. These results suggest that these leukemic cells are the most likely candidate for immunoglobulin g kappa monoclonal protein production, and structural abnormalities of c-myc and c-jun proto-oncogenes may have contributed to the evolution of leukemic cells in this patient.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
5/46. T-ALL with monoclonal gammopathy and hairy cell features.The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL) with hairy cell (HC) features and monoclonal gammopathy. The disease process had an acute onset and followed a rapid, progressive course. The patient had minimal splenomegaly and bicytopenia, but the bone marrow displayed increased numbers of reticulin fibers. The blasts were positive for tartrate-resistant acid phosphatase (TRAP) and CD11c. Molecular analysis revealed rearrangement of immunoglobulin heavy chain genes and a rearranged T-cell receptor (TcRbeta) beta gene. The patient responded to conventional ALL therapy. Acute T-cell ALL with HC features in childhood has not been reported previously, either alone or in association with monoclonal gammopathy. We propose "T-ALL with hairy cell features" to describe this case.- - - - - - - - - - ranking = 0.2keywords = leukemia (Clic here for more details about this article) |
6/46. Karyotypic abnormalities and clinical aspects of patients with multiple myeloma and related paraproteinemic disorders.Karyotypic abnormalities were detected in the malignant cells of 6 of 18 patients with multiple myeloma (MM). Six patients with benign monoclonal gammopathy, one with amyloidosis of immunoglobulin origin, and two with Waldenstrom's macroglobulinemia had normal karyotypes. All six MM patients with aneuploidy were in a group of 10 patients in an accelerated or relapse phase of their disease and four had high serum paraprotein levels (7.92, 6.24, 6.80, and 4.24 g/dl, respectively) when their abnormal karyotypes were detected. Five of the 6 MM patients with aneuploidy had received prior chemotherapy. aneuploidy was not observed in 8 stable MM patients. Abnormalities of chromosome 14 were present in all 6 patients, with a 14q marker in 5 and loss of No. 14 in 1. A translocation between Nos. 11 and 14 was found in aneuploid cells of 2 patients who had plasma cell leukemia (PCL). However, the break point in the long arm of No. 11 differed in the 2 patients. A gain of Nos. 5, 9, and 11 was seen in 3 patients, a gain of No 1 in 2, and rearrangements of No. 1 in 5 MM patients, including all 4 who had a 14q marker chromosome initially. A deletion of chromosome 6 at band q25 was detected in 2 MM patients and a pericentric inversion of No. 6 (6p21 to 6q13) was seen in the patient with PCL. Three of 4 MM patients had a nonrandom loss of one chromosome 8. Two other MM patients, who were treated with melphalan and prednisone, developed acute nonlymphocytic leukemia (ANLL) 2 and 4 years after the diagnosis of MM. Marrow cells of one patient showed a 5q- chromosome and a constitutional translocation involving Nos. 13 and 14 during the preleukemic stage; during the leukemic phase, the karyotype evolved to 50 chromosomes including extra chromosomes 1, 6, 8, 10, and 21 and a missing 7, in addition to the originally detected 5q- and the 13/14 translocation. The peripheral blood from the other patient was hypodiploid, with a missing chromosome 7 and a translocation between 3q and 9p. These patterns of chromosome change resemble those of ANLL rather than MM and are similar to the changes seen in ANLL after treated malignant lymphoma.- - - - - - - - - - ranking = 0.4keywords = leukemia (Clic here for more details about this article) |
7/46. Chronic myelomonocytic leukemia with paraproteinemia but no detectable plasmacytosis: a detailed cytological and immunological study.A patient with chronic myelomonocytic leukemia with IgG K paraproteinemia, but no detectable plasmacytosis, is described. The patient was entering a blastic phase at the time of the most detailed studies. Cytological, cytochemical, and ultrastructural studies revealed a mixed myeloid proliferation with granulocytic forms predominating over monocytic elements. A variety of ultrastructural abnormalities, including defective granulation, was observed but no cells with highly developed rough endoplasmic reticulum were observed. Immunological marker studies showed that the mature myeloid cells possessed receptors for the Fc of IgG and weakly expressed the Ia-like P29/34 antigen. The mature myeloid cells also expressed both surface and intracytoplasmic Ig restricted to IgG K, and this IgG K persisted after 4 weeks in culture. A reverse plaque assay showed that the myeloid cells were capable of releasing IgG K in vitro, but studies involving the incorporation of radio-labeled amino acids showed no detectable Ig production by the myeloid cells. The possible interpretations of these data are discussed in some detail in relation to previous reports of paraproteinemia in myeloid proliferative disorders.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
8/46. An unusual presentation of plasma cell dyscrasias: cardiac tamponade due to myelomatous infiltration.Pericardial involvement, a rare complication of multiple myeloma (MM), is caused by amyloidosis, infections, bleeding abnormalities or plasma cell infiltration, usually at a late or terminal stage of the disease. Three cases of MM with pericardial involvement are reported here and discussed in the light of current literature. In a retrospective review of all patients with MM at two institutions, three cases of pericardial involvement were identified. In one case, we were able to obtain cytospin preparations of the pericardiocentesis fluid. In the remaining two patients, the pericardial biopsy specimen was obtained via a pericardial window. All patients had progressive dyspnea and signs of pericardial tamponade. The pericardiocentesis fluid showed infiltration with plasma cells in one of the three patients, who had a progressive and fatal course. In the second patient pericardial invasion was proven by biopsy and the third was diagnosed with a plasma cell leukemia but developed a pericardial effusion demonstrated by pericardial biopsy. All these three patients died of progressive disease without any response to chemotherapy and supportive measures. In conclusion, optimal treatment for malignant involvement of the pericardium by myeloma cells has not yet been established and is often fatal.- - - - - - - - - - ranking = 0.2keywords = leukemia (Clic here for more details about this article) |
9/46. Development of a transient monoclonal gammopathy after chemotherapy in a patient with biphenotypic acute leukemia.The development of a IgGk monoclonal gammopathy after a phase of bone marrow aplasia following chemotherapy is described in a patient suffering from biphenotypic acute leukemia (BAL). Paraprotein was followed by the relapse of the disease and disappeared during a further chemotherapy. Paraprotein could have been caused by an additional chemotherapy-induced genetic mutation or by a dysfunction in T-B cooperation observed in the phase of reconstitution of the immune system after medullar aplasia.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
10/46. Simultaneous occurrence of monoclonal gammopathy and acute secondary leukemia with overexpression of p-glycoprotein.A 52-year-old woman, previously treated with chemo- and radiotherapy for Hodgkin's disease, developed an acute non-lymphoid leukemia and, contemporarily, an IgG-kappa paraproteinemia. cytogenetic analysis showed a major clone, representing 90% of observed metaphases, with monosomy of chromosomes 5 and 14. In addition, leukemic cells exhibited a high expression of the p-glycoprotein, a transmembrane glycoprotein involved in the multidrug-resistance mechanism. Possible explanations for this cluster of findings are provided.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
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