bluuebunny/crossref_metadata_2025 huggingface dataset:
Posttransfusion purpura associated with alloantibody specific for the platelet antigen, Pena
Authors: Simon Toby L. , Collins Janice , Kunicki Thomas J. , Furihata Kenichi , Smith Kenneth J. , Aster Richard H.
Published: February 2007

Posttransfusion purpura (PTP) and severe thrombocytopenia occurred 9 days after transfusion of red blood cells to a 48‐year‐old, multiparous Navajo woman. The platelet count rose to hemostatic levels after treatment with prednisone and three plasma exchange transfusions. Serologic studies showed that the patient's serum contained the potent antibody reactive with platelets from nearly all normal subjects, but nonreactive with autologous platelets obtained after recovery. This antibody was found to be specific for a high‐frequency, platelet‐specific antigen, designated Pena, implicated previously as an immunogen in neonatal alloimmune thrombocytopenic purpura. An exchange of serum showed that Pena is identical with an alloantigen designated Yukb by Japanese workers. We conclude that PTP can occur in association with alloimmunization against Pena (Yukb).
Anticoagulant therapy for prevention of spontaneous abortion in a patient with a lupus anticoagulant
Authors: Joffe A. Mark , Hoskins Christopher F. , Ghitter‐Mannes Sandra C. , Mant Michael J.
Published: February 2007

Lupus anticoagulants are associated with venous and arterial thrombosis and with recurrent spontaneous abortion resulting from placental infarction. Treatment with high‐dose pred‐nisone and aspirin has been reported to reduce the otherwise very high frequency of spontaneous abortion in affected women. We report the case of a young woman with an idiopathic lupus anticoagulant who had a history of arterial and venous thrombosis and of previous spontaneous abortion; anticoagulation throughout pregnancy was associated with normal fetal growth and with an absence of placental infarction. We conclude that anticoagulation without concurrent prednisone or aspirin may provide an alternative approach to prevention of habitual abortion in some women with lupus anticoagulants.
Permanent neurological complications in patients with thrombotic thrombocytopenic purpura
Authors: Ben‐Yehuda Dina , Rose Michal , Michaeli Yossef , Eldor Amiram
Published: February 2007

According to previous clinical studies, the neurological manifestations observed in patients with thrombotic thrombocytopenic purpura (TTP) are considered to be transient. The introduction of plasma therapy in 1977 as major treatment modality for TTP has changed the prognosis of the disease. In a clinical survey of 38 patients with TTP who received plasma therapy, we have observed five patients who developed permanent neurological deficits despite their prompt recovery from TTP. In this study, we describe the new complication of TTP and summarize the neurological manifestations observed in these patients during their first episode of TTP and during the relapses, which occurred in 12 of them.
Association of large granular lymphocyte/natural killer cell proliferative disease and second hematologic malignancy
Authors: Bassan Renato , Rambaldi Alessandro , Allavena Paola , Abbate Mauro , Marini Basilio , Barbui Tiziano
Published: February 2007

We describe the first three patients with a large granular lymphocytosis/lymphocytic leukemia and another blood malignancy. In two, a myeloproliferative disorder developed soon after the diagnosis of abnormal proliferation of large granular lymphocytes‐natural killer (LGL‐NK) cells, a myelodysplastic syndrome evolving to acute leukemia and a Philadelphia‐positive chronic myelogenous leukemia. In these cases, LGLs expressed the phenotype of CD2 + NK and CD3 ‐ NK cells, respectively, and were clonal in the first patient as demonstrated by T‐cell receptor gene rearrangement study. In the third case, a similarly clonal excess of LGLs, phenotypically CD3 + NK cells, was detected following a diagnosis of B‐cell hairy‐cell leukemia. Clinically, the concurrence of LGL proliferation and other leukemia did not seem to confer a worse prognosis on the patients. Although an association by chance remains a possible explanation, a common origin from an altered precursor cell for both myeloid and LGL proliferations in the first two cases is discussed, whereas in the third it might be related to the severe immune derangement frequently observed in hairy‐cell leukemia.
Totally implantable intravenous catheters in the management of sickle cell anemia
Authors: Phillips George , Slingluff Craig , Hartman John , Thomas Patricia , Akwari Onye
Published: February 2007

Totally implantable catheters (TICs) have recently been employed for long‐term central venous access in patients with sickle cell disease (SCD). We have reviewed our experience with 10 TICs inserted in patients with SCD. These were compared to 33 TICs inserted in patients without SCD (controls). The primary diagnosis was malignancy in most of the controls. The SCD patients experienced a marked increase in total complications (70 vs. 24%), as well as in complications requiring catheter removal (50 vs. 3%). No variable explained these differences except the presence of SCD. The complications requiring catheter removal from SCD patients were infections, catheter thrombosis, and venous thrombosis. The increased risk of these complications must be considered before a catheter is inserted; however, the average useful life of these catheters exceeded 12 months. They remained useful in the care of patients with poor venous access and multiple complications of SCD.
Comparative evaluation of the bone marrow by the volumetric method, particle smears, and biopsies in pediatric disorders
Authors: Ozkaynak M. Fevzi , Scribano Philip , Gomperts Edward , Izadi Parvin , Millin Rachel , Jr Hart Isaacs , Ettinger Lawrence J.
Published: February 2007

Bone marrow cellularity estimated by biopsy was compared to the cellularity of the aspirate particle smear and the volumetric method in two groups of children. In the first group, 101 consecutive bone marrow biopsies and aspirates were evaluated from patients with various diagnoses. In the second group, 20 patients with acute nonlympho‐blastic leukemia were studied with 80 biopsies and aspirates at diagnosis and following chemotherapy. A wide discrepancy was noted between bone marrow cellularity confirmed by biopsy vs. the particle smear or the volumetric method in both groups. Neither the volumetric nor the particle method provides a good correlation of bone marrow cellularity. We also compared the volumetric method with that of the biopsy to evaluate the efficacy of the former method in detecting bone marrow infiltration by solid tumors. The volumetric method is an accurate modality of identifying solid tumor infiltration in the bone marrow.
Myelofibrosis: A concise review of clinical and pathologic features and treatment
Authors: Smith Roy E. , Chelmowski Mark K. , Szabo Edward J.
Published: February 2007

This paper reviews and clarifies the current understanding of the clinical and pathologic features and treatment of MF. Recent investigations indicate that MF may be mediated by platelet‐ and megakaryocyte‐derived growth factors, impaired prostaglandin‐mediated stem cell growth inhibition, or excessive endothelial cell and fibroblast proliferation. Immunologic disorders have been associated with MF. MF may be either a primary or a secondary phenomenon. Secondary MF often regresses with appropriate treatment of this underlying disorder. Primary MF may require androgen therapy, splenectomy, splenic irradiation, bone curettage, chemotherapy, or bone marrow transplantation.
Production of B‐cell growth factor interleukin 2 and gamma interferon by peripheral blood lymphocytes from patients with chronic lymphocytic leukemia of B‐cell type
Authors: Rossi Jean‐François , Commes Therese , Grenier Jean , Jourdan Michel , Chichehian Behrouz , Klein Bernard
Published: February 2007

Chronic lymphocytic leukemia of B‐cell type (B‐CLL) is a malignant disease characterized by monoclonal proliferation of small lymphocytes of B‐cell origin, usually associated with suppression of polyclonal B‐cell activation (i.e., proliferation and differentiation). Normal human B‐cell proliferation is controlled by different T‐cell‐derived lymphokines, including interleukin 2 (IL2) and gamma interferon (γ‐IFN), that account for the majority of the B‐cell growth factor (BCGF) activity produced by mitogen‐activated peripheral blood mononuclear cells (PBMCs). We have previously shown an increased and dysregulated secretion of IL2 in peripheral blood from patients with BCLL. BCGF, IL2, and γ‐IFN productions by phytohemagglutinin (PHA)‐stimulated PBMCs were investigated in 13 patients with active untreated B‐CLL and 11 healthy donors. BCLL PBMCs produced a significant amount of BCGF (6 U/ml) despite the low percentage of T cells (10%) associated with this disease as compared with that found in healthy donors (61%). BCGF production in normal controls and B‐CLL patients was tripled after irradiation of PBMCs or addition of indomethacin. γ‐IFN secretion in B‐CLL patients was decreased when compared with normal controls. Therefore, when γ‐IFN was calculated per fixed number of T cells, production was significantly higher in B‐CLL patients than in normal controls, showing a dilution of the productive cells. This study suggests that T cells from B‐CLL patients are functional in terms of BCGF production despite their decreased percentage and abnormalities in surface markers.
The behavior of asialotransferrin‐iron in the rat
Authors: Beguin Yves , Bergamaschi Gaetano , Huebers Helmut A. , Finch Clement A.
Published: February 2007

The effect of desialylation of rat and human transferrins on hepatocyte processing of the protein and its iron was studied in rats. No alteration in early transferrin catabolism was observed. Radioiron disappearance from the plasma and liver iron uptake were more rapid for asialotransferrins than for normal transferrins (P<.001). Furthermore, radio‐iron plasma clearance of human tri‐sialotransferrin was faster (P < .05) and liver uptake higher (P < .002) than for human pentasialotransferrin. When the asialoglycoprotein receptor was blocked by the prior injection of asialofetuin, asialotransferrin behaved like normal transferrin. When the transferrin receptor was blocked by the prior injection of 50 mg human diferric transferrin, iron uptake from all transferrins was delayed to such an extent that uptake through both receptors seemed to be affected. Approximately 90% of the hepatic radioiron from all transferrins was chelated by desferrioxamine and excreted into the bile, indicating its uptake by the hepatocyte rather than the reticulo‐endothelial (RE) cell. The rate of iron release into the plasma and its subsequent accumulation in the red cell mass over a 2‐week period was similar for human asialotransferrin, ferritin, and hemoglobin iron. This study 1) confirmed that asialotransferrin‐iron uptake by the hepatocyte is mediated by both transferrin and asialoglycoprotein receptors; 2) demonstrated that not only asialotransferrin but also transferrin of low sialic acid content will increase iron turnover and lead to excessive iron loading of the hepatocyte; 3) and showed that the intrahepatocyte metabolism of asialotransferrin‐iron did not differ from that of iron delivered by normal transferrin.
Pharmacology and special clinical applications of low‐molecular‐weight heparins
Authors: Harenberg J. , Heene D. L.
Published: February 2007

In this overview, the rationale of the development of low‐molecular‐weight (LMW) heparins and their toxicological, anticoagulant, fibrinolytic, lipolytic, and protamine interactions are summarized. Clinical experiences are reviewed on the benefit of LMW heparin for anticoagulation in patients with bleeding and other complications on conventional anticoagulants and during pregnancy. It is concluded that animal experiments have demonstrated the safety of LMW heparins, that the pharmacologic profile is improved compared with normal heparin, and that the simple and safe applicability of LMW heparins gives rise to new indications for the long‐term prophylaxis of thromboembolism.
A polyclonal CD4+ and CD8+ lymphocytosis in a patient doubly infected with HTLV‐I and HIV‐1: A clinical and molecular analysis
Authors: Ehrlich Garth D. , Davey Frederick R. , Kirshner Jeffrey J. , Sninsky John J. , Kwok Shirley , Slamon Dennis J. , Kalish Randy , Poiesz Bernard J.
Published: February 2007

HTLV‐I is associated with adult T‐cell leukemia/ lymphoma (ATL) characterized by monoclonal expansions of CD4+ T‐lymphocytes. In this report we describe a histologically benign, polyclonal HTLV‐I infection in a patient exhibiting both an absolute CD4+ and CD8+ lymphocytosis. Three T‐cell lines containing integrated HTLV‐I proviral copies established from this patient were initially polyclonal, but with time all grew out the same two clones as determined by analysis of their T‐cell antigen receptor beta chain gene rearrangements. The patient subsequently developed pulmonary and nasopharyngeal nodules containing HTLV‐I infected cells. Restriction analysis of the patient's HTLV‐I provirus revealed no differences from prototype HTLV‐I and the tax gene was normally expressed in vivo and in vitro. The patient's T‐lymphocytosis and HTLV‐I+ pulmonary tract nodules were put into a complete clinical remission by treatment with alkylating agents and steroids. Subsequently, the patient developed a severe immunodeficiency state and expired. Retrospective serologic and gene amplification assays for HIV‐1 demonstrated that he had been doubly infected from the time of presentation. Postmortem analysis by polymerase chain reaction revealed the presence of both HTLV‐I and HIV‐1 in lymphatic tissues and the testes; HIV‐1 was also detected in brain tissue.
Hermansky‐pudlak syndrome: Correction of bleeding time by 1‐desamino‐8D‐arginine vasopressin
Authors: Wijermans P. W. , van Dorp D. B.
Published: February 2007

The effect of the synthetic vasopressin derivative 1‐desamino‐8D‐arginine vasopressin (DDAVP = desmopressin) on bleeding time was studied in three patients with Hermansky Pudlak syndrome. A good response was observed in this type of storage pool disease. DDAVP might be useful in managing the bleeding disorder found in patients with the Hermansky‐Pudlak syndrome.
Microgranular promyelocytic leukemia: A multiparameter examination
Authors: Krause John R. , Stolc Viktor , Kaplan Sandra S. , Penchansky Lila
Published: February 2007

Six cases of microgranular variant acute promyelocytic leukemia (M3v) were studied by use of a multiparameter approach including morphology, cytochemistry, flow cytochemistry, flow cytometry, cytogenetics, and gene rearrangement. Three of six cases demonstrated both myeloid and monocytoid associated surface markers by flow cytometry. One of six cases had strong alpha‐naphthyl‐butyrate esterase (α‐NBE) activity in addition to myeloperoxidase activity. There was no correlation between percentage of positive monocytoid surface markers and intensity of cytoplasmic α‐NBE activity. Four of six cases also had a T‐cell‐associated surface antigen. Further studies indicated that the T‐cell markers appeared to be on the promyelocytes and that the T‐B receptor gene was not rearranged. Similarly, cytogenetics studies indicated only one clonal abnormality t(15q+; 17q‐). Whether these cases represent true “lineage infldelity” remains to be answered. Future important studies are needed on normal hematopoietic progenitor cells at early stages of development and childhood to study lineage‐specific characteristics and to determine whether co‐expression normally exists during early development.
Modified bleeding time in the infant
Authors: Andrew Maureen , Castle Valerie , Mitchell Lesley , Paes Bosco
Published: February 2007

The present authors developed a modified template bleeding time for use in the newborn infant. The sensitivity of the bleeding time to the presence of antibiotics, indomethacin, generalized illness, and thrombocytopenia was tested in 242 infants. Both indomethacin and thrombocytopenia similarly prolonged the BT, and the latter could be corrected by raising the platelet count.
Detection of hypodiploidy using multi‐parameter flow cytometric analysis: A prognostic indicator in multiple myeloma
Authors: Morgan Robert J. , Gonchoroff Nick J. , Katzmann Jerry A. , Witzig Thomas E. , Kyle Robert A. , Greipp Philip R.
Published: February 2007

We analyzed 49 patients with multiple myeloma (MM) using propidium iodide (PI) staining and flow cytometry to assess DNA content of MM cells in bone marrow. In 12 patients the presence or absence of DNA content aneuploidy could not be determined by analysis of single‐parameter DNA histograms alone. Using forward light scatter measurement, resolution of ploidy was readily obtained in all but three cases. Assuming the DNA content of the low light scatter population to be diploid, we were able to classify 7 cases as hypodiploid and 39 as hyperdiploid or diploid. Hyperdiploid or diploid patients survived a median of 24 months and hypodiploid patients only 2.5 months. We conclude that multiparameter analysis of DNA content and light scatter provides easier analysis for the presence or absence of aneuploidy. Patients with DNA content hypodiploidy have a short survival.
Thrombocytosis as a presenting feature of acute lymphoblastic leukemia in childhood
Authors: Blatt Julie , Penchansky Lila , Horn Marianna
Published: February 2007

To determine the incidence of thrombocytosis at presentation in acute lymphoblastic leukemia (ALL), medical records of all children diagnosed at the Children's Hospital of Pittsburgh from 1980 to 1987 were reviewed. Out of 217 such patients, 7 (3.2%) had platelet counts greater than 400,000/mm3. All of the seven were boys compared with a male:female ration of 1.4:1 in the entire ALL population. Other than sex, no characteristics were clearly associated with thrombocytosis, including white blood cell count, hemoglobin, lymphoblast morphology, and immunologic or chromosomal markers. Apart from ALL, no inflammatory or infectious process which might have caused a thrombocytosis, was detected in any of these patients. The period of induction therapy was notable for the preservation of platelet counts greater than 20,000/mm3 in all patients. However, of the seven children with thrombocytosis, two had major induction complications: one, a cavernous sinus thrombosis; and the other, gastrointestinal bleeding with duodenal perforation.

We conclude that thrombocytosis at diagnosis can be seen in children, particularly boys, with ALL. Based on small numbers, this group of patients may be at risk for major events during induction therapy. Large numbers, longer follow‐up, and platelet function studies on similar patients will be of interest.
Congenital erythroleukemia: A case report with morphological, immunophenotypic, and cytogenetic findings
Authors: Allan Randy R. , Wadsworth Louis D. , Kalousek Dagmar K. , Massing Bonnie G.
Published: February 2007

We report a lethal case of congenital erythroleukemia presenting on the first day of life with peripheral blast cells and a leukemic infiltrate in the placenta. Although initial bone marrow examination did not fulfill the French‐American‐British (FAB) cooperative group criteria for acute myelogenous leukemia (AML), including M6, a malignant clone was confirmed by cytogenetic analysis: 49,XX,+8,+19,+21. Evolution to erythroleukemia (M6) occurred over a two‐month period. The diagnosis of erythroleukemia was supported by immunophenotyping employing an antibody to glycophorin A. The clinical course was complicated by liver failure of unknown etiology. Comparison to previously reported cases of early childhood erythroleukemia is made.
Artefactual low lymphocyte activity caused by platelet contamination in the mononuclear cell preparations
Authors: Rueda F. , Marti F. , Pinol G. , Remacha A.
Published: February 2007

Platelet (PLT) contamination in preparations of mononuclear cells (MNC) produces a significant decrease (P<0.02) in lymphoproliferative activity and interleukin 2 (IL2) production. This effect was observed in MNC preparations from either healthy blood donors or polyglobulic patients. In a dose‐response curve carried out with increasing PLT number per MNC, progressive inhibition was found, reaching to 62.6% and 49.5%, respectively, to phytohemagglutinin and IL2 lymphoproliferative response and 87.8% in IL2 production, at 80 PLTs/MNC.
Prolymphocytic transformation of chronic lymphocytic leukemia: A case report of lengthy survival after intensive chemotherapy
Authors: Roberts John D. , Tindle Barbara H. , Macpherson Bruce R.
Published: February 2007

A case of prolymphocytic transformation of chronic lymphocytic leukemia (CLL) is presented in which complete peripheral morphometric remission and lengthy survival were observed after intensive chemotherapy. The case is discussed within the context of the reported therapeutic experience.
Spontaneous regression in non‐hodgkin's lymphoma: Clinical and pathogenetic considerations
Authors: Drobyski William R. , Qazi Raman
Published: February 2007

The spontaneous regression (SR) of tumor has been noted in a variety of neoplastic conditions. In non‐Hodgkin's lymphoma, this phenomenon has been reported in indolent histologic subtypes, with a frequency of 10–20% in selected series. Invstigators evaluating new therapies for lymphomas with a favorable histology need to be cognizant of SR's impact. Mechanisms which have been proposed to explain SR have included the role of contemporaneous bacterial or viral infection, as well as an augmented host immune response which is able to mediate tumor regression via humoral and cellular effector mechanisms. The ability to recapture immunoregulatory control is aptly illustrated by lymphomas developing after organ transplantation where reduction of immunosuppression has, on occasion, resulted in tumor regression. The importance of immune regulation of B‐cell lymphoma is also suggested by the tumor's responses to immunotherapy and interferons in vivo and by the biologic and pathologic characteristic of indolent lymphomas being analogous, in may respects, to benign neoplasms. Indolent lymphomas which differ from aggressive lymphomas in their clinical and biological behavior may be more responsive to these host immunoregulatory influences. Review of clinical experience as well as proposed mechanisms of spontaneous regression in non‐Hodgkin's lymphoma will be explored in this report.
Role for platelet von willebrand factor in supporting platelet‐vessel wall interactions in von willebrand disease
Authors: Castillo R. , Escolar G. , Monteagudo J. , Ordinas A. , Garrido M. , Moia M. , Federici A. B. , Mannucci P. M.
Published: February 2007

Twelve infusions of plasma concentrates of von Willebrand factor (vWF) were given to four patients with severe (type III) von Willebrand disease (vWD). Their prolonged bleeding times were either completely or partially corrected after five infusions and had not changed after the remaining seven. In contrast, the low platelet coverage of the subendothelial surface of rabbit aorta perfused with normal washed platelets and red cells resuspended in preinfusion patient plasma was completely or partially corrected in ten instances by replacing preinfusion plasma with postinfusion plasma and remained unchanged in two. Postinfusion improvement in surface coverage was greater than that in bleeding time, suggesting that vWF from normal platelets is needed to support optimal platelet‐vessel wall interactions in vWD. This possibility was further explored through other perfusion experiments. The subendothelial surface covered by platelets from an untreated patient with type III vWD (containing no measurable vWF) or from a type IIA vWD patient (containing dysfunctional vWF) resuspended in normal plasma was much smaller than that covered by normal platelets resuspended in normal plasma. These results establish that platelet vWF is important in supporting platelet‐vessel wall interactions in vWD and also provide experimental support in favour of the therapeutic transfusion of normal platelets in addition to vWF concentrates to correct the bleeding time in vWD patients.
Immunodiagnosis of acute leukemia displaying ectopic antigens: Proposal for a classification of promiscuous phenotypes
Authors: Vecchio Luigi Del , Schiavone Ettore Mariano , Ferrara Felicetto , Pace Emilio , Pardo Catia Lo , Pacetti Maurizio , Russo Maria , Cirillo Dora , Vacca Clemente
Published: February 2007

The nature of the blast cells in 163 cases of acute leukemia was investigated by immunophenotyping, with particular emphasis on the expression of “ectopic” surface membrane structures. Although no antigen included in our panel except CD3 revealed absolute lineage restriction, immunological typing allowed a definite characterization of blast cells in more than 90% of cases. Four groups of patients were identified (A, B, C, D) with different degrees of antigen ectopic expression. We classified as group A leukemias (74%) those expressing conventional antigenic patterns, in absence of cross‐lineage markers. Samples classified as group B (18%) showed a single ectopic membrane specificity, apparently discordant with the overall composite phenotype; such a “low‐grade deviation” did not prevent a definite immunodiagnosis. Pattern C specimens (5%) revealed a promiscuous coexpression of markers related to different lineages (biphenotypic leukemias), whereas group D included unclassifiable phenotypes, characterized by no antigen or DR‐only expression. Our findings suggest the possibility of interpreting complex phenotypic constellations of membrane markers in a consistent and logical manner.
T‐cell non‐hodgkin lymphoma in human immunodeficiency virus‐1‐infected individuals
Authors: Lust John A. , Banks Peter M. , Hooper W. Craig , Paya Carlos V. , Kueck Brian D. , Hanson Gerald A. , Ritch Paul S. , Woloschak Gayle E.
Published: February 2007

We present two patients with human immunodeficiency virus‐1 (HIV‐1) infection in whom T‐cell non‐Hodgkin lymphoma developed, based on pathologic diagnosis, immunophenotyping, and T‐cell receptor gene rearrangement. Both cases were positive for human immunodeficiency virus‐1 by enzyme‐linked immunosorbent assay and immunoblot methods. Histologic sections from each patient showed a high‐grade pleomorphic T‐cell non‐Hodgkin lymphoma, and immunophenotyping demonstrated a prevalence of reactivity for CD4 (helper) over CD8 (suppressor) antigens. T‐cell receptor β‐chain gene rearrangement studies revealed a rearranged pattern with either the Hindlll or BamHI enzymes, whereas immunoglobulin heavy chain genes retained a germ‐line configuration. Viral sequences specific for human T‐cell leukemia virus‐1, human T‐cell leukemia virus‐II, or HIV‐1 were not detected. Thus, although rare, T‐cell non‐Hodgkin lymphoma may be observed in HIV‐1‐infected individuals.
Hyponatremia and seizures in young children given ddavp
Authors: Smith Thomas J. , Gill Joan C. , Ambruso Daniel R. , Hathaway William E.
Published: February 2007

Desmopressin (DDAVP), a synthetic vasopressin, temporarily corrects bleeding abnormalities associated with mild hemophilia A, von Willebrand disease, and disorders of platelet function. The side effects of DDAVP are considered benign although most of its use has been in adults and older children. We report four children under the age of 2 years who became hyponatremic after intravenous DDAVP administration (0.3 μg/kg). Three of them developed grand mal seizures. A review of the literature and these cases indicate that associated risk factors for hyponatremia after DDAVP administration include stress, surgery, anesthesia and narcotics (endogenous release of antidiuretic hormone), vomiting (loss of Na+), liver disease (hindered metabolism of DDAVP), renal tubular acidosis (chronically low serum Na+), multiple doses of DDAVP, and overhydration with hyponatremic fluids. DDAVP is not a benign drug in this age group and shows a serious potential for hyponatremia and seizures. Fluid restriction, avoidance of hyponatremic solutions, and close monitoring of serum electrolytes and urine output for at least 15–20 hr after the administration of DDAVP, when used in children under the age of 2 years, is warranted.
Hemolysates reduce iron released from transferrin
Authors: Zhan Hui , Pollack Simeon , Weaver Janet
Published: February 2007

Transferrin donates iron to reticulocytes as follows: it binds to a receptor on the reticulocyte surface; the complex is endocytosed; both irons are released and the transferrin is recycled to the cell exterior. It has been proposed that the trigger for iron release after transferrin endocytosis is acidification of the endocytic vesicle. But this could account for removal of only one of transferrin's two irons, since only one of the irons is labile at acid pH. Moreover, iron continues to be removed from transferrin when acidification of the vesicle is blocked by a chloride‐transport inhibitor. Thus a detailed explanation of iron removal from transferrin remains elusive. In earlier work we showed that iron can be removed from transferrin by whole hemolysates and also by the combined action of hemoglobin and ATP at pH 7. We now show that the iron released from transferrin by hemolysates, and by hemoglobin and ATP, is in the Fe(II) oxidation state. We also show that ADP and DPG can substitute for ATP and that NADH and NADPH can substitute for the hemoglobin, although with these substitutions Fe(II) is generated less efficiently. The reductive release of iron from transferrin is rapid enough to account for all the iron processed by a young reticulocyte. We speculate that transferrin iron may be reduced to Fe(II) before reaching the mitochondria.
Serum and red cell folate and serum vitamin B12 levels in hyperthyroidism
Authors: Ford H. C. , Carter J. M. , Rendle M. A.
Published: February 2007

Serum and red blood cell folate levels and serum B12 concentration were determined by radioassay in 20 hyperthyroid patients and compared with values obtained when the same patients had been euthyroid for at least 4 months. In hyperthyroidism, the levels of serum and red blood cell folate were significantly (P < .01) higher than when euthyroidism was achieved. There was no significant change in serum B12 concentration. Declines in serum and red blood cell folate levels between hyperthyroidism and euthyroidism occurred in 15 and 16 of the 20 patients, respectively. Although the explanation for the relative elevations of serum and red blood cell folate levels in hyperthyroid patients is unclear at present, our findings do not support the view that hyperthyroidism in man is associated with depletion of folate stores or subclinical deficiency of the vitamin.
Complete remission in acute promyelocytic leukemia despite the persistence of the 15;17 translocation
Authors: Wallace Paul J.
Published: February 2007

Acute promyelocytic leukemia (APL) is a morphologically distinct subtype of acute non‐lymphocytic leukemia (ANLL) characterized cytogenetically by the presence of a trans‐location between chromosomes 15 and 17 (t(15;17)). In contrast to other subtypes of ANLL, morphologic examination of the bone marrow in the early postinduction period fails to identify patients adequately who have a high likelihood of entering remission. Remission has frequently been achieved in APL despite the failure to attain marrow aplasia or eliminate dysplastic progranulocytes after initial induction chemotherapy. In a patient with APL who failed to achieve cytoreduction after a single course of induction chemotherapy, serial cytogenetic studies demonstrated persistence of metaphases with the t(15;17) up to 23 days after induction. However, complete morphologic and cytogenetic remission was subsequently attained by day 29 without further therapy. In APL remission may occur even when induction therapy fails to achieve marrow aplasia or to eradicate replicative cells with abnormal karyotype promptly. In order to avoid excessive exposure to toxic therapy, new strategies may be necessary to identify, early in treatment, patients with APL who will require only one course of induction chemotherapy to enter remission successfully.
Adult T‐cell leukemia initially manifesting as facial diplegia
Authors: Sawada H. , Matsui M. , Udaka F. , Nishimura M. , Fujita M. , Kameyama K.
Published: February 2007

We report an unusual case of adult T‐cell leukemia (ATL) initially manifesting as facial palsy due to ATL meningitis, which was caused by lymphoid cells in the cerebrospinal fluid (CSF) with a different phenotype from those in the blood. A DNA southern blot analysis of the blood cells confirmed the diagnosis of ATL. The aberrant cells in the CSF contained nuclei with simple indentations and responded well to the initial chemotherapy, while leukemic cells in the blood showed lobulated or convoluted nuclei and were resistant to treatment. Flow cytometry performed before treatment demonstrated that the majority of the cells in the CSF were CD3 + CD4 + CD8‐CD25 +, consistent with ATL, whereas they expressed the CD45R antigen, which the blood ATL cells did not. These differences in the subphenotype, cell morphology, and responsiveness to treatment among the ATL cells in blood and CSF suggest that a single clone or subclone with heterogeneous nature was not generated, before the final development of ATL.
Ferrokinetic study of splenic erythropoiesis: Relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis
Authors: Beguin Yves , Fillet Georges , Bury Jean , Fairon Yvette
Published: February 2007

Splenic erythropoiesis was demonstrated by surface counting of 59Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agno‐genic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodys‐plastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombo‐cythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II‐III myelofi‐brosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis 1) is encountered in a variety of clinical conditions; 2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myelo‐proliferative disorders; 3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and 4) can be evaluated in a semiquantitative manner by surface counting.
Whole blood aggregation in von willebrand disease
Authors: Sweeney Joseph D. , Hoernig Lynne A. , Fitzpatrick John E.
Published: February 2007

Platelet function testing in von Willebrand disease is generally performed in platelet rich plasma using an optical system. The characteristic abnormality is an abnormal response to the agglutinating agent, ristocetin. Platelet aggregation can be also studied in whole blood using either an impedance aggregometer or a particle counter. Fifteen patients with von Willebrand disease and fifteen normal subjects were studied using both whole blood methods. In the impedance system, normal subjects responded to ristocetin (1 mg/ml) with short lag phases (<70 sec) and normal maximum aggregation (>5 Ω). Only three patients with von Willebrand disease responded with normal maximal aggregation but each of these had a prolonged lag phase, and this may be a useful diagnostic parameter. In the particle counter, discrimination between normals and some von Willebrand disease patients was possible but an overlap between normals and von Willebrand patients is evident. It is concluded that the platelets in patients with von Willebrand disease exhibit the same abnormalities in whole blood as in platelet rich plasma and that the combination of impedance aggregometry and a factor VIII procoagulant assay is a time‐efficient and sensitive method to screen for von Willebrand disease.
Distribution of breakpoint within the breakpoint cluster region (bcr) in chronic myelogenous leukemia with a complex philadelphia chromosome translocation
Authors: Nishigaki Hikari , Inazawa Johji , Misawa Shinichi , Nishida Kazuhiro , Okuda Tsukasa , Horiike Shigeo , Tsuda Shoichiro , Taniwaki Masafumi , Abe Tatsuo
Published: February 2007

We analyzed, by Southern blot hybridization, the site of breakpoint within the breakpoint cluster region (bcr) in six patients with a complex Philadelphia chromosome (Ph) translocation and in 23 unselected patients with a standard Ph. The breakpoint was found within the 5.8 kb bcr in all 29 patients. When the bcr was subdivided into four parts, fragments I–IV, based on the restriction enzyme sites, among the six patients with a complex Ph, two had a breakpoint at fragment I, three at fragment II, and one at fragment III. This distribution of breakpoints in patients with a complex Ph did not differ significantly from that in patients with a standard Ph. A deletion of an allele within the bcr was found in three patients (50%) with a complex Ph and in three (13%) with a standard Ph. The internal bcr deletion may be more common in patients with a complex Ph.
B‐cell acute lymphocytic leukemia in HIV‐antibody‐positive patients
Authors: Gold Jay E. , Castella Antonio , Zalusky Ralph
Published: February 2007

B‐cell non‐Hodgkin lymphoma (NHL) has been well described in association with human immunodeficiency virus (HIV) and the acquired immunodeficiency syndrome (AIDS). Many of these lymphomas are of the diffuse, aggressive, subtype of B‐cell NHL, including Burkitt and Burkitt‐like lymphoma. Recently, there have been reports of B‐cell acute lymphocytic leukemia (ALL), Burkitt type, in patients who were either HIV antibody‐positive or at high risk for AIDS. We have seen three cases of B‐cell ALL, Burkitt type, and herein describe their clinical and laboratory characteristics. All patients were HIV antibody‐positive. Since stage IV Burkitt lymphoma in blood phase and B‐cell ALL, Burkitt type, represent a continuum of the same disease, and since it is also an aggressive B‐cell malignancy, we suggest that B‐cell ALL, Burkitt type, in HIV antibody‐positive patients should support the diagnosis of AIDS.
Alveolar cell carcinoma complicating sickle cell anemia: A chance occurrence?
Authors: Labi Marlon , Haponik Edward F. , Welsh Ronald A. , Summer Warren R.
Published: February 2007

Alveolar cell carcinoma, a neoplasm associated with pre‐existing inflammatory lung disease, has not been reported previously in patients with sickle cell disease. We present such a patient and suggest that chronic pulmonary scarring in this setting predisposed to development of his malignancy. As survival increases in individuals with sickle cell disease, alveolar cell carcinoma must be considered in these differential diagnosis of otherwise unexplained chronic pulmonary infiltrates.
Successful treatment of refractory anemia with high‐dose methylprednisolone
Authors: Motoji Toshiko , Teramura Masanao , Takahashi Masatomo , Oshimi Kazuo , Okada Michiko , Kusakabe Kiyoko , Mizoguchi Hideaki
Published: February 2007

Five patients with refractory anemia were treated with high‐dose methylprednisolone. An immediate and long‐lasting hematological improvement without serious side effects was achieved in two of the patients, although chromosomal abnormalities persisted in both. The clinical course and laboratory data of these two patients are described. Restoration of normal hematopoiesis was achieved regardless of the enhancement of colony formation of granulocyte progenitor cells by the simultaneous addition of hydrocortisone in vitro. The same treatment was given to six patients who had refractory anemia with an excess of myeloblasts (RAEB), but no improvement was observed in any of these patients. It appears that high‐dose methylprednisolone can be valuable in the treatment of refractory anemia, but is not useful for RAEB.
Evaluation of reticulocyte counts by flow cytometry in a routine laboratory
Authors: Ferguson David J. , Lee Siow‐Fong , Gordon Philip A.
Published: February 2007

Reticulocyte counting using flow cytometry and a membrane‐permeable fluorescent dye, thiazole orange, was evaluated as an alternative to the conventional manual method. The flow cytometric method was more precise (mean c.v. of 4.3%) than the imprecise manual technique (mean c.v. of 22.4%). Linearity was highly acceptable (r = 0.99) over the reticulocyte count range of 1.8–30.1%.

Flow cytometry allows processing of large numbers of samples with reduction in technologist time, and the improved precision and tenfold increase in the number of cells counted should considerably improve the clinical utility of the reticuloctye count.
Thrombocytopenic purpura in a patient with lupus anticoagulant: Requirement of both immunosuppressive and antithrombotic therapies
Authors: Iwahara Yoshihito , Niiya Kenji , Yamato Kenji , Miyoshi Lsao
Published: February 2007

A 33 year old woman with severe thrombocytopenic purpura complicated by typical lupus anticoagulant developed repeated spontaneous abortion, deep venous thrombosis, and cerebral thrombosis. The platelet count fluctuated from 4,000 to 400,000/mm3 during the 13 year clinical course. The physical and laboratory findings at the time of severe thrombocytopenic purpure were compatible with the criteria of idiopathic thrombocytopenic purpura except for positive lupus anticoagulant. Both immunosuppressive therapy with prednisolone and antithrombotic therapy with warfarin and aspirin were necessary for the control of bleeding and venous and arterial thrombosis.
Sickle hemoglobinopathies in sicily
Authors: Schilirò G. , Spena M. , Giambelluca E. , Maggio A.
Published: February 2007

The clinical and hematological features of 202 Sicilian subjects with sickle cell disease are reported, 41 being homozygous for betaS (βSβS), 64 with betas thal betaS (βsβS), and 97 beta+ thal betaS (β+ βS). Analysis of the findings showed that the disease observed in Sicilians is of intermediate severity and falls between the severe form observed in patients of African origin and the milder one seen in subjects of Arabian origin.
Thrombin Vs. Plasmin generation in disseminated lntravascular coagulation associated with various underlying disorders
Authors: Takahashi Hoyu , Tatewaki Wataru , Wada Ken , Hanano Masaharu , Shibata Akira
Published: February 2007

In order to assess the thrombin and plasmin generation in vivo in disseminated intravascular coagulation (DIC), plasma levels of thrombin‐antithrombin III (ATIII) complex (TAT) and plasmin‐alpha 2‐antiplasmin (a2AP) complex (PAP) were measured together with standard coagulation and fibrinolytic parameters in 80 patients with DIC. Both TAT and PAP were markedly elevated in patients with DIC. When plotted by the underlying disease categories, differences in the magnitude of the elevations of these complexes were recognized among groups. Patients with acute promyelocytic leukemia (APL) had the highest PAP, the lowest TAT/PAP ratio, low a2AP, and low fibrinogen, indicating that the most excessive fibrinolysis can occur in APL. Similar profiles, although less marked, were observed in patients with other leukemias and vascular diseases. Patients with sepsis showed the highest TAT/PAP ratio and the lowest PAP with no decrease in a2AP or fibrinogen, demonstrating a relatively impaired fibrinolysis. Patients with cancer had a relatively high TAT and high TAT/PAP ratio. In addition, both TAT and PAP were markedly elevated in patients with shock. From these, it was suggested that, although laboratory manifestations in DIC are extremely variable from patient to patient, underlying disorders are, at least in part, responsible for the observed variations. Recognition of this variable activation of coagulation and fibrinolysis would be helpful for the proper management of patients with DIC.
The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia
Authors: Benbassat Jochanan , Gilon Dan , Penchas Shmuel
Published: February 2007

The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myelod metaplasia (AMM). We searched the literature (Medline, 1970‐1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or transfusion requirements in patients with AMM. We employed formal decision analysis to determine the relative value of medical and surgical treatment of advanced AMM.

Results of data synthesis showed that splenectomy in AMM is associated with an operative mortality of 13.4% (95% confidence intervals (Cl): 9.5‐1 ‐17.2%), an early morbidity of 45.3% (Cl: 39.6‐51.1%), and a late morbidity of 16.3% (Cl: 9.9‐22.5%). Almost all patients with portal hypertension and painful splenomegaly, but only about half of those with thrombopenia and anemia were reported to have experienced relief in their symptoms or signs after splenectomy. We found no evidence that splenectomy affects survival in AMM.

We concluded that splenectomy in advanced AMM is a palliative procedure that carries a substantial risk. It may be considered for symptomatic patients after they have been informed about the operative mortality, morbidity, and chances of palliation. Decisions about treatment of advanced AMM should be guided predominantly by the patient's preferences.
Decreased tetranectin in multiple myeloma
Authors: Nielsen Henrik , Clemmensen Inge , Nielsen Hans J. , Drivsholm Aage
Published: February 2007

Tetranectin, a recently described human protein widely distributed in the body and with possible importance for cell growth and differentiation, has previously been observed to be decreased in patients with solid malignant tumors. In patients with multiple myeloma, either untreated or previously treated, serum levels were found to be significantly reduced. A negligible interindividual variation was observed. Levels of serum tetranectin were not correlated with serum albumin, hemoglobin, or M‐component. Low levels of tetranectin may be related to the growth and spread of malignant cells or reflect a general catabolic state of chronic disease.
Brief report: Pure red cell aplasia following pernicious anemia
Authors: Dan Kazuo , Ito Toshiharu , Nomura Takeo
Published: February 2007

A rare case of pure red cell aplasia (PRCA) in association with pernicious anemia is reported. A 40‐year‐old man presented with typical clinical and laboratory features of pernicious anemia and received intramuscular injections of vitamin B12, with satisfactory response. Anemia recurred 6 months later despite continued therapy, and the patient was noted to have PRCA, which was treated successfully with two courses of high‐dose bolus methylprednisolone therapy. His peripheral mononuclear cells before the therapy suppressed colony formation of early erythroid precursors (BFU‐E) from normal bone marrow; such a suppressive effect was not found after recovery from anemia.