Cases reported "Pelger-Huet Anomaly"

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1/3. Normal bone marrow function over 6 years in a patient with dysplastic hematopoiesis and a complex karyotype.

    Myelodysplasia associated with a complex karyotype is usually associated with advanced stage myelodysplastic syndrome (MDS) and an enhanced risk to develop secondary leukemia. We report on a 36-year-old female patient who was first presented in 1997 because of 'Pseudo Pelger-Huet' neutrophils. The remaining blood and differential counts were normal. bone marrow examination revealed dysplasia in the erythroid and granulocytic series, no increase in blasts, and a karyotype with complex aberrations involving chromosomes 7, 13, 20 and 22. Almost all metaphases examined appeared to be affected. During the next few months, the patient was closely monitored and considered as candidate for bone marrow transplantation. However, blood counts remained stable without occurrence of significant cytopenias or an increase in blasts. Re-examinations of the bone marrow in 1998 and 1999 disclosed identical results compared to that obtained in 1997. After a total follow up of 6 years, the patient is still in good health with normal blood counts and persisting 'Pseudo Pelger-Huet' neutrophils. This exceptional case supports the notion that complex chromosomes are not invariably associated with rapid disease evolution in MDS.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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2/3. Acquired pelger-huet anomaly in association with concomitant tacrolimus and mycophenolate mofetil in a liver transplant patient: a case report and review of the literature.

    pelger-huet anomaly is a congenital or acquired abnormality of neutrophil nuclear segmentation. The acquired form may be a result of a clonal myeloid malignancy, such as myelodysplastic syndrome, or may be a secondary nonclonal change related to a variety of underlying causes, including infections and medications. We report a case of a 56-year-old man who developed acquired pelger-huet anomaly following liver transplantation while on the immunosuppressive agents tacrolimus and mycophenolate mofetil. These medications have been reported in association with this abnormality, but usually as a single agent or in combination with other drugs. In our case, the pelger-huet anomaly may be the result of the combination of these 2 drugs or mycophenolate alone with subsequent desensitization because resolution of the abnormality occurred after a reduction in mycophenolate mofetil dose, and the abnormality did not recur when mycophenolate mofetil was increased to a dose previously associated with pelger-huet anomaly during the time that tacrolimus was discontinued.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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3/3. A variant myelodysplastic syndrome with multilineage Pelgeroid chromatin.

    An unusual myelodysplastic syndrome with similar features in two patients is described. The entity is characterized by a maturation arrest at the myelocyte stage, strikingly clumpy chromatin, and a clinical course marked primarily by difficulties caused by anemia and thrombocytopenia. Electron microscopic description of the characteristic abnormal clumpy chromatin cells is included. While the disorder is unquestionably a myelodysplastic syndrome, it is clearly distinct from chronic myelogenous leukemia, chronic myelomonocytic leukemia, and the spectrum of refractory anemias with excess blasts.
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ranking = 6
keywords = myelodysplastic syndrome, myelodysplastic
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