1/34. Screening for azathioprine toxicity: a pharmacoeconomic analysis based on a target case.The risk of azathioprine-induced myelosuppression can be predicted by detecting patients with intermediate or low thiopurine methyltransferase (TPMT) activity. population studies have shown that 89% of whites have high TPMT activity, 11% have intermediate TPMT activity, and 0.3% have low TPMT activity. Three specific mutations in the TPMT gene that cause decreased TPMT activity have recently been identified. patients homozygous for the TPMT mutations have low TPMT activity, and patients heterozygous for TPMT mutations have intermediate TPMT activity. This has led to the development of a technique for TPMT genotype analysis that will identify patients at risk of azathioprine-induced myelosuppression. We report a case of a patient with bullous pemphigoid who experienced azathioprine-induced myelosuppression and who was later found to be homozygous for TPMT mutant alleles. Using the cost of treatment required for this patient and the estimated population prevalence of TPMT mutations, we examined the cost impact of screening for TPMT mutations in all patients being considered for azathioprine therapy. We calculated that screening is cost-neutral assuming patients homozygous for TPMT mutations experience myelosuppression, and that it is cost-beneficial assuming patients heterozygous for TPMT mutations also experience myelosuppression while receiving azathioprine. Screening patients for TPMT mutations will reduce the risk of azathioprine-induced myelosuppression, and our study suggests that it may also be a cost-attractive strategy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/34. Complete haematological and cytogenetic response to interferon alpha-2a of a myeloproliferative disorder with eosinophilia associated with a unique t(4;7) aberration.A female patient with eosinophilia and cardiac symptoms was found to have a unique chromosomal aberration [t(4;7)(q11;p13)] of bone-marrow precursors. The disorder was classified as a chronic myeloproliferative syndrome with eosinophilia. Due to a significant increase in the white blood cell and eosinophil count during initial treatment with prednisone and hydroxyurea, Interferon alpha-2a was administered at a dose of 3-5 x 10(6) I.U. s.c., five times per week, and induced a long-term complete haematological and cytogenetic response. The clinical features of this case are presented and discussed in the context of the current literature.- - - - - - - - - - ranking = 239.21931223425keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
3/34. Immunophenotype of a transient myeloproliferative disorder in a newborn with trisomy 21.Cytologic, immunologic, and cytogenetic studies were performed on the blast cells of a newborn with down syndrome and transient myeloproliferative disease. This hematologic disorder is uncommon, and occurs primarily in infants with down syndrome. This boy presented with a high white blood cell count and a high percentage of blast cells, without anemia or thrombocytopenia. Chromosome analysis showed a constitutional trisomy 21 without any other clonal abnormality. A three-color flow cytometric analysis was performed and revealed two different CD45 dim, CD34( ), CD117( ), CD56( ) immature subpopulations: the normal immature myeloid precursor and an immature blast cell population that expressed CD41, CD42, CD61, CD36, CD13, CD1a, and CD2. We postulate that this population could be the leukemic precursor involved in the acute megakaryoblastic leukemia frequently observed in children with down syndrome.- - - - - - - - - - ranking = 239.21931223425keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
4/34. A review of myeloproliferative disease with presentation in the head and neck region.The diagnosis of an essential thrombocytosis is demonstrated in this presentation of a well-looking 53 year old man who had a five-year history of increasing facial asymmetry as evidenced by deviation of his mandible to the right and malocclusion. The enlarged mandibular condyle was the first manifestation of his underlying myeloproliferative disorder. His management will be discussed. Neoplastic diseases of the multipotent haematopoietic stem cells result in four major diseases: chronic myelogenous leukaemia (CML); polycythaemia vera (PV); agnogenic myeloid metaplasia with myelofibrosis (AMM/MF); essential thrombocytosis (ET). CML: demonstrates increased production of neutrophils and marked splenomegaly. It is divided into a chronic phrase typified by hyperplasia of mature bone marrow elements and a blastic or acute phase which evolves into a proliferation of immature marrow elements and can develop into acute myelogenous leukaemia. PV: associated with increased production of all myeloid cells but dominated by increased red blood cells with splenomegaly. AMM/MF: allows the neoplastic stem cells to proliferate and lodge in multiple sites outside the bone marrow. splenomegaly and fibrosis of marrow spaces also occurs. ET: resulting in a markedly elevated platelet count in the absence of a recognizable stimulus. Treatment revolves around measures to maintain hydration, to relieve arthralgias, to prevent thrombotic episodes, and to prevent infections.- - - - - - - - - - ranking = 35.437855357318keywords = blood cell (Clic here for more details about this article) |
5/34. Transient myeloproliferative disorder with erythroid differentiation in down syndrome.A newborn with a karyotype of 47, XY, 21 presented at birth with a white blood cell count of 27 700/microL of which 61% were blast cells. The blast cell morphologic structure was initially not characteristic of any particular lineage, although the cytoplasm contained fine granules and occasional small vacuoles. Routine cytochemical stains were negative, except one for nonspecific esterase that was faintly positive in most of the blast cells. Flow cytometric analyses showed that the blast cells expressed glycophorin A with a subset dimly coexpressing CD45 and were negative for CD34, CD71, myeloid, lymphoid, and platelet-associated antigens. These immunophenotypic findings were consistent with an abnormal erythroid phenotype. A few days postpartum, markedly dysplastic erythroid precursor cells appeared in the peripheral blood and increased in number as the early blast cells decreased. After a period of subdued blast cell production, a second wave of increase in the number of blast cells and dysplastic erythroblasts followed and ended with the disappearance of circulating abnormal cells. The child is now 5 years old and no major illness has been reported since the remission of this disorder. This case most likely belongs to the category of transient myeloproliferative disorders, although the erythroid-like phenotype of blast cells and the evidence of single-lineage maturation to circulating dysplastic erythroid precursors allow the suggestion that this process could represent a special form of a self-limited hematologic disorder in down syndrome.- - - - - - - - - - ranking = 239.21931223425keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
6/34. Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper-IgE: clinical, immunological and cytogenetic features and therapeutic approach. A case report.A 23-year-old, white male metallurgist presented with pruritic erythematous maculo-papules over the trunk and upper limbs and 6 months later developed erythroderma, eosinophilia and multi-organ dysfunction. A diagnosis of chronic eosinophilic leukaemia was made on the basis of myeloproliferative involvement of both peripheral blood and bone marrow, associated with eosinophilic differentiation and a t(5;12)(q33;p13) translocation. The initial therapeutic approach was interferon alfa-2b plus cytosine arabinoside, for 13 months, followed by hydroxyurea plus vincristine. There was improvement of skin lesions, disappearance of eosinophilia and decrease of serum immunoglobulin e, towards normal values.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/34. light and electron microscopic studies of the bone marrow and blood cells in chronic panmyelosis including polycythemia vera and primary thrombocythemia.Both bone marrow and peripheral blood was investigated light and electron microscopically in 3 cases with polycythemia vera, 2 cases with primary thrombocythemia and 1 case with panmyelosis. In 5 cases the peripheral blood showed persistent increase in cells of two or three hematopoietic systems. Giant thrombocytes in the peripheral blood were seen in 3 cases. erythroblasts, granulocytic young forms, and megakaryocytes were often observed in the blood. Histologic bone marrow examination showed prominent proliferation of all 3 hematopoietic cells in every case. Cytological and electron microscopical examinations of the bone marrow revealed many mitotic figures, morphological abnormalities, and unbalanced nucleocytoplasmic maturation in various hematopoietic cells. These findings suggested that the proliferation of all 3 hematopoietic cells in the bone marrow was not simply reactive in nature, but an idiopathic progressive process. It is considered that these disorders and primary myelosclerosis represent no separate entities and must be unified as "chronic panmyelosis".- - - - - - - - - - ranking = 141.75142142927keywords = blood cell (Clic here for more details about this article) |
8/34. Pure red cell aplasia developing into myeloproliferation with myelodysplasia and subsequent leukemia after cyclosporin A therapy.We describe a very rare case of a patient who presented with red cell aplasia that later developed into myeloproliferation with myelodysplasia and eventually leukemia. A 63-year-old man presented with anemia and reticulocytopenia in May 1997. A bone marrow examination revealed erythroid aplasia with normal production of myeloid cells and megakaryocytes with a normal karyotype. After the diagnosis of pure red cell aplasia was made, the patient was treated with prednisolone and then with cyclosporin A (CyA). Two weeks after the initiation of CyA treatment, the peripheral reticulocyte count began to increase with a regrowth of erythroid cells in the bone marrow. Meanwhile, the peripheral white blood cell and platelet counts also increased to more than 10,000/microL and 1,000,000/microL, respectively. Examination of a bone marrow aspirate in December 1997 revealed myelodysplastic changes with trisomy 8. Despite the discontinuation of CyA and the administration of 1-beta-D-arabinofuranosylcytosine stearyl monophosphate, leukemia developed in August 1998. In September 1998, the patient died of sepsis during a neutropenic period that followed remission-induction therapy. In the mechanism of pathogenesis, CyA may induce upon pure red cell aplasia a secondary myeloproliferative disorder with myelodysplasia and leukemia. An alternative possibility is that CyA reduces autoimmune-mediated suppression of the underlying stem cell disorder and that the result of this reduction is the manifestation of myeloproliferation and leukemia.- - - - - - - - - - ranking = 239.21931223425keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
9/34. Nephrotoxicity and purpura associated with levofloxacin.OBJECTIVE: To report a patient with lung cancer and idiopathic myelofibrosis with myeloid metaplasia who developed purpura and acute renal failure while receiving levofloxacin, and review the existing literature on quinolone nephrotoxicity. CASE SUMMARY: A 73-year-old white man, with a medical history of non-small-cell lung cancer and idiopathic myelofibrosis with myeloid metaplasia, was prescribed levofloxacin because of a lower urinary tract infection. Three days later, he presented with palpable purpura and erythematous skin lesions over the lower limbs and trunk, with a markedly reduced urinary output. serum creatinine and urea nitrogen were 6.4 and 190 mg/dL, respectively. levofloxacin was discontinued, and prednisone, furosemide, and intravenous fluids were given. The patient fully recovered over the ensuing 4 weeks. CONCLUSIONS: Nephrotoxicity associated with levofloxacin is uncommon. Allergic interstitial nephritis or vasculitis is believed to be the underlying pathologic process. Definitive diagnosis requires performance of renal biopsy, although this is not always feasible. In this case, a return of renal function to normal, with the disappearance of purpura following the discontinuation of levofloxacin and corticosteroid treatment, supports the presumptive diagnosis of a hypersensitivity reaction to levofloxacin.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/34. Transplant decision-making strategies in the myeloproliferative disorders.Myelofibrosis with myeloid metaplasia, also known as idiopathic myelofibrosis (IF) or agnogenic myeloid metaplasia, is one of the characteristic manifestations of polycythemia vera (PV) in the spent phase, and has a particularly adverse prognosis. IF may also present de novo. To date, treatment strategies for both spent-phase PV and IF have frustrated both clinicians and patients, with little clear progress made over the past 50 years. Treatment modalities with some benefit in chronic myeloid leukemia (CML), such as interferon (IFN), have been used to shrink the massive organomegaly seen in these patients and to improve their marrow function, but are not curative, and not all patients respond or can tolerate the agent. A curative approach is allogeneic peripheral hematopoietic stem cell transplantation. The preparative regimens used in fully ablative techniques rule out older patients for consideration, and many younger patients with good prognostic criteria may do sufficiently well on medical treatment or observation to avoid transplantation. Older patients may have the option to undergo a human leukocyte antigen (HLA)-identical sibling transplant using a reduced intensity preparative regimen in order to minimize peritransplant mortality. Thus a prerequisite to the broad use of transplantation is objective determination of candidacy. Several evaluation methods agree that anemia, age, and cytogenetic abnormalities all predict poor survival in IF, suggesting that patients with anemia and an abnormal karyotype are the prime candidates for allogeneic transplantation. Experimental peripheral blood models that may reflect the degree of marrow fibrosis, such as the serum procollagen 3 peptide assay, have been used to determine if they are more informative of patient status than a single, random bone marrow sampling. Marrow fibrosis may be patchy, and thus a marrow biopsy alone without other data about marrow function may be misleading. Considerable long-term success in eradicating fibrosis and restoring normal cytogenetics, normal bone marrow morphology, and normal complete blood cell counts through transplantation has been reported. Many questions remain to be answered, however, before the appropriate role of hematopoietic stem cell transplantation in the setting of both spent-phase PV and IF can be determined.- - - - - - - - - - ranking = 35.437855357318keywords = blood cell (Clic here for more details about this article) |
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