Ordering folate assays is no longer justified for investigation of anemias, in folic acid fortified countries.

Background: Since 1998, in the countries where there is mandatory fortification of grain products with folic acid, folate deficiency has become very rare. Consequently, we decided to find out whether there is any justification for ordering folate assays for investigation of anemias.

Methods: We reviewed serum folate (SF) and red cell folate (RF) data at two teaching hospitals in Canada.

Hereditary high phosphatidylcholine hemolytic anemia: report of a new family and review of the literature.

Hereditary high phosphatidylcholine hemolytic anemia (HHPCHA) is a hematological disorder characterized by chronic hemolytic anemia with a dominant pattern of inheritance. The affected members show increased numbers of target cells and/or stomatocytes in peripheral blood smears, have reduced erythrocyte osmotic fragility, increased autohemolysis as well as markedly increased erythrocyte membrane Na+, K(+)-ATPase activity. Erythrocyte membrane phosphatidylcholine is increased but plasma levels of this phospholipid are normal.

Comparison of two APTT methods of monitoring heparin therapy. APTT ratio and heparin response of pooled normal plasma.

The sensitivity of the reagents for activated partial thromboplastin time (APTT) test varies greatly. Consequently the physicians who prescribe heparin based on certain APTT ratios may order different doses of heparin and produce different levels of anticoagulation in their patients, depending on the sensitivity of APTT reagent used by the laboratory. The authors have been recommending that physicians in their hospital use a therapeutic range for heparinization based on the sensitivity of the APTT reagent and keep the APTT of patients within the range of APTT of pooled normal plasma containing 0.

The variations between heparin sensitivity of different lots of activated partial thromboplastin time reagent produced by the same manufacturer.

The variations between different lots of activated partial thromboplastin time (APTT) produced by three major North American suppliers were evaluated over the past eight years. The authors found significant variations between the heparin sensitivity of the APTT reagents produced under the same name by the same supplier. The variations were so much that, using the recommended APTT ratio or prolongation of APTT for monitoring heparin therapy, one would have achieved significantly different intensity of heparinization from year to year.

Effect of heparin on platelet count and platelet aggregation.

The in vitro effect of heparin on platelet aggregation was studied in three groups: in 26 subjects recently treated with heparin, in 18 subjects on maintenance hemodialysis, and in 20 normal controls. With the aid of Technicon H6000, platelet counts and platelet aggregations were compared in whole blood samples collected in ethylenediaminetetraacetic acid (EDTA) and in heparinized tubes. Although there was no significant difference between platelet count of heparinized and EDTA blood in the control group, the dialysis group and the group recently treated with heparin showed significantly lower platelet counts and more platelet aggregation in heparinized tubes than in EDTA tubes.

The effect of toluidine blue and methylene blue in immunochemical reactions in vitro.

Our observation that the effect of a patient's autoantibody with a potent antithrombin activity could be inhibited by toluidine blue and methylene blue led us to investigate the effect of these dyes in several immunochemical reactions in vitro. Both dyes reduced the titer or prevented the detection of IgG-specific single- and double-stranded DNA-binding antibodies and rheumatoid factors but did not reduce the titer of antinuclear factor and had no effect on detection of microsomal and thyroglobulin antibodies. These dyes did not aggregate or precipitate the immunoglobulins and their inhibitory effect in serum could be removed by dialysis of serum.

Hematological aspect of Rh deficiency syndrome: a case report and a review of the literature.

The hematological aspects of the original case of Rhmod are reported. The subject, as in other reported cases, had a chronic hemolytic anemia characterized by stomatocytosis, reduced osmotic fragility, and abnormal autohemolysis correctable with the addition of glucose. The 51Cr red cell survival studies showed the spleen to be the preferential site of red cell destruction and splenectomy produced a dramatic improvement in red cell survival.

An autoantibody with potent antithrombin activity whose action could be inhibited by toluidine blue or methylene blue.

A 41-year-old man with posthepatic liver cirrhosis and bleeding tendency was found to have a circulating anticoagulant with potent antithrombin activity. The thrombin time of the patient's purified fibrinogen was normal. This anticoagulant was residing in the patient's IgG fraction, and his IgG fraction could prolong the thrombin time of purified fibrinogen, suggesting that this circulating anticoagulant was an antibody.

Disseminated intravascular coagulation in lupus erythematosus responding to prednisone therapy.

A 48-year-old man with systemic lupus erythematosus (SLE) developed disseminated intravascular coagulation (DIC) with clinical bleeding. Since no other cause for DIC could be demonstrated, he was treated with prednisone, which rapidly corrected his DIC. This case demonstrates the need for a complete hematological investigation of patients with SLE who present with hemostatic abnormalities.

Isolated granulocytic sarcoma: report of a case and review of the literature.

Granulocytic sarcoma (GS) is an extramedullary tumor composed of granulocytic precursor cells. The tumor usually develops during the course of myelogenous leukemia or myeloproliferative disorders and may represent the initial manifestation of leukemia. Rarely, GS is recognized as an isolated tumor without any evidence of leukemia.

Folic acid and vitamin B12 deficiency in pregnancy and in the neonatal period.

The fetus, the neonate, and the pregnant woman have a greater requirement for folic acid and vitamin B12 and are more likely to suffer from a deficiency of these vitamins. This article reviews the source, requirement, absorption, and metabolism of these vitamins and discusses the problems attributed to their deficiency in pregnancy and in the neonatal period.

Aortocolic fistula: a rare cause of profuse rectal bleeding.

A 62-year-old man with multiple myeloma survived for 19 months following massive hemorrhage from an aortocolic fistula secondary to an aortoiliac aneurysm that was adherent to the sigmoid colon. The literature indicates that early death from multisystem failure is to be expected and that sepsis is an inevitable consequence of aortocolic fistula. However, in this case the patient did not die despite multisystem complications and his eventual death was unrelated to sepsis or graft complications.

Hereditary lecithin-cholesterol acyltransferase deficiency and Bloom syndrome in the same individual.

A couple who were first cousins had three children: an older son with Bloom syndrome (BLS) and homozygous lecithin-cholesterol acyltransferase (LCAT) deficiency; the second child (a son) and the parents are LCAT deficiency and the youngest child (a daughter), is homozygous for LCAT deficiency. The use of genetic markers gave no evidence of linkage of BLS and LCAT loci.

Hereditary lecithin-cholesterol acyltransferase deficiency. Report of 2 new cases and review of the literature.

Two new cases of hereditary lecithin-cholesterol acyltransferase (LCAT) deficiency in a brother and sister born to consanguinous parents are reported. Both have corneal opacity, splenomegaly and mild hemolytic anemia. The brother, the older of the 2, also has significant proteinuria.

Hereditary plasma lecithin-cholesterol acyl transferase deficiency: a heterozygous variant with erythrocyte membrane abnormalities.

LCAT esterifies cholesterol to cholesterol ester. This study describes a family with plasma LCAT deficiency with a heterozygous variant. It was found that both parents and one son had half the normal plasma LCAT activity whereas their only daughter and other son exhibited zero plasma LCAT activity, suggesting an autosomal co-dominant inheritance of the enzyme deficiency.

Oral contraceptives: effect of folate and vitamin B12 metabolism.

Women who use oral contraceptives have impaired folate metabolism as shown by slightly but significantly lower levels of folate in the serum and the erythrocytes and an increased urinary excretion of formiminoglutamic acid. The vitamin B12 level in their serum is also significantly lower than that of control groups. However, there is no evidence of tissue depletion of vitamin B12 associated with the use of oral contraceptives.