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1/159. Primary myeloid leukemia presenting concomitantly with primary multiple myeloma: two cases and an update of the literature.

    We report one case of primary acute myelogenous leukemia (AML) and one case of refractory anemia with excess blasts in transformation (RAEB-T) each presenting concomitantly with multiple myeloma, an unusual finding. The twin diagnoses in each patient were confirmed by cytochemical and immunohistochemical studies, and in one of our cases, by ultrastructural, flow cytometric, and molecular studies. The last three methods have not been previously used to document this phenomenon. ( info)

2/159. Spontaneous remission in myelodysplastic syndrome.

    A 73-year-old man was admitted for investigation of pancytopenia. His physical examination was unremarkable and the bone marrow aspirate was compatible with myelodysplastic syndrome (RAEB). cytogenetic analysis of the bone marrow revealed a trisomy 21. The patient received transfusions of packed red cells, and his condition remained stable for the next 7 months. He was then admitted with a chest infection and was treated with broad-spectrum antibiotics with satisfactory response. During his hospitalization there was a gradual increase in his complete blood count values, which persisted, resulting in a normal peripheral blood after 3 months. A bone marrow aspirate performed at that time revealed normal findings with no karyotypic abnormalities, indicating a spontaneous remission. The patient remained stable for the next 6 months; then he recurred with 20% blasts in his bone marrow and reappearance of trisomy 21 in 42% of the metaphases examined. Several hematologic malignancies with spontaneous remissions have been described to date, but they have generally been short and recurrence is the rule, as in the case described. The role of endogenous cytokines in triggering these spontaneous remissions is under question, as the exact mechanism is unknown. ( info)

3/159. Myelodysplastic syndrome and associated skin lesions: a review of the literature.

    The skin involvement of the myelodysplastic syndrome (MDS) can take the form of either a neoplastic infiltration or various non specific lesions. The occurrence of these lesions may be the presenting feature of the disease (MDS) or may herald its progression to acute leukemia. Recognition and early diagnosis have therapeutic and prognostic significance. ( info)

4/159. A new recurrent translocation, t(5;11)(q35;p15.5), associated with del(5q) in childhood acute myeloid leukemia. The UK Cancer cytogenetics Group (UKCCG)

    Partial deletion of the long arm of chromosome 5, del(5q), is the cytogenetic hallmark of the 5q-syndrome, a distinct subtype of myelodysplastic syndrome-refractory anemia (MDS-RA). Deletions of 5q also occur in the full spectrum of other de novo and therapy-related MDS and acute myeloid leukemia (AML) types, most often in association with other chromosome abnormalities. However, the loss of genetic material from 5q is believed to be of primary importance in the pathogenesis of all del(5q) disorders. In the present study, we performed fluorescence in situ hybridization (FISH) studies using a chromosome 5-specific whole chromosome painting probe and a 5q subtelomeric probe to determine the incidence of cryptic translocations. We studied archival fixed chromosome suspensions from 36 patients with myeloid disorders (predominantly MDS and AML) and del(5q) as the sole abnormality. In 3 AML patients studied, this identified a translocation of 5q subtelomeric sequences from the del(5q) to the short arm of an apparently normal chromosome 11. FISH with chromosome 11-specific subtelomeric probes confirmed the presence of 11p on the shortened 5q. Further FISH mapping confirmed that the 5q and 11p translocation breakpoints were the same in all 3 cases, between the nucleophosmin (NPM1) and fms-related tyrosine kinase 4 (FLT4) genes on 5q35 and the Harvey ras-1-related gene complex (HRC) and the radixin pseudogene (RDPX1) on 11p15.5. Importantly, all 3 patients with the cryptic t(5;11) were children: a total of 3 of 4 AML children studied. Two were classified as AML-M2 and the third was classified as M4. All 3 responded poorly to treatment and had short survival times, ranging from 10 to 18 months. Although del(5q) is rare in childhood AML, this study indicates that, within this subgroup, the incidence of cryptic t(5;11) may be high. It is significant that none of the 24 MDS patients studied, including 11 confirmed as having 5q-syndrome, had the translocation. Therefore, this appears to be a new nonrandom chromosomal translocation, specifically associated with childhood AML with a differentiated blast cell phenotype and the presence of a del(5q). ( info)

5/159. Fine needle aspiration of extramedullary myeloid cell tumor in myelodysplastic syndrome. A report of three cases.

    BACKGROUND: Soft tissue tumors are rare in myelodysplastic syndrome (MDS), and the role of fine needle aspiration (FNA) cytology in their diagnosis has not been explored. CASES: Two patients with refractory anemia with excess blasts in transformation (RAEB-t) developed soft tissue swellings during the course of the illness. In a third patient, soft tissue swelling was a presenting feature. The swellings in all three cases were diagnosed as extramedullary myeloid cell tumor (EMT) on FNA and showed increased blasts (10-14%), dyspoietic changes, Auer rods and monocytosis. CONCLUSION: Soft tissue tumors appearing in MDS are likely to be EMTs. FNA is therefore particularly valuable in their diagnosis as morphology, cytochemistry, immunophenotyping and flow cytometric analysis of hematopoietic cells are best studied on aspirated material. We suggest that FNA be preferred over excisional biopsy for the diagnosis of soft tissue swellings in MDS. ( info)

6/159. Smoldering acute myelogenous leukemia in the elderly.

    Out of 75 consecutive elderly AML patients who did not receive anti-leukemic treatment (52 pts) or failed to respond to differentiating agent (23 pts), 6 patients had survivals of 13.2 to 98 months with treatment restricted to supportive care. This cut-point is far longer than the median survival of the 235 elderly patients (3.5 mo.), either untreated (med. survival: 1 mo.) or treated (with treatment ranging from conventional induction to palliative chemotherapy) (4 mo.), admitted to our department within the same period of time. These cases of smoldering AML (4 women, 2 men) were all of AML2 FAB subtype (4 de novo, 2 post MDS) and presented with a significantly better performance status, lower WBC and circulating blast counts, higher platelet counts and with lower bone marrow infiltration than AML cases with more rapid progression. Cytogenetical analysis when available (3 pts) showed normal karyotypes and clonogenic assay performed in 3 of these patients showed a lack of (2 pts) or reduced in vitro leukemic cell growth (1 pt). The identification of specific characteristics of smoldering leukemia in the elderly might be an important development in the understanding of the physiopathology of acute leukemia and a tool for helping decision-making when selecting the time and intensity of cytotoxic treatment in these older patients. ( info)

7/159. Refractory anemia with ringed sideroblasts in children: two diseases with a similar phenotype?

    Three pediatric patients with refractory anemia with ringed sideroblasts (RARS) are presented. Bone marrow aspirates were examined using Romanowsky and Prussian blue iron stains in all three patients, and electron microscopic analysis was performed in one patient. All three patients had cytogenetic analysis of the bone marrow. Other studies included analysis of serum iron, total iron-binding capacity, ferritin, copper, vitamins B6 and B12, and folate levels. Antibody titers to parvovirus, hiv, and other viruses were measured. The patients had contrasting clinical courses. patients 1 and 2 had dysplastic hematopoietic features and cytogenetic findings (with either partial or one allele loss of chromosome 7), suggestive of myelodysplastic syndrome. Patient 1 experienced acute myeloid leukemia (AML) and had a good response to AML-directed therapy. Patient 2 had prolonged cytopenias and underwent bone marrow transplantation (BMT). Patient 3 had features suggestive of refractory anemia associated with mitochondrial cytopathy, including normal cytogenetics with pronounced vacuolization of marrow precursors. His anemia regressed spontaneously a few months after diagnosis. These patients represent two subgroups of pediatric RARS. patients with the myelodysplastic syndrome (MDS) type may progress to cytopenias or leukemia and may require aggressive therapy; the type is characterized by clonal cytogenetic findings. The non-MDS type, which may relate to mitochondrial cytopathy, often shows spontaneous regression and requires only supportive treatment; it has normal cytogenetic findings. ( info)

8/159. Atypical Sweet's syndrome in a neutropenic patient with acute myeloid leukemia, secondary to a RAEB-T, simulating thrombophlebitis.

    We report a rare case of a patient with acute myeloid leukemia following refractory anemia with excess of blasts transformed (RAEB-T) who presented a clinical picture suggestive of thrombophlebitis. The ultrasonographic procedure and the response to corticosteroid treatment suggest that this condition was compatible with an atypical Sweet's syndrome. ( info)

9/159. Downregulation of CD43 in RAEB and RAEB-T patients. Report of 3 cases.

    CD43 (leukosialin, sialophorin) is a cell surface mucin expressed at high levels on most leukocytes and is reported to be involved in adhesion, anti-adhesion, and signal transduction prodders. Regulation of its expression is thought to take place through methylation of the dna in the nonproducing cells, and the methylation inhibitor 5-azacytidine induces expression of the sialophorin gene. Here we report three cases of patients with myelodysplastic syndromes in which acquired severe deficiency of the CD43 antigen on the surface of most hemopoietic cells was observed. Peripheral blood mononuclear (PBMC) cells from 32 MDS patients and 20 healthy individuals were analyzed by flow cytometry after labeling with an anti-CD43 (DF-T1) monoclonal antibody. In 1 patient with refractory anemia with excess of blasts (RAEB) and 2 patients with refractory anemia with excess of blasts in transformation (RAEB-t), the percentages of CD43( ) PBMC were 3.8%, 6%, and 9.9%, respectively. The deficiency was observed at protein and rna level as confirmed by western and southern blot, while analysis of the dna by single-strand conformation polymorphism and sequencing did not reveal any difference in the gene sequence between the CD43( ) and CD43(-) cells of these patients. It is known that patients with MDS may have normal and dysplastic population of hemopoietic cells. Further studies are needed to reveal the mechanism of downregulation of the gene in these 3 patients and whether the phenomenon is related to the dysplastic population only or not. ( info)

10/159. Monocyte deactivation and its reversal in a patient with chemotherapy-induced leukopenia and severe systemic infection.

    BACKGROUND: Serious infections constitute a major problem for patients with cancer, and new approaches must be found in dealing with these. The pathophysiology of neutropenic infection is not well understood, although there is some evidence that, as in sepsis in the primarily immunocompetent host, a pro- and an antiinflammatory phase can be discriminated. In the recent literature is described a series of nonneutropenic patients with sepsis in whom interferon-gamma was successfully administered during the immunoparalytic phase, a concept that might possibly be extended to immunocompromised hosts. PROCEDURE: A 14-year-old patient with RAEB-T/hypoplastic M2 and chemothera py-induced neutropenia developed a severe infection and continued to deteriorate clinically despite maximum supportive measures, including broad antibacterial and antifungal coverage. On the basis of monocyte de-activation this patient was considered to be in the immunoparalytic phase of sepsis and consequently treated with 60 microg/m(2) of interferon-gamma per day for 10 days. RESULTS: The patient made a rapid clinical recovery, and biochemical markers of infection improved promptly. At the same time, the fraction of activated monocytes normalized rapidly and stably. We hypothesize that treatment with interferon-gamma effected this rapid restoral of monocyte activation and that monocyte reactivation might have contributed to the patient's prompt recovery from his severe infection. interferon-gamma treatment was well tolerated. CONCLUSIONS: Immunostimulation with interferon-gamma might prove to be a valuable adjuvant treatment for patients with chemotherapy-induced neutropenia during the rare scenario of infection with immunoparalysis. ( info)
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