bluuebunny/crossref_metadata_2025 huggingface dataset:
Evidence for rapid rapid mobilization of platelets from the spleen during intensive plateletpheresis
Authors: Lee Edward J. , Schiffer Charles A.
Published: February 2007

The role of the spleen as a reservoir capable of adding platelets to the circulating blood cells has been well documented in animals but poorly documented in humans. As part of a program of platelet cryopreservation, we have intensively plateletpheresed a group of patients with acute leukemia in remission who had undergone splenectomy. The results of the 64 plateletphereses in these 11 patients (average platelet yield 4.2 × 1011 ± 2.4, range 1.2‐12.4) were compared with 50 consecutive plateletphereses in nonsplenectomized leukemia patients (average yield 4.9 × 1011 ± 3.2, range 2.1‐18.3) and 50 consecutive plateletphereses in normal donors (average yield 3.8 × 1011 ± 1.4, range 1.7‐7.6). There was no difference in the prepheresis platelet counts among the three groups. Plateletphereses were done in 1.5‐3 hr by intermittent flow differential cell centrifugation technique. The expected platelet yield was calculated by multiplying the pre‐platelet count –(pre‐hct/post‐hct × post plt count) by the estimated blood volume (70 ml/per kg body weight). The mean ratios of the observed platelet yield to expected platelet yield were similar for the nonsplenectomized leukemia patients (mean ratio = 1.32 ± 0.50, range 0.73‐3.04) and normal donors (mean ratio = 1.31 ± 0.49, range 0.52‐2.9), implying mobilization of platelets from outside of the blood pool. In contrast, in the splenectomy group, the mean ratio (0.76 ± 0.32, range 0.31‐2.9) was significantly lower (p < 0.001). These data indicate that there is replenishment of the circulating platelet pool by the spleen in response to the rapid removal of platelets.
Treatment of patients with acute nonlymphocytic leukemia in relapse: A leukemia intergroup study
Authors: Goldberg J. , Grunwald H. , Vogler W. R. , Winton E. , Browman G. , Walker I. , Benger A. , Miller K. B. , Rai K. , Gottlieb A. J. , Brennan J. K. , Bennett J. M. , Chervenick P. , Joyce R. , Azarnia N. , Priore R. L. , Preisler H. D.
Published: February 2007

Forty patients with acute nonlymphocytic leukemia (ANLL) in first relapse were treated at eight member institutions of the Leukemia Intergroup with a 10‐day continuous intravenous infusion of cytosine arabinoside and an anthracycline antibiotic administered on days 1, 2, and 3. Twenty of the 40 patients achieved a complete response. Seven of the patients who did not enter remission were drug‐resistant failures, while 13 patients failed to enter remission for reasons other than persistent leukemia. Pretreatment parameters such as age, presence of infection, platelet count, and liver function tests were important predictors of survival. The percent bone marrow cellularity, the percent circulating abnormal (leukemic) cells, and the height of the white blood cell count prior to treatment were helpful in distinguishing patients who would enter remission from those who would not enter remission because of persistent leukemia.
Recurring thromboembolic disease and pulmonary hypertension associated with severe hypoplasminogenemia
Authors: Lottenberg Richard , Dolly F. Ray , Kitchens Craig S.
Published: February 2007

In a patient with pulmonary hypertension and a history of recurrent venous thrombosis, plasma concentrations of all known coagulant and inhibitor proteins were normal except for severe deficiency of plasminogen. Repeated analyses showed the circulating plasma plasminogen level to be 30% of normal by either functional or immunologic methods. We sought evidence for either increased activation of plasminogen or for dysplasminogen. There was no evidence for the former. Purified plasminogen studies disclosed a normal number of active sites and normal activation. Generated plasmin had normal catalytic activity. Isoelectric focusing disclosed normal distribution of isoforms. Affinity chromatography with lysine‐sepharose showed the presence of the two variant forms; however, an increased proportion of the protein eluted in the first peak. Danazol administration induced an increase in circulating plasminogen, but the differences in affinity chromatography elution profile remained. We conclude that this patient has a deficiency of normally functioning plasminogen, probably due to decreased synthesis.
Perfluorocarbon compounds: Effects on the rheological properties of sickle erythrocytes in vitro
Authors: Reindorf C. A. , Kurantsin‐Mills J. , Allotey J. B. , Castro O.
Published: February 2007

The effects of oxygenated perfluorotributylamine (Fluosol‐43) on the rheological properties of sickle (HbSS) erythrocytes have been determined by means of microviscometry and positive pressure cell filtration. Incubation of deoxygenated sickled erythrocytes (pO2 = 30 mmHg) with oxygenated Fluosol‐43 reduced the percentage of sickled erythrocytes from about 63 to 33%. Deoxygenation of 40% suspension of sickle erythrocytes in autologous plasma increased the viscosity by about 160% at shear rate of 1.15 sec−1. Incubation of the deoxygenated sickled erythrocytes with oxygenated Fluosol‐43 significantly reduced the viscosity at the low shear rates. Filtration of 0.2% suspension of deoxygenated sickle erythrocytes through capillarysized Nuclepore filters showed high resistance at low flow rates. Oxygenated Fluosol‐43 increased the deformability of HbSS erythrocytes and thereby reduced the resistance at flow rates less than 1 ml/min. These data suggest that perfluorocarbons may be useful in reducing the propensity of hemoglobin S polymeriztion and sickling and thereby prevent tissue infarction in vaso‐occlusive crisis. Therefore, the concept of examining the potential application of perfluorochemicals for alleviating severe vasoocclusive events may be useful.
Transfusion‐associated AIDS
Authors: Perkins Herbert A.
Published: February 2007

Acquired Immunodeficiency Disease (AIDS) may be transmitted by transfusion of blood components, although the risk remains extremely small. This report reviews the evidence for transmission by transfusion, the risk in several different situations, and the steps blood banks have taken to prevent transfusion of blood from donors at risk for AIDS. A test for the antibody to the virus responsible for AIDS (HTLV‐III) will shortly be in routine use in blood banks, but the frequency with which the results will be falsely negative or falsely positive in a blood donor population remains to be established.
A case of factor V inhibitor
Authors: Chong Lai‐Lu , Wong Yuk‐Cheung
Published: February 2007

A 57‐year‐old married Chinese male without a family history of bleeding disorder was presented with severe hemorrhagic tendency and was subsequently found to be suffering from an acquired inhibitor against coagulation factor V. The prolonged prothrombin time and activated partial thromboplastin time could not be corrected by the addition of normal plasma. Subnormal value of factor V level was noted accompanied with an abnormal platelet factor III availability test. With specific antisera and staphylococcal protein A, the inhibitor was characterized as an IgG(lambda) antibody. The hemorrhagic tendency and abnormal laboratory data were corrected after treating the patient with platelet concentrate transfusion and cyclophosphamide.
Therapy of chronic relapsing thrombotic thrombocytopenic purpura with prednisone and azathioprine
Authors: Moake Joel L. , Rudy Christine K. , Troll Joseph H. , Schafer Andrew I. , Weinstein Mark J. , Colannino Noreen M. , Hong Suchen L.
Published: February 2007

As a 51‐year‐old woman recovered from an initial acute episode of thrombotic thrombocytopenic purpura (TTP), her plasma was found to contain unusually large von Willebrand factor (vWF) multimers. Clinical, hematological, and vWF studies of her siblings and children were normal. The unusually large vWF forms were presumably derived from endothelial cells, persisted in her plasma after recovery, and were associated with recurrent episodes of TTP during the subsequent 6 months. After the last episode of relapse they disappeared from her plasma following 3 ½ weeks of therapy with prednisone and did not return during 17 months of treatment with prednisone and/or azathioprine. She is now receiving no drugs, has normal plasma vWF forms, and has not had any more episodes of TTP. We conclude that our patient had an acquired defect in the conversion of unusually large vWF multimers derived from endothelial cells to the somewhat smaller vWF forms usually present in circulation. The defect may have been immune‐mediated, because it was eliminated during therapy with immunosuppressive drugs.
Clonal development from a progenitor with restricted differentiative expression in acute lymphoblastic leukemia
Authors: Ferraris Anna Maria , Canepa Letizia , Massimo Luisa , Dini Giorgio , Broccia Giorgio , Meloni Tullio , Forteleoni Gavino , Melani Cecilia , Gaetani Gian Franco
Published: February 2007

Three patients with acute lymphoblastic leukemia (ALL) and heterozygous for the Mediterranean variant of glucose‐6‐phosphate dehydrogenase (G6PD) have been investigated with the 2‐deoxyglucose‐6‐phosphate (2dG6P) method to determine the number and type of progenitor cells in which the disease arose. A monoclonal origin was established for the lymphoblasts, while the other hemopoietic cell lines were not involved in the leukemic process.
Release of granulocyte‐macrophage colony‐inhibiting activity by normal human postthymic precursor cells
Authors: López‐Karpovitch X. , Padrós‐Semorile M. R. , Rojas R. , Martínez‐Sánchez L.
Published: February 2007

Seven normal human peripheral blood cell fractions (buffy coat, mononuclear cells, non‐T, T, Fc‐IgM receptor‐depleted T‐lymphocyte, Fc‐IgG receptor‐depleted T‐lymphocyte, and autologous rosette‐forming T‐cell‐depleted T‐lymphocyte subpopulations) treated with phytohemagglutinin (PHA) were examined for the production of granulocyte‐macrophage colony‐stimulating activity (CSA). It was found that medium conditioned by a T‐lymphocyte subpopulation depleted of autologous rosette‐forming T‐cells (Tar cells, a postthymic precursor subpopulation that inhibits Ig synthesis) stimulated colony‐forming units of granulocyte and macrophages (CFU‐GM) to a greater extent than did the other conditioned media (CM) analyzed. Based on this finding, CM from an enriched Tar subpopulation was prepared and thus showed that PHA‐treated Tar cells release a factor capable of inhibiting CFU‐GM growth. The inhibitory activity of this factor persisted after heat inactivation, suggesting that cause of the colony‐inhibiting activity (CIA) is other than interferon. Further studies revealed that Tar‐derived inhibitory factor acts either directly upon CFU‐GM or via monocytes/macrophages (Mϕ/Ma), enhancing CIA, and not the level of CSA production by Mϕ/Ma. The overall data are interpreted as demonstrating the presence of CIA in a specific T‐lymphocyte subpopulation that may represent a new relationship between lymphocytic and myelocytic systems in the human.
A subpopulation of suppressor cells in Richter's syndrome with both monocytic and T‐lymphocytic characteristics
Authors: Lahat N. , Aghai E. , Quitt M. , Nir E. , Froom P.
Published: February 2007

We evaluated T‐lymphocyte functions in the peripheral blood of a patient with B‐cell chronic lymphocytic leukemia after transformation to large cell lymphoma (Richter's syndrome). A subpopulation of E‐rosette adherent cells were found with T‐lymphocytic surface markers (OKT3+/8+/4+), monocytic characteristics (latex ingestion, nonspecific esterase staining), and suppressor activity. In contrast to the patient's nonadherent T‐cells, this subpopulation suppressed PHA proliferation of autologous lymphocytes, pokeweed mitogen (PWM)‐induced proliferation of normal non‐T cells, and a mixed lymphocyte reaction. Further studies are warranted in patients with Richter's syndrome, in order to determine the frequency and significance of our findings.
An increased calcium accumulation in ATP‐depleted red cells of the patients with paroxysmal nocturnal hemoglobinuria
Authors: Shimoda Masaoo , Yawata Yoshihito
Published: February 2007

A marked increase in calcium (Ca) uptake (85.5 ± 39.0 nmoles/ml RBC/2 hr) was observed in ATP‐depleted red cells of patients with paroxysmal nocturnal hemoglo‐binuria (PNH) as compared to ATP‐depleted normal red cells (8.0 ± 2.0). The extent of increased Ca uptake in the PNH red cells was related to the extent of increased sensitivity to complement, as judged by the sugar water test (p < 0.001) and the acidified serum test (p < 0.001), and inversely correlated to the decreased activity of red cell acetylcholinesterase (p < 0.01). The results may indicate that increased calcium uptake in PNH red cells is a possible intrinsic defect of the red cell membranes in the disorder.
Transferrin sialic acid contents of patients with hereditary hemochromatosis
Authors: Tsan Min‐Fu , Scheffel Ursula
Published: February 2007

Recent evidence suggests that asialotransferrin may enhance hepatic uptake of trivalent metal ions through the asialoglycoprotein‐receptor pathway. In hereditary hemochromatosis, there is preferential uptake of iron by hepatocytes. We purified transferrin from two patients with this disease. Their transferrins had normal electrophoretic patterns and contained normal amounts of sialic acid. We conclude that the preferential uptake of iron by hepatocytes in hereditary hemochromatosis is not due to the presence of asialotransferrin in their plasma.
The incidence of thrombotic and hemorrhagic disorders in association with extreme thrombocytosis: An analysis of 129 cases
Authors: Buss David H. , Stuart John J. , Lipscomb Gary E.
Published: February 2007

We have presented a retrospective study of 129 patients with platelet counts of 1,000 × 109/L or more—72 with myeloproliferative disorders (MPD), and 57 with reactive thrombocytosis (RT)—in an effort to determine the incidence of thrombohemorrhagic complications. Thrombotic disorders occurred with approximately equal frequency in the two groups, being found in three patients (4%) with MPD and three (5%) with RT when the platelet count was over 1,000 × 109/L and in 11% of the patients in each group when the platelet count was less than 1,000 × 109/L. In contrast, bleeding manifestations were more common in MPD, where 36% of patients were affected on one or more occasions versus only 4% of those with RT. However, bleeding was generally not severe, with only six patients requiring transfusions, and three of these patients had been receiving aspirin or corticosteroids when they developed severe gastrointestinal bleeding.
Adult Niemann‐Pick disease masquerading as sea blue histiocyte syndrome: Report of a case confirmed by lipid analysis and enzyme assays
Authors: Landas Steve , Foucar Kathy , Sando Gloria N. , Ellefson Ralph , Hamilton Henry E.
Published: February 2007

We present the clinical, pathologic, and metabolic findings of an adult woman with debilitating coronary artery disease and hepatosplenomegaly who was discovered to have multiorgan infiltration by sea blue histiocytes. A diagnosis of sea blue histiocyte (SBH) syndrome was made and no further workup performed. The patient suffered from progressive heart failure and sepsis following coronary artery bypass surgery and died 9 months after presentation. Tissues examined at autopsy showed pronounced infiltrates of both granular sea blue histiocytes and foamy, vacuolated histiocytes, which were morphologically compatible with Niemann‐Pick cells. Ultra‐structural examination of these cells revealed lamellar myelin‐like figures as described in Niemann‐Pick (N‐P) disease. Fibroblast enzyme assay studies and liver lipid analyses performed after the patient's death revealed pronounced sphingomyelinase deficiency and a lipid profile diagnostic of N‐P disease, type B. This case adds further support to the claim that some cases of apparent SBH syndrome actually represent a type of N‐P disease.
Post‐transfusion purpura associated with alloimmunization against the platelet‐specific antigen, baka
Authors: Keimowitz Rudolph M. , Collins Janice , Davis Kent , Aster Richard H.
Published: February 2007

Post‐transfusion purpura (PTP) with severe thrombocytopenia occurred eight days after transfusion in a 28‐year‐old woman and responded to treatment with prednisone and plasma exchange. In contrast to nearly all previously studied cases of PTP, the patient's platelets were PIAI‐positive and anti‐PIAI antibody could not be detected in serum obtained during the thrombocytopenic episode. Her serum was found to contain an antibody specific for a recently described platelet‐specific alloantigen, Baka, in addition to multiple HLA‐specific antibodies. The patient's platelets, typed following recovery, were Baka‐negative. These findings indicate that post‐transfusion purpura can occur in association with alloimmunization to platelet‐specific antigens other than PIAI.

In performing the serologic studies, a close relationship and possible identity between Baka and another recently reported platelet antigen, Leka, was observed. A method for analyzing mixtures of cytotoxic platelet‐reactive antibodies without separating the individual antibodies is described.
The distribution of lysozyme, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin in normal hematopoietic cells and in myeloid leukemias: An lmmunoperoxidase study on cytocentrifuge preparations, smears, and paraffin sections
Authors: Krugliak Lena , Meyer Paul R. , Taylor Clive R.
Published: February 2007

Blood and bone marrow samples from 20 individuals with reactive conditions and 26 cases of acute and chronic myeloid leukemias were tested for the presence of lysozyme, alpha‐1‐antitrypsin (α‐1‐AT), and alpha‐1‐antichymotrypsin (α‐1‐ACT). We compared the reactivity of samples in smears, cytocentrifuge preparations, and paraffin sections.

Lysozyme, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin were found only in polymorphonuclear leukocytes and monocytes and their precursors. Lymphocytes, Erosetting cells, Con A‐activated lymphocytes, natural killer (NK) cells, red blood cells, erythroblasts, and megakaryocytes were consistently negative.

Leukemic myeloblasts showed definite reactivity for both alpha‐1‐antitrypsin and α‐1‐ACT, but not for lysozyme. By contrast, lysozyme was present in poorly differentiated leukemic monoblasts, while alpha‐1‐antitrypsin and alpha‐1‐antichymotrypsin showed only weak reactivity. More mature myeloid and moncytic cells showed positive staining for all three antigens tested with differences in staining distribution and intensity.

In four cases of chronic myeloid leukemia (CML), circulating mature polymorphonuclear leukocytes were deficient in both lysozymne and alpha‐1‐antitrypsin.

The use of lysozyme, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin identifies normal and leukemic cells of the myeloid‐monocytic series at all stages of maturation and is applicable to a variety of sample preparations.
Successful renal transplantation for epstein syndrome
Authors: Alving Barbara M. , Tarassoff Peter G. , Moore Jack , Leissenger Cindy A. , Fernandez‐Bueno C.
Published: February 2007

Successful cadaveric renal transplantation was accomplished in a patient with Epstein syndrome, a triad of macrothrombocytopenia, partial high‐frequency hearing loss, and nephritis, which often progresses to complete renal failure. The success of the transplant demonstrates that the macrothrombocytopenia which occurs in this syndrome is not a contraindication to aggressive management of end‐stage renal disease.
Hemoglobin knossos: A clinical, laboratory, and epidemiological study
Authors: Fessas Phaedon , Loukopoulos Dimitris , Kokkinou Stavroula , Papasotiriou Yannis , Karaklis Aris
Published: February 2007

Hb Knossos is a β‐chain variant (β 27 Ser ± Ala) that is unrecognizable by conventional separation methods but detectable by globin electrophoresis on urea‐Triton X‐acrylamide gels or by IEF. Hb Knossos is characterized by reduced synthesis and by interaction with β‐thalassemia, in which the double heterozygotes display typical features of thalassemia intermedia. The present paper summarizes the salient genetic, clinical, and biochemical characteristics of five such cases hitherto identified in three families along with the same features on 12 heterozygous Hb Knossos carriers. Hb Knossos displays a slightly decreased oxygen affinity; this factor may compensate in part for the severe anemia of the double heterozygotes. Hb Knossos is relatively rare in our population, since a prospective survey on 610 individuals has failed to disclose any heterozygotes. However, the mutation appears to have spread over the Mediterranean countries and may be more common elsewhere.
T‐cell chronic lymphocytic leukemia with a helper/inducer membrane phenotype: A distinct clinicopathologic subtype with a poor prognosis
Authors: Witzig Thomas E. , Phyliky Robert L. , Li Chin‐Yang , Homburger Henry A. , Dewald Gordon W. , Handwerger Barry S.
Published: February 2007

T‐cell chronic lymphocytic leukemia (T‐CLL) accounts for about 2% of the various types of CLL and can be subtyped into helper/inducer (h/i) and cytotoxic/suppressor (c/s) cell membrane phenotypes. Seven patients with CLL were shown to have T‐CLL with a h/i cell membrane phenotype; four with monoclonal antibody reagents and three by demonstration of the E‐rosette receptor and focal acid alpha naphthyl acetate esterase activity. The clinical courses, treatment responses, and laboratory findings of these seven patients were reviewed to determine the prognosis and unique clinicopathologic features of this subtype. Two patients presented with skin rashes, and five were diagnosed during evaluation for other medical problems. Initially, four patients had splenomegaly and two had lymphadenopathy, but none of the patients had hepatomegaly. Morphologic examination revealed uniform, small lymphocytes in three patients, and the lymphocytes had nuclear indentations in four patients. Sera from the three patients tested were negative for antibody to the human T‐cell leukemia/lymphoma virus I. Peripheral blood mononuclear cells from one patient showed normal interleukin‐2 production and lacked antibody‐dependent cell‐mediated cellular cytotoxicity and natural killer activity. Cytogenetic analysis was done on one patient, revealing an abnormal clone with several chromosomal abnormalities, including an X; 14 translocation with a break point at 14q11. All patients required chemotherapy, and all died a median of 21 months from the time of diagnosis. The findings in these patients, in addition to those in 31 patients described in the literature, indicate that h/i T‐CLL is associated with a poor prognosis and has distinct clinical and pathologic features that separate it from c/s T‐CLL, adult T‐cell leukemia/lymphoma, the cutaneous T‐cell lymphomas, and B‐CLL.
von willebrand disease type IIC with different abnormalities of von willebrand factor in the same sibship
Authors: Batlle J. , Fernandez M. F. Lopez , Lasierra J. , Villamor A. Fernandez , Berges C. Lopez , Borrasca A. Lopez , Ruggeri Z. M. , Zimmerman T. S.
Published: February 2007

A family with von Willebrand disease has been identified in which different members of the same sibship exhibit different abnormalities of von Willebrand factor (vWF). The two most severely affected sibs (bleeding time over 20 min) had abnormalities of vWF similar to those seen in type IIC. The smallest detectable multimer was increased and the triplet structure of individual multimers was replaced with a single band. The largest multimers could not be detected and there were relatively more small multimers than intermediate sized forms. vWF antigen (vWF:Ag) was decreased to 12.5‐17% by electroimmunoassay (EIA) and to 3.2‐5.5% by immunoradiometric assay (IRMA). In the less severely affected sibling (bleeding time 12.5 min) there was a similar relative increase in the smallest detectable multimer. However, the larger multimers were present and the relative concentration of large to small multimers was similar to normal. The triplet structure was altered in that the relative proportion of satellite bands to the central predominant band was decreased. vWF:Ag concentrations were moderately decreased (40‐80% by EIA and 25‐35% by IRMA). The father and grandfather showed a vWF multimeric pattern similar to the less severely affected sibling but there was no decrease in vWF:Ag concentration and their bleeding times were normal. These observations suggest that the interplay of several genetic factors is responsible for the expression of von Willebrand disease in this family.
Hereditary antithrombin III deficiency: Case report and review of recent therapeutic advances
Authors: Miller Nancy , Hultin Mae B. , Gounder Mohanambal , Zarrabi M. Hosein
Published: February 2007

We report on a newly diagnosed family with hereditary antithrombin III deficiency, with thromboembolic complications in the propositus. Both the propositus and his asymptomatic sister had decreased plasma levels of antithrombin III antigen and activity (28‐52% of normal with good agreement between functional and immunologic assays). The propositus developed deep venous thrombosis, followed by massive pulmonary emboli despite heparin therapy and was treated with streptokinase and heparin with excellent results. Shortly thereafter, small bowel obstruction required surgical intervention, and antithrombin III concentrate, recently available in the United States as an investigational new drug (I. N. D.), was administered with no postoperative thrombotic complications. He was subsequently asymptomatic while on warfarin prophylaxis but twice developed venous thrombosis when he failed to take warfarin. The addition of danazol therapy led to a sustained rise in the antithrombin III level. Each of these therapeutic approaches is discussed and the literature reviewed with emphasis on the newer agents‐streptokinase, antithrombin III concentrate, and danazol.
Hematologic malignancy in sickle cell disease: Report of four cases and review of the literature
Authors: Stricker Raphael B. , Linker Charles A. , Crowley Timothy J. , Embury Stephen H.
Published: February 2007

Hematologic malignancy has rarely been reported in adults with sickle cell disease. We describe four sickle cell patients (two with hemoglobin SC, two with hemoglobin SS) who developed hematologic malignancy (acute myeloblastic leukemia, multiple myeloma, malignant histiocytosis, and Hodgkin's disease). Three of the cases represent the first adult association between SC or SS hemoglobinopathy and the particular malignancy involved. Sickle hemoglobin does not appear to exert a protective effect against childhood hematologic malignancies, suggesting that better survival in sickle cell disease may be accompanied by an increased incidence of hematologic neoplasms in adulthood. Karyotypic analysis revealed alterations of chromosome 5 in two sickle cell patients with leukemia, raising the possibility of a chromosomal link between the two diseases. Further epidemiologic and cytogenetic studies are needed to define the relationship between hematologic malignancy and sickle cell disease.
Post‐transfusion purpura: A report of five patients and a review of the pathogenesis and management
Authors: Vogelsang Georgia , Kickler Thomas S. , Bell William R.
Published: February 2007

We report five cases of post‐transfusion purpura. Despite having profound thrombocytopenia, their management consisted only of steriod administration if serious bleeding was present. In one patient without bleeding, no specific therapy was given. This patient recovered uneventfully. Two patients died. One of these died from complicating illnesses rather than bleeding. The other suffered brain death, the result of a ruptured cerebral aneurysm. Four patients were PLA1 negative. The fifth patient had an antibody to an unidentified platelet‐specific antigen.
Thrombotic thrombocytopenic purpura subsequent to acute myelogenous leukemia chemotherapy
Authors: Byrnes John J. , Baquerizo Hernan , Gonzalez Michael , Hensely George T.
Published: February 2007

A woman in complete remission from acute myeloblastic leukemia developed thrombotic thrombocytopenic purpura (TTP) subsequent to the third intensive consolidation cycle of cytosine arabinoside and daunorubicin chemotherapy. The constellation of clinical manifestations indicative of TTP were recognized only in retrospect, as they were initially attributed to more usual complications of bone marrow‐ablative chemotherapy. The manifestations, probably fueled by numerous red cell and platelet transfusions, increased at the time of recovery of hematopoiesis. At postmortem examination, characteristic microvascular lesions were found in most organs. Similar thrombotic microangiopathy has been described with mitomycin‐based chemotherapy regimens and with the combination of cisplatin, vinblastine, and bleomycin. Successful management of this serious complication of chemotherapy requires increased awareness and earlier recognition.
Common variable hypogammaglobulinemia in pregnancy: Treatment with high‐dose immunoglobulin infusions
Authors: Madsen Douglas L. , Catanzarite Valerian A. , Varela‐Gittings Frances
Published: February 2007

Pregnancy outcomes when the mother has common variable hypogammaglobulinemia are poor unless immunoglobulin levels are returned to normal or near‐normal range. Intravenous immunoglobulin infusions are the favored mode of therapy. Here, we report the fifth patient so treated. Weekly doses of 200 mg/kg during the third trimester were required. A healthy term baby was delivered and the postpartum course was uncomplicated.
Induction of differentiation in the human leukemia cell line SPI‐802: Morphological, immunological, and isoenzymatic changes
Authors: Drexler Hans Guenter , Gaedicke Gerhard , Komiyama Atsushi , Minowada Jun
Published: February 2007

The phorbolester 12‐0‐tetradecanoylphorbol 13‐acetate (TPA) was used for the induction of differentiation in cells of the human leukemia cell line SPI‐802. The cellular morphology, surface marker antigen expression, and isoenzyme profiles of four enzymes (carboxylic esterase, acid phosphatase, hexosaminidase, and lactate dehydrogenase) served as parameters for monitoring the induced phenotypical changes. TPA led to distinct alterations of the morphology and significantly affected the growth rate with cessation of cell proliferation. No major increase in the number of nitro blue tetrazolium‐positive cells or aggregation of cells, phagocytosis of latex beads, adherence to plastic surface, or development of pseudopodia were observed. As TPAtreated SPI‐802 cells remained negative for these markers of the monocyte‐macrophage complex, it can be concluded that the cells did not differentiate into monocytes and macrophages. The immunological marker profile based on testing of 55 monoclonal antibodies, terminal deoxynucleotidyl transferase and two erythrocyte rosette tests indicated a differentiation of SPI‐802 cells along the granulocytic cell lineage. This was confirmed by isoenzyme analysis, especially that of carboxylic esterase. An isoenzyme specific for monocytes and macrophages was not detected. In earlier studies it was found that SPI‐802 cells produce hemoglobin upon exposure to TPA or hemin. This latter observation and the present results suggested a comparison with the two erythroleukemia cell lines K‐562 and HEL. SPI‐802 cells appear to have the potential to differentiate along several cell lineages.
Unexplained periparturient thrombocytopenia
Authors: Freedman J. , Musclow E. , Garvey B. , Abbott D.
Published: February 2007

Since the advent of routine automated platelet counting we have observed unexplained periparturient thrombocytopenia (PPT) in an unexpected number of periparturient women, ie, during labor or within 24 hr postpartum. Mean ± SD platelet count in 686 random blood donors was 236 ± 50 ± 109/L and 1.02% had a platelet count < 136 ± 109/L; in 2,204 random prenatal and postpartum women mean count was significantly higher (275 ± 86 ± 109/L; p < 0.001). Of 1,621 periparturient women, 74 (4.6%) had unexplained PPT (mean ± SD platelet count 122 ± 24 ± 109/L, range 21‐135 ± 109/L, N = 74). Platelet count in PPT usually rose to normal within 1 week of delivery; in 10% thrombocytopenia persisted > 6 months. PPT occurred in successive pregnancies with normal intervening platelet counts. Nine of 34 newborns of mothers with PPT were thrombocytopenic; there was no correlation between mother's and baby's platelet counts. In no case of PPT was there excessive bleeding in mother or infant. Positive indirect platelet radioactive antiglobulin tests (PRAT) were seen in 11% of normal postpartum women and in 90% of 22 women with PPT; 65% of the positive tests in PPT were due to reactions with anti‐C3 only. In contrast, pregnant women with autoimmune thrombocytopenic purpura (AITP) had positive PRAT primarily because of anti‐IgG ( ± anti‐C3); only 10% were positive only with anti‐C3. Results were concordant in all of eight women with PPT tested by both indirect and direct PRAT. Amount of C3 bound per platelet in direct or indirect PRAT was not predictive of degree of thrombocytopenia, but there was correlation of fg C3 per platelet detected by the two assays in individual patients (r = 0.8). Mean levels of serum C3, C4, and factor B in women with PPT did not differ from normal; individual patients had abnormal serum complements but no characteristic pattern was observed. Increased immune complexes were observed in 6% of normal subjects and 33% of women with PPT.

Etiology and mechanism of PPT is unclear. Despite lack of clinical evidence in women with PPT of syndromes associated with increased platelet destruction, the presence of preeclampsia cannot be absolutely excluded. Similarly, although the pattern of antiglobulin sensitization in PPT differed markedly from that seen in AITP, autoimmune disorder cannot be excluded. Alloantibodies did not appear to be responsible for PPT. While PPT is usually benign, some patients had a markedly reduced platelet count. Recognition of the phenomenon may be important in obstetrics.
Activation of platelet function in Fabry's disease
Authors: Igarashi Takashi , Sakuraba Hitoshi , Suzuki Yoshiyuki
Published: February 2007

Increased platelet aggregation and high plasma concentration of β‐thromboglobulin were observed in hemizygotes and heterozygotes of Fabry's disease. Carbamazepine and phenytoin administered for the treatment of pains in these patients showed no significant effect on platelet aggregation. No activation of platelets was observed after the addition of ceramide trihexoside, the storage lipid of this disease. Mitral valve prolapse was found in eight of 12 patients. Although the pathogenesis of platelet activation and mitral valve prolapse are not known, the platelet activation could be an early indicator and an accelerating factor of thromboembolic vascular change in this disease.
Multimeric pattern discrepancy between platelet and plasma von Willebrand factor in type IIC von Willebrand disease
Authors: Batlle J. , Fernandez M. F. Lopez , Villamor A. Fernandez , Berges C. Lopez , Zimmerman T. S.
Published: February 2007

von Willebrand factor (vWF) from platelet lysate and plasma, collected in the presence of protease inhibitors, was studied in two patients with type IIC von Willebrand disease (vWD). Platelet and plasma vWF showed the smallest multimer increased, but the latter had a repeating single band whereas the former had a repeating “doublet.” This platelet‐plasma discrepancy observed for the first time in these patients suggests that the repeating “doublet” or single band described in other type IIC patients represent minor subgroups of type IIC vWD.
Impaired immunoglobulin synthesis in multiple myeloma: A B‐cell dysfunction
Authors: Carter A. , Silvian I. , Tatarsky I. , Spira G.
Published: February 2007

Peripheral blood samples collected from normal individuals and patients with benign monoclonal gammopathy and multiple myeloma were separated and assayed for their T‐ and B‐cell subpopulations as well as immunoglobulin (IgG, IgM) synthesis in vitro. To rule out any functional or quantitative alterations related to therapy, only newly diagnosed multiple myeloma patients and subjects with benign monoclonal gammopathy were included in our study. Multiple myeloma patients were further subdivided into two groups. Group A consisted of patients with low and intermediate tumor burdens, while group B included patients with high tumor mass. B‐ and T‐cell subset analysis revealed an abnormal ratio between B/T and OKT4+/OKT8+ lymphocytes compared to healthy controls. These alterations were especially prominent in group B multiple myeloma. Immunoglobulin synthesis was studied in pokeweed‐mitogen‐stimulated lymphocyte cultures. The results indicate that the failure to synthesize and secrete immunoglobulin resides within the B‐cell lineage and is probably due to a functional B‐cell defect. T‐cell immunoregulatory functions seem to be unaffected in both multiple myleoma and benign monoclonal gammopathy patients.
Two new glucose‐6 phosphate dehydrogenase (G6PD) variants associated with hemolytic anemia: G6PD amman‐1 and G6PD amman‐2
Authors: Karadsheh Naif S. , Awidi A. S. , Tarawneh M. S.
Published: February 2007

Two glucose‐6‐phosphate dehydrogenase (G6PD) variants were investigated. G6PD Amman‐1 was partially purified from the red cells of a patient suffering from recurrent jaundice and spontaneous episodic attacks of severe hemolysis in the absence of oxidant drugs, infection, or fava beans. The enzymatic characteristics of G6PD Amman‐I were markedly reduced activity, fast eletrophoretic mobility, slightly increased km for NADP, normal km for G‐6‐P, normal heat stability, normal utilization of substrate analogues 2‐deoxy G‐6‐P and deamino‐NADP, and a monophasic pH curve with a peak at 8.5 to 9.3. The second variant, G6PD Amman‐2, was partially purified from the red cells of a patient suffering from recurrent jaundice with episodic mild hemolysis caused by infection or unknown factors. G6PD Amman‐2 characteristics were severely reduced activity, slow electrophoretic mobility, normal km for NADP, decreased km for G‐6‐P, decreased heat stability, increased utilization of substrate analogues, and a monophasic pH curve with a narrow peak at pH 9.5. The red cell level of reduced glutathione was markedly decreased with twofold increase in the activity of glutathione reductase in the patient with G6PD Amman‐2.
Interaction between leukocytes and serum plasminogen: An essential mechanism in peripheral blood fibrinolytic activity
Authors: Ghezzo F. , Savoca P. , Vallero P. , Bellone G.
Published: February 2007

Intense fibrinolytic activity (FA) by granulocytes, mononuclear adherent cells, and natural killer (NK) cells was demonstrated by using 125I‐fibrin‐coated wells. This FA was greatly dependent on serum plasminogen, provided that serum inhibitors were destroyed by heating at 56°C. Using monoclonal antibodies, it was also observed that these leukocytes produce urokinase and that this enzyme is secreted during adhesion by mononuclear adherent cells and during the cytotoxicity reaction against neoplastic cells by NK cells. Since cell intactness is necessary for leukocyte‐serum interaction, stress is placed on the membrane function. We conclude that leukocytes are essential in peripheral blood FA and that transport of urokinase by circulating cells, inside their cytoplasm, could offset its lack of affinity for fibrin and thus avoid undesirable systemic effects.
Myeloid cell surface phenotype in myelodysplasia: Evidence for abnormal persistence of an early myeloid differentiation antigen
Authors: Baumann Michael A. , Keller Robert H. , McFadden Patrick W. , Libnoch Joseph A. , Patrick Catherine W.
Published: February 2007

A panel of monoclonal antibodies recognizing myeloid cell surface differentiation‐associated antigens was used to study the peripheral blood myeloid population of 23 patients with myelodysplastic syndromes (MDS) and 23 controls. A marker for immaturity, defined by the presence of the antigen recognized by the My9 antibody, was found to persist on the surface of mature neutrophils in a subgroup of MDS patients. Abnormal My9 positivity was concentrated primarily, but not exclusively, in previously described morphologically defined subgroups of MDS patients considered to be at highest risk for leukemic conversion. Longitudinal study of a larger number of patients will be required to test the hypothesis that abnormal persistence of My9 may have prognostic significance.
Sickle cell anemia associated with α‐thalassemia in Malaysian Indians
Authors: Lie‐Injo Luan Eng , Hassan Khalid , Joishy Suresh K. , Lim Mu Lan
Published: February 2007

The Indian rubber estate workers in Negri Sembilan, Malaysia, who originated from Orissa in India were found to have a high frequency of Hb S (Joishy SK, Hassan K: Clin Res 28:280, 1980). Unlike the usually severe clinical picture of sickle cell anemia seen in African and American blacks, the clinical picture of the disease in this population was mild and many have reached old age. We studied the leukocyte DNA of 12 patients with sickle cell anemia, ranging in age from 4 to 61 years and 30 sickle cell trait carriers, ranging in age from 7 to 63 years, for the presence of α‐globin gene deletions by gene mapping according to Southern (Southern EM: J Mol Biol 98:503, 1975), using α‐ and ζ‐globin gene probes obtained by nick translation of the α‐ and ζ‐globin genes cloned into plasmid. All 12 sickle cell anemia patients were found to have α‐thalassemia2 (α‐thal2), either in the homozygous or heterozygous condition. Of the Hb S trait carriers, six did not have α‐that2 or or‐thal1 and 24 had α‐thal2 (15 heterozygous, 9 homozygous). Seven of these Hb S trait carriers with α‐thal2 had an additional gene abnormality. Five of them had a fast‐moving Eco RI fragment 5.6 kb long that hybridized with ζ‐specific probe but not with α‐specific probe. An unusual DNA pattern of a different type was further found in the other two. Bgl II restriction analysis showed that the α‐thal2 was mostly of the rightward deletion α‐thal2 genotype. None of the sickle cell anemia patients and Hb S trait carriers had deletion type α‐thal1. The sickle cell anemia patients had very high levels of Hb F and low levels of Hb A2. The Hb S trait carriers with α‐thal2 had relatively low levels of Hb S.
Paravertebral extramedullary hematopoiesis associated with improvement of anemia in congenital dyserythropoietic anemia type II
Authors: Lugassy Gilles , Michaeli Joseph , Harats Noam , Libson Eugene , Rachmilewitz Eliezer A.
Published: February 2007

Thoracic masses resulting from extramedullary hematopoiesis developed in two sisters of Moroccan origin with congenital dyserythropoietic anemia type II (HEMPAS). In one patient, the diagnosis of extramedullary hematopoiesis was confirmed histologically. The appearance of extramedullary foci of hematopoiesis mimicking mediastinal tumors has not been previously described in HEMPAS. These masses result from persistent erythropoietic stimulation associated with chronic hemolytic anemia. In both patients, detection of the asymptomatic masses was preceded by normalization of hemoglobin levels. Thus unexpected correction of a chronic refractory anemia associated with the appearance of mediastinal masses might be the heralding manifestation of an effective extramedullary hemopoiesis.
Acute leukemia with mediastinal mass, lymphadenopathy, and monocytic precursor cells
Authors: Schwarzmeier Josef D. , Bettelheim Peter , Radaszkiewicz Thaddäus , Prischl Friedrich , Schwabe Michael , Fuhrmann Manfred
Published: February 2007

Cell marker analysis with monoclonal antibodies (MoAb) as well as differentiation studies with the chemical inducer TPA were used to identify a population of apparently lymphoid cells as monocytic precursor cells in a patient with acute leukemia. The initial manifestation of the disease with mediastinal mass and lymphadenopathy was followed by the appearance of small lymphocyte‐like blast cells in bone marrow (BM) and peripheral blood (PB). Although a lymph node biopsy revealed an infiltration with monoblasts, the leukemic cells in BM and PB were not classifiable. However, when the patient relapsed after chemotherapy with large monoblasts and again with morphologically lymphoid blast cells, the latter could be classified by treatment with TPA. After incubation with the inducer (12–48 hr) the cells became positive with the MoAB VIM‐D5, showed a strong reaction with α‐napthyl‐acetate‐esterase and developed macrophage like morphology, as well as phagocytic properties. During the terminal phase of the disease, small, lymphocyte‐like blast cells predominated. These cells could be classified by a panel of MoABs. They expressed myeloid determinants (VIM‐D5, VIM‐2, MY 9, VIM‐12, VIM‐13) but showed no reactivity with MoABs specific for lymphocytic cells.
Sialic acid of platelets and granulocytes in polycythemia vera
Authors: Streichman S. , Menasheoff D. , Tatarsky I. , Marmur A.
Published: February 2007

Previous studies have shown that erythrocytes of polycythemia vera (PV) patients have an increased content of sialic acid on their membrane compared with normal erythrocytes. This has been attributed to the presence of an abnormal clone known to proliferate in this disease. Since granulocytes and platelets originate from the same stem cell that is involved in this disease, it was of interest to see whether these cells also bear increased amounts of sialic acid compared with their normal counterparts. While normal values were found for granulocyte populations, elevated values were found for the platelet populations of PV patients.
Massive hemolysis following inhalation of volatile nitrites
Authors: Bogart Lee , Bonsignore Justine , Carvalho Angelina
Published: February 2007

Volatile nitrites are drugs which have become widely used for their mood‐altering and aphrodisiac effects. Their use has been associated with methemoglobinemia and mild hemolysis; more significant hemolysis has been noted in a patient with G6PD deficiency. We report here a case of massive hemolysis following inhalation of volatile nitrites in the absence of an apparent intrinsic red cell defect. It is important that health care workers are aware of side effects from these commonly abused substances.
Bone marrow transplantation for good‐risk patients with leukemia in a university affiliated hospital
Authors: Verdirame Joseph D. , Bruckman James E. , Feagler John R. , Commers James R.
Published: February 2007

Of the first 14 patients with acute or chronic leukemia to undergo bone marrow transplantation at our hospital, 9 (64%), all good‐risk, are still alive in remission at 18 to 42 months of follow‐up (mean, 29 months) with their Karnofsky performance status between 80% and 100%. The conditioning regimen of fractionated‐dose irradiation and high‐dose chemotherapy eradicated their disease; only two patients relapsed after transplantation. Toxicity was acceptable. Acute graft‐versus‐host disease developed in six patients (43%) (grade I or II in four, grade IV in two) and progressed to chronic graft‐versus‐host disease in four. Viral pneumonitis developed in three patients (21%), but none had idiopathic interstitial pneumonitis. The mean hospital charge was $54,355. These preliminary results suggest that good‐risk patients with acute or chronic leukemia can be treated with bone marrow transplantation in a university affiliated hospital with appropriate staff and support facilities and achieve results comparable to those in research institutions at a competitive cost.