| We describe a patient with pulmonary tuberculosis and a rare disturbance of leukocyte segmentation, known as "pelger-huet anomaly", which can be observed in various diseases such as malignancies and/or infections. The importance of this association is equivocal: some authors have related to the association the particular severity of tuberculosis or the death they observed; in the case reported we noted no evidence of such a relation, notwithstanding the presence of the homozygous form of the pelger-huet anomaly. We suggest therefore that, when pelger-huet anomaly is found, an underlying disease should be searched for; the course of this illness, however, might not be affected. ( info) |
2/30. Eosinophilic acquired pelger-huet anomaly in acute myeloblastic leukemia. A cytochemical study. Decreased nuclear segmentation and coarse chromatin only in mature eosinophils was seen in a patient with acute myeloblastic leukemia. These eosinophils were considered to have acquired pelger-huet anomaly. On cytochemical study they displayed a strong PAS-positive reaction. ( info) |
| A 38-year-old Japanese woman with severe aplastic anemia received an allogeneic bone marrow transplant from her serologically HLA-identical father. cyclosporine and methotrexate were administered to prevent graft-versus-host disease (GVHD). However, grade III acute GVHD developed on day 44, which was successfully treated with methylprednisolone and tacrolimus. fluconazole therapy was started for oral candidiasis on day 112, but she complained of headache soon after. In addition to glycosuria and increased serum creatinine levels, pelger-huet anomaly of granulocytes was found in her blood, which disappeared after discontinuation of tacrolimus. Transient occurrence of Pelger-Huet cells may be associated with tacrolimus toxicity due to drug interaction with fluconazole. ( info) |
4/30. Acquired and reversible pelger-huet anomaly of polymorphonuclear neutrophils in three transplant patients receiving mycophenolate mofetil therapy. Deficient nuclear segmentation and abnormal chromatin condensation define pelger-huet anomaly of polymorphonuclear neutrophils. Next to the hereditary irreversible form, acquired forms both reversible and irreversible have been described. We describe three transplant patients who were all investigated for a left shift in the absence of symptoms or signs of infection and in whom acquired reversible pelger-huet anomaly was discovered. The abnormal PMN phenotype was induced by mycophenolate mofetil (MMF). MMF is a necessary but not sufficient condition for the development of the anomaly. In our three patients a dose-response effect was observed regarding plasma MMF concentration and severity of neutrophil dysplasia. Except for one slightly elevated value, the patients' plasma MMF levels were within the therapeutic range. None of the patients, one who was neutropenic at presentation and two who were non-neutropenic, developed infectious complications. From our three cases as well as those of other authors, we identify previous graft rejection episodes as a potential predisposing factor for the development of PHA. In the first patient, drug withdrawal led to normalization of PMN morphology. In the other two patients, the left shift disappeared after dose reduction. In these latter two patients, a form of desensitization to the effect of MMF on neutro- phils was observed following re-augmentation of MMF dose. ( info) |
5/30. 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. ( info) |
6/30. Neutrophil dysplasia (acquired pseudo-pelger anomaly) caused by ganciclovir. Two thoracic organ-transplant recipients with persistent cytomegalovirus infection developed morphologic abnormalities in their circulating neutrophils while receiving either ganciclovir or its prodrug valganciclovir. Neither patient was receiving concomitant treatment with mycophenolate mofetil or azathioprine. In both patients, the morphologic abnormalities preceded the development of neutropenia and bone-marrow hypoplasia. The morphologic changes most likely reflected interference of dna polymerization within bone marrow progenitor cells. The changes resolved completely after the drug was withdrawn. ( info) |
| Congenital pelger-huet anomaly (PHA) is an autosomal dominant disorder characterized by hypolobulated neutrophils with coarse clumping of the nuclear chromatin. PHA has been recently linked to the gene encoding the lamin B receptor, located at chromosome 1q41-43. The authors report a case of PHA in a child with interstitial deletion of the 1q subtelomeric region (1q42.3-44), providing supportive evidence to this linkage. All neutrophils in the peripheral blood smear had the characteristic unsegmented or bilobed appearance. Additional features in this child included failure to thrive, developmental delay, cleft palate, seizure disorder, and dysmorphic facial features. ( info) |
8/30. 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. ( info) |
9/30. White blood cell left shift in a neonate: a case of mistaken identity. We present a full-term male infant who presented with tachypnea and an increased band count on his complete blood count (CBC) with an immature to total neutrophil (I:T) ratio of 0.6 raising suspicion of early onset sepsis. A blood culture was drawn and he was started on appropriate antibiotics. The patient's clinical condition rapidly improved; however, the white cell count 'left shift' persisted. When a detailed family history was obtained, it was discovered that the father, paternal uncle and the grandfather had been diagnosed with pelger-huet anomaly (PHA). As the urine, blood and CSF cultures were all negative in this now well-appearing infant, the left shift on the CBC was believed to be due to inheritance of the PHA. We present this case to emphasize that even in this age of sophisticated laboratory evaluation, a good clinical history, including family history, and clinical evaluation, are essential for accurate diagnosis. ( info) |
10/30. Effects of G-CSF, GM-CSF, and IL-5 on nuclear segmentation of neutrophils and eosinophils in congenital or acquired pelger-huet anomaly. We studied the in vitro effects of granulocyte colony-stimulating factor (G-CSF), granulocyte-macrophage colony-stimulating factor (GM-CSF), and interleukin 5 (IL-5) on nuclear segmentation of neutrophils and eosinophils from three patients with congenital or acquired pelger-huet anomaly. After a 24-h incubation with G-CSF, the majority of neutrophils showed nuclear development characterized by a bilobed appearance. In contrast to neutrophils, the nuclear segmentation of eosinophils was not induced after incubation with G-CSF, GM-CSF, or IL-5. These results suggest that G-CSF plays some role in the nuclear development of neutrophils, whereas IL-5 may not have such an effect on eosinophil maturation in the individual cases studied. ( info) |