American Journal of Hematology

Pagliaro, Pasqualepaolo; Arrigoni, Luisa; Muggiasca, Maria Luisa; Poggio, Marina; Russo, Umberto; Rossi, Edoardo

doi: 10.1002/(SICI)1096-8652(199609)53:1<6::AID-AJH2>3.0.CO;2-Vpmid: 8813089

Pregnancy in patients with primary thrombocythemia (PT) is reported to be often complicated by recurrent abortion and fetal growth retardation. Fifteen pregnancies in nine patients with PT are reported. Nine pregnancies had a good outcome, with the birth of a healthy infant. There were two spontaneous abortions and three intrauterine deaths. One pregnancy was electively terminated after extensive thrombosis in the splanchnic district requiring surgical entero‐resection. In five pregnancies the mother received no treatment; in ten pregnancies acetylsalicylic acid (ASA) was prescribed to the mother as soon as she was found pregnant, subcutaneous heparin was added from the middle trimester in seven cases. In patients treated with ASA and subcutaneous heparin pregnancies had a good outcome. Administration of ASA and heparin during pregnancy appears to improve the outcome in patients with PT and can prevent severe maternal complications, but requires close monitoring. © 1996 Wiley‐Liss, Inc.

Chang, Jae C.; Shipstone, Ash; Llenado‐Lee, Marian A.

doi: 10.1002/(SICI)1096-8652(199609)53:1<11::AID-AJH3>3.0.CO;2-8pmid: 8813090

Among 47 patients with thrombotic thrombocytopenic purpura (TTP), 8 patients were diagnosed to have postoperative‐TTP. Two patients underwent vascular surgery, 5 patients coronary artery bypass grafts, and 1 patient resection of myocardial sarcoma. Prior to surgery, all patients except one had normal hemograms and platelet counts, and blood smears showed no schistocytes. Five to 19 days after surgery, all 8 patients developed postoperative TTP, which clinical feature was characterized by unexplained progressive encephalopathy, thrombocytopenia, and microangiopathic hemolytic anemia. In addition, in 3 patients, progressive gangrene of the toes also developed. Four patients achieved complete remission following exchange plasmapheresis and 1 patient spontaneous remission. Due to complicated surgical settings after surgery, recognition of TTP was often delayed and it contributed to death in 3 patients despite treatment with exchange plasmapheresis. In view of occurrence of postoperative TTP following cardiac and vascular surgeries, pathogenic mechanism for postoperative TTP may be explained on the basis of injury of diseased endothelial surface and release of a humoral factor(s) that results in platelet aggregation in the capillaries and arterioles. Our experience with these cases indicates that TTP may occur as a serious complication of cardiac and vascular surgeries, and early recognition of the diagnosis and institution of exchange plasmapheresis are of paramount importance for favorable outcome. © 1996 Wiley‐Liss, Inc.

Fuse, Shigeto; Tomita, Hideshi; Yoshida, Masaki; Hori, Tsukasa; Igarashi, Chiharu; Fujita, Shigeru

doi: 10.1002/(SICI)1096-8652(199609)53:1<18::AID-AJH4>3.0.CO;2-8pmid: 8813091

In several animal experiments, high doses of antithrombin III concentrates have shown beneficial effects on mortality and reversal of coagulation abnormalities which had resulted from disseminated intravascular coagulation. Other experiments have suggested that antithrombin III infusion without heparin is effective in the treatment of organ failure. We clinically treated children suffering disseminated intravascular coagulation only with antithrombin concentrate. Four patients suffering disseminated intravascular coagulation with organ failure were selected. We started antithrombin III concentrate infusion as soon as the diagnosis was established. The dosage of antithrombin III was 120–250 units/kg/day for 2 or 3 days. Heparin was not used. All 4 patients recovered completely and quickly without any complications within 14 days. We suggest that the high‐dose antithrombin III infusion without heparin is an effective and safe therapy for disseminated intravascular coagulation with organ failure. © 1996 Wiley‐Liss, Inc.

Nakakuma, Hideki

doi: 10.1002/(SICI)1096-8652(199609)53:1<22::AID-AJH5>3.0.CO;2-7pmid: 8813092

Paroxysmal nocturnal hemoglobinuria (PNH) hemolysis requires both intravascular complement activation and affected erythrocytes susceptible to complement. This susceptibility is explained by a deficiency in complement regulatory membrane proteins that are attached to the membrane by a glycosylphosphatidylinositol (GPI) anchor. Affected cells lack a series of GPI‐anchored membrane proteins with various functions. The lack is caused by a synthetic defect of the anchor due to an impaired transfer of N‐acetylglucosamine to phosphatidylinositol which is an early metabolic precursor in the anchor synthesis. Moreover, PIG‐A gene responsible for the membrane defect was recently cloned. Further, a possible mechanism of complement activation has been proposed, especially for an infection‐induced hemolytic precipitation which is clinically crucial. Thus, the molecular events, leading to intravascular hemolysis characteristic of PNH, has been virtually clarified. Next major concern is the nature of PIG‐A: How does PIG‐A explain the complex pathophysiology of PNH which exhibits various clinical manifestations? © 1996 Wiley‐Liss, Inc.

Yufu, Yuji; Sadamura, S.; Ishikura, H.; Abe, Y.; Katsuno, M.; Nishimura, J.; Nawata, H.

doi: 10.1002/(SICI)1096-8652(199609)53:1<30::AID-AJH6>3.0.CO;2-6pmid: 8813093

The EVI1 DNA‐binding protein gene on chromosome 3q26 has been reported to be activated in some leukemia cells with alterations in 3q26. We present an acute myelogenous leukemia (AML) patient with a rare chromosomal translocation, t(3;13)(q26.2;q13–14). By reverse transcription‐ polymerase chain reaction, we detected active transcription of the EVI1 gene in the patient's leukemia cells. The retinoblastoma susceptibility (Rb) gene, a tumor‐suppressor gene, is located at chromosome 13q14 and is within the other translocation breakpoint in this patient. The expression of the Rb gene product was found to be substantially decreased in the patient's leukemia cells by Western blotting. Southern blot analysis, however, revealed no gross abnormalities of the Rb gene. Although it is unlikely that the Rb gene is directly involved in this translocation, the loss of the Rb gene product combined with the activation of the EVI1 gene may have led to the development of leukemia. © 1996 Wiley‐Liss, Inc.

Corey, Seth J.; Beutler, Ernest

doi: 10.1002/(SICI)1096-8652(199609)53:1<35::AID-AJH7>3.0.CO;2-6pmid: 8813094

A new mutation of the G‐6PD gene at position 910 is described. Biochemical analysis suggests that a conformational change results in the enzymatic deficiency associated with G‐6PDWest Virginia. © 1996 Wiley‐Liss, Inc.

Gurgey, Aytemiz; Mesci, Lutfiye; Renda, Yavuz; Olcay, Lale; Kocak, Nurten; Erdem, Gulsen

doi: 10.1002/(SICI)1096-8652(199609)53:1<37::AID-AJH8>3.0.CO;2-6pmid: 8813095

The factor V Leiden mutation in 12 children with thrombosis and in 20 controls was investigated. Five heterozygous individuals and 1 homozygous individual among the cases with thrombosis and 1 heterozygous individual among controls were found. Central nervous system thromboses were increased in children with the factor V mutation, associated with protein S deficiency. © 1996 Wiley‐Liss, Inc.

Pasqualetti, Paolo; Collacciani, Antonio; Casale, Raffaele

doi: 10.1002/(SICI)1096-8652(199609)53:1<40::AID-AJH9>3.0.CO;2-5pmid: 8813096

The diurnal rhythm in the circulating serum levels of erythropoietin (EPO) were determined in a group of 20 adult clinically‐healthy subjects, in a group of 10 patients with myeloma without renal impairment and 10 patients with myeloma and renal failure. Venous blood samples were drawn during the span of a whole day and every 4 hr, starting from midnight, for the measurement of serum EPO levels by radioimmunoassay (RIA). Statistical analysis was carried out by means of the “cosinor” method. Results show that the controls and the myeloma patients without renal insufficiency present significant (P < 0.05) circadian rhythms in serum EPO levels; no rhythm (P < 0.05) was detected in patients with myeloma and renal failure. Patients with myeloma and renal failure have significant (P < 0.05) lower mean daily levels and diurnal fluctuations of EPO than the other groups, whereas the patients with myeloma without renal involvement present higher (P < 0.05) mean daily levels and lower (P < 0.05) diurnal variations of EPO than controls; no differences (P > 0.05) exist between the groups regarding peaks of rhythms. These data confirm the existence of a physiological circadian rhythm in serum EPO concentrations, with maximum in the afternoon, and they suggest that renal failure is an important cause of anemia and loss of EPO circadian rhythm in patients with myeloma. © 1996 Wiley‐Liss, Inc.