1/12. Massive immune haemolysis after allogeneic peripheral blood stem cell transplantation with minor ABO incompatibility.Immune haemolysis as a result of minor ABO incompatibility is an underappreciated complication of haematopoietic transplantation. The increased lymphoid content of peripheral blood stem cell (PBSC) transplants may increase the incidence and severity of this event. We observed massive immune haemolysis in 3 out of 10 consecutive patients undergoing HLA-identical, related-donor PBSC transplants with minor ABO incompatibility. Non-ablative conditioning had been given in 9 of these 10 cases, including two with haemolysis. Cyclosporin alone was used as prophylaxis against graft-vs.-host disease (GVHD). Catastrophic haemolysis of 78% of the circulating red cell mass led to anoxic death in the first case seen, but severe consequences were avoided by early, vigorous donor-compatible red cell transfusions in the subsequent two cases. Haemolysis began 7-11 d after PBSC infusion and all patients with haemolysis had a positive direct antiglobulin test (DAT), with eluate reactivity against the relevant recipient antigen. However, neither the intensity of the DAT, the donor isohaemagglutinin titre, nor other factors could reliably be used to predict the occurrence of haemolysis. Our data indicate that haemolysis may be frequent and severe after transplantation of minor ABO-incompatible PBSCs when utilizing cyclosporin alone to prevent GVHD. Meticulous clinical monitoring and early, vigorous donor-compatible red cell transfusions should be practiced in all instances.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/12. Pneumococcal arthritis affects performance status in patients with chronic GVHD of the skin following allogeneic bone marrow transplantation.We encountered 2 patients with pneumococcal arthritis following bone marrow transplantation (BMT). Both patients received grafts from unrelated human lymphocyte antigen (HLA)-matched donors and had suffered from chronic graft-versus-host disease (GVHD). One, a 10-year-old boy, suffered from Epstein-Barr virus-related lymphoproliferative disease (EB-LPD) and received oral 6-mercaptopurine and methotrexate to manage lymphadenopathy. Twenty-four months after BMT and 7 months after the onset of EB-LPD, pneumococcal arthritis occurred in both knee joints. The other patient, a 10-year-old girl, received multiagent immunosuppressive therapy for her chronic GVHD. At 51 months following BMT, pneumococcal arthritis occurred in her left knee joint. Chronic GVHD of the skin delayed the recovery from the arthritis in both patients. This complication is quite rare but can be very serious, in regard to the patient's performance status following BMT. Although vaccination against pneumococcus or preventive antibiotics should be administered to high-risk patients, early diagnosis and treatment may be the best strategy for pneumococcal arthritis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/12. Atypical generalized eruptive histiocytosis associated with acute monocytic leukemia.Histiocytoses are diseases caused by proliferation of either dendritic cells/langerhans cells or of monocytes/macrophages. Generalized eruptive histiocytosis belongs to the cutaneous non-Langerhans cell histiocytoses and is a rare monocyte-macrophage proliferative disorder that usually follows a benign clinical course. We present the case of a 59-year-old man who presented with a 7-month history of progressively developing erythematous macules and slightly elevated papules widely distributed over the trunk, neck, face, and thighs. Ultrastructurally, no Birbeck granules were observed, and immunochemistry did not reveal any S-100 protein or CD1a antigen in any of the lesional cells, excluding Langerhans cell histiocytosis. In addition, the histiocytic infiltrate in the skin of our patient was shown to strongly express MS-1 high molecular weight protein, a marker described as highly characteristic for cutaneous non-Langerhans cell histiocytoses. Bone-marrow smear examination and flow cytometric analysis revealed monocytic leukemia. This is the second report of generalized eruptive histiocytosis associated with acute monocytic leukemia. We discuss the differential diagnoses of the clinical picture and stress that this benign cutaneous disorder may indicate an underlying hematologic malignancy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/12. Bone marrow granulomas in acute myeloid leukaemia following interleukin 2 and lymphokine-activated killer cells.Granulomas are thought to represent an immune reaction to antigenic stimulation. Bone marrow granulomas are uncommon, but in the following case report we show their transient appearance after interleukin 2 (IL-2) and autologous lymphokine-activated killer cell therapy. This is a previously unreported association, and may implicate IL-2 in the pathogenesis of granulomas.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/12. Simultaneous occurrence of myelomonocytic leukemia and multiple myeloma: involvement of common leukemic progenitors and their developmental abnormality of "lineage infidelity".We investigated the origin of leukemic progenitors in a case of the simultaneous occurrence of myelomonocytic leukemia and multiple myeloma (IgG-kappa). At presentation, myeloperoxidase and nonspecific esterase-positive myelomonocytic cells had proliferated up to 12.2 x 10(9)/liter in the peripheral blood. Bone marrow cell differentials revealed the coexistence of myelomonocytic cells (30%) and atypical plasmacytoid cells (26%). Myelomonocytic cells in peripheral blood expressed both myeloid antigens (CD11b, CD13, CD14, CD15, CD33) and T/B-lymphoid antigens (CD2, CD4, CD5, CD7, CD10, PCA-1). Bone marrow mononuclear cells (BMMC) could be divided into PCA-1 strongly positive and PCA-1 weakly positive populations, which were considered to represent myeloma cells and myelomonocytic cells, respectively; the former were CD2-positive (CD2 ), CD14-, and CD15-, whereas the latter were CD2 , CD14 , and CD15 . Immunohistochemical analysis revealed that, in addition to plasmacytoid cells, a minority of myelomonocytic cells showed a positive reaction for IgG staining, and production of IgG was observed in the culture supernatant of CD14 myelomonocytic cells in peripheral blood. Southern blot analysis revealed the presence of two identical rearrangement bands of immunoglobulin heavy chain gene in both BMMC containing myeloma cells and myelomonocytic cells and CD14 myelomonocytic cells in peripheral blood. In a long-term methylcellulose assay, peripheral blood mononuclear cells produced large compact colonies consisting of macrophages and IgG plasmacytoid cells (M phi/P colonies), while BMMC produced a different type of colonies consisting of CD14 myelomonoblasts, macrophages, and IgG plasma cells (Mb/M phi/P colonies) in addition to M phi/P colonies. Recloning experiments showed that primary Mb/M phi/P colonies gave rise to both secondary M phi/P and Mb/M phi/P colonies. These observations strongly suggest that common leukemic progenitors provide both myeloma and myelomonocytic leukemia cells, and the mechanism of "lineage infidelity" is probably involved in the development of their "bilineal" differentiation.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/12. Treatment of refractory CMV-infection following hematopoietic stem cell transplantation with the combination of foscarnet and leflunomide.BACKGROUND: Treatment of cytomegalovirus (CMV) disease after allogeneic hematopoietic stem cell transplantation (HSCT) is limited by toxicities of current antiviral drugs and the occurrence of drug resistant strains. Leflunomide, an immunosuppressive agent used for treatment of rheumatoid arthritis, also has activity against CMV by impairing viral assembly. Here we report the control of refractory CMV disease by the combined use of foscarnet and leflunomide. patients AND RESULTS: A 1S-year-old boy with juvenile myelo-monocytic leukemia (JMML) received an allogeneic HSCT with bone marrow stem cells from a mismatched, unrelated donor (MMUD, recipient and donor CMV-positive). CMV-reactivation two months post transplantation (Tx) could only be controlled by the use of cidofovir. Because of secondary graft failure, the boy received a second HSCT with peripheral blood stem cells (PBSC) of the same donor after overall 6 months. CMV-infection was noticed three weeks later, associated with a considerable rise of both CMV-copy number and pp65-antigen. Since reinduction with cidofovir was ineffective and ganciclovir not warranted due to the history of graft failure, the child then received a combination of foscarnet/leflunomide, leading to a rapid decline of his CMV-copy number and to an afebrile state. Hematological, hepatic or renal toxicities were not observed. CONCLUSION: This case report suggests that leflunomide may be of use in the management of transplant recipients with CMV-infection refractory or intolerant to conventional antiviral therapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/12. Dysmegakaryocytopoiesis and thrombocytosis in a patient with acute myelomonocytic leukemia and long evolution.A patient with acute myelomonocytic leukemia (M4), dysmegakaryocytopoiesis and thrombocytosis is presented. immunophenotyping and blast colony assay showed the presence of blasts with IaDr, CD 33 and CD 14 antigens. cytogenetic analysis and level of thrombopoietin were normal. This atypical case represents yet another example of the transitional zone between atypical myeloproliferative disease and acute leukemia, and the apparent absence of any cytogenetic abnormality is noteworthy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/12. Relationship between acute myelomonoblastic leukemia and infection due to human immunodeficiency virus.infection due to the human immunodeficiency virus (hiv) has been complicated by the development of acute nonlymphocytic leukemia in five patients whose cases have previously been reported; other manifestations, including preleukemia, myelofibrosis, and myeloid hyperplasia, have also been reported in patients infected with hiv. We report the sixth case of an hiv-infected patient who developed acute myelomonocytic leukemia; hiv infection was documented by tests for serum antibodies (enzyme-linked immunosorbent assay and western blotting), by a markedly elevated p24 antigen level in plasma, and by cultures of CSF and peripheral blood that were positive for hiv. Furthermore, myelomonoblasts that were cultured without the addition of growth factors displayed evidence of hiv replication through the presence of p24 antigen and reverse transcriptase activity, both of which lasted for 4 weeks in the supernatant fluid of the cell cultures. This case report provides the first data indicating that hiv may infect myelomonoblasts in vivo and represents the sixth reported case of an association between hiv infection and pure acute nonlymphocytic leukemia.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/12. Immunophenotypic, cytogenetic and molecular investigations in two cases of CALLA positive acute myeloid leukemia.Clinicopathological and cytogenetic features of two patients with acute myelogenous leukemia (AML) whose blast cells coexpressed myeloid-associated antigens and CALLA are described. Leukemia cells revealed myelomonocytic (FAB-M4) and monocytic (FAB-M5) features, while the nonblast cell population exhibited trilineage myelodysplasia in both cases, a finding suggestive of multiple-cell-lineage involvement. Cytogenetically, a deletion of the long arm of chromosome 6 was found in one patient, and normal metaphases were detected in the other. Molecular studies disclosed a rearrangement of the IgH locus in one patient. Clinically, these patients were unresponsive to antimyeloid regimens including daunorubicin and cytarabine, two agents normally also effective on lymphoblastic leukemias, possibly indicating the need for alternative protocols for the treatment of CALLA positive AML.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/12. Acute myelomonocytic leukemia in a patient with multiple myeloma: evidence for different clonal origin.A case of 77-year-old female with multiple myeloma (IgG-k) developed acute myelomonocytic leukemia (AMMoL) following a myelodysplastic stage after chemotherapy with melphalancyclophosphamide combinations for 6 years. The leukemic blast cells expressed both myeloid antigens (CD11b, CD13, CD14, CD15, CD33 and CD34) and T/B lymphoid antigens (CD2, CD4, CD22 and PCA1). cytogenetic analysis revealed a chromosome deletion -7. Analysis of immunoglobulin genes showed the heavy chain genes in germ line configuration. These findings indicate that the AMMoL was a therapy-related stem cell leukemia and was a clonal origin genetically different from multiple myeloma irrespective of plasma cell phenotype.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
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