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Biomedical subjects

J S Krauss

Publications and source records attributed to J S Krauss.

At least 19 recordsLinked to original sources

Clinical commentary: granulocytic fragments in sepsis.

Granulocytic fragments have been described in the peripheral blood of patients with sepsis and the systemic inflammatory response syndrome (SIRS). Although initially proposed as a morphologic clue for distinguishing the leukoerythroblastosis of sepsis from that of myelophthisis or marrow replacement by tumor, granulocyte-derived fragments may be part of a spectrum of cellular fragmentation associated with pathological inflammation and thrombosis, and thus play an important role in the pathophysiology of sepsis and SIRS. Pathologists, hematologists, and medical technologists should be aware of their existence, the morphologic features that distinguish them from macrothombocytes and schistocytes, and their potential significance.

Granulocytes↗

The proportion of hybrid heterodimers in homozygous or doubly heterozygous beta chain variant hemoglobinopathies associated with alpha chain hemoglobin variants.

Four alpha genes exist on chromosome 16, but one or more of these genes can be deleted in association with Hemoglobin (Hb)G-Philadelphia in cis to alpha-thalassemia-2 in African-Americans. Therefore, the proportion of HbG-Philadelphia in HbG heterozygotes is trimodal at about 25% for alphaGalpha/alpha alpha, 33% for alphaG-/alpha alpha, and 50% for alphaG-/alpha alpha in patients with HbA. Those who are homozygous or doubly heterozygous for beta chain variants (betaX2 or betaXbetaY) have neither HbA nor the alpha chain variant (alphaX2 betaA2), but have hybrid heterodimers (alphaX2 betaX2). The proportion of hybrid heterodimers here should also be trimodal mirroring alpha gene status. Eleven patients were identified: 4 with Hb SSG, 3 with Hb SCG, and 1 each with Hb OCG, HbSSMontgomery, HbSSChicago, and HbSSBourmedes. Heterodimer proportions were: 43.3 +/- 1.5, 33.5 +/- 2.3, and 15.8 +/- 1.1% for 2, 3, and 4 respective alpha genes which had been studied in 8/11 of the patients (r = 0.98), implying that the prime determinant of the proportion of hybrid heterodimers in this patient group is the number of functional alpha genes.

Dimerization↗

African-American factor VII-deficient variants in Georgia (FVII variants).

We studied African-American Factor (FVII)-deficient variants and carriers in Georgia by measuring their levels of FVII antigen (FVIIAG) and FVII procoagulant (FVIIC). Factor VIIAG was determined using enzyme-linked immunoassay (ELISA), whereas FVIIC was measured in two ways: 1) by fibrin clotting methods that employed human recombinant (HRFVIIC), human placental (HPFVIIC), rabbit brain (RBFVIIC), and bovine brain (BBFVIIC) thromboplastins; and 2) by an amidolytic method (AMFVIIC). Prothrombin time tests (PT) were also performed by standard methods. These 4 FVII-deficient patients and 3 carriers demonstrated the following results: PT: 18.2 +/- 6.5 sec; FVIIAG: 73.0 +/- 14.9%; HRFVIIC: 30.6 +/- 20.3%; HPFVIIC: 30.5 +/- 21.4%; RBFVIIC: 25.3 +/- 21.4%; BBFVIIC: 30.6 +/- 17.5%; AMFVIIC: 44.1 +/- 18.3%. We conclude that a group of clinically mild African-American FVII-deficient variants exists in Georgia. This group is characterized by the presence of FVIIAG and decreased FVIIC, using a variety of thromboplastins; and excellent correlation was noted for both human thromboplastins.

Antibodies↗

Failure of microchromatographic measurement of fetal hemoglobin in beta zero thalassemia-hereditary persistence of fetal hemoglobin.

We report microchromatographic measurement of fetal hemoglobin (HbF) proportions in a 36-year-old African-American multigravida woman. At 34 weeks she delivered a 630-g male infant who subsequently did well. Hemoglobin electrophoresis of the hemolysate revealed nearly 100% HbF without HbA, an extremely unusual naturally occurring sample. Family studies revealed a combination of hereditary persistence of fetal hemoglobin (HPFH) and beta zero-thalassemia minor. Southern blot technique confirmed heterozygous alpha 2 thalassemia and HPFH but failed to identify the beta thalassemic lesion. The absence of HbA and the very high amounts of HbF led us to measure HbF by several methods to confirm the accuracy of microchromatography of HbF at values approaching 100%. HPLC revealed a 14% F1 suggestive of microchromatographic underestimation due to glycated HbF. We conclude that cation-exchange microchromatography and the Betke method of alkali denaturation underestimate HbF values as they approach 100% and do not recommend these procedures in this rare situation.

Adult↗

Maternal coagulation inhibitors and the effects of cesarean delivery.

Maternal hypercoagulability in normal pregnancy results from significant increases in blood factors that promote thrombosis or decreases in factors that inhibit thrombosis, such as antithrombin III (AT-III) and proteins C and S. The precise role of these factors in puerperal hemostasis is not clear. In 10 normal, pregnant women at term undergoing scheduled repeat cesarean section, the percent activities of AT-III, proteins C and S, and C4b-binding protein were determined in peripheral venous blood preoperatively and in samples of uterine venous blood before the uterine incision was made and 5 and 15 minutes after placental delivery using the Laurell Rocket electroimmunodiffusion technique. The mean percent activities of AT-III (73%), protein S (81%) and C4b-binding protein (85%) were lower than those in nonpregnant controls, were similar in peripheral and uterine venous blood and were unchanged after placental delivery. These data suggest that such factors may not play an important role in acute uteroplacental hemostasis during normal pregnancy.

Antithrombin III↗

Stroke in a young adult with Fletcher trait.

We have reported the case of a young woman with prekallikrein deficiency who had a cerebral infarction despite a prolonged APTT. She had bleeding complications with heparin administration. From the limited information in the literature, anticoagulation may be hazardous in these patients, and it should be undertaken with caution.

Adult↗

Granulocytic fragments in sepsis.

We report here three patients with sepsis and one with acute pancreatitis and possible sepsis who developed granulocytic fragments on blood smears obtained prior to death. In case 1, these fragments were identified cytochemically. In case 3, granulocytic cytoplasmic projections and fragments were identified by electron microscopy of the buffy coat. All patients had leukerythroblastosis. The average corrected white blood count (WBC) was 46 X 10(9)/liter with 34 nucleated red blood cells (nRBC)/100 WBC. Patient 1 had thrombocytosis whereas patients 2, 3, and 4 were thrombocytopenic. Terminal complement levels were decreased in patients 3 and 4 as previously noted in sepsis (Sprung CL, Shultz DR, Marcial E, et al.: Complement activation in septic shock patients. Crit Care Med 14:525, 1986). A general correlation between nRBC and granulocytic fragments/100 hpf (high power field) was observed in patients 3 and 4. Granulocytic fragments were not identified on the blood smears of several patients with leukemoid reactions without erythroblastosis. Although the precise etiology of these fragments is unclear, we believe their recognition is important because all patients died within 32 hours after granulocytic fragments were identified. Furthermore, these fragments can falsely elevate the platelet count. Although myeloid fragments have previously been noted in leukemia and lymphoma, this is the first report of their association with conditions unrelated to hematologic neoplasms. These fragments can easily be recognized by careful examination of the blood smear and represent a newly recognized aspect of the septic shock syndrome.

Anemia, Myelophthisic↗

The vacuolated glycogen-laden leukemic lymphocytes of a T-cell lymphoproliferative disorder.

In this report, we present a case of peripheral T-cell leukemia/lymphoma having mostly small- to medium-sized cells with abundant clear cytoplasmic vacuoles. The presentation at the time of diagnosis was one of leukemia/lymphoma. The phenotype of the leukemic cells of the peripheral blood was T1+, T11+, TQ1+, interleukin-2+, T3-, T4-, and T8-. The cells in the peripheral blood as well as those obtained from lymph node biopsy were strongly periodic acid-Schiff positive; the positivity was diastase sensitive. Electron microscopy confirmed the presence of glycogen in the cytoplasmic vacuoles. Serologic tests were negative for human T-cell lymphotropic virus type 1 antibody. The data presented in this article support the existence of a vacuolated variant of peripheral T-cell leukemia/lymphoma and further expand the morphological spectrum of T-cell lymphoproliferative disorders.

Aged↗

Factor VII deficiency and pregnancy.

Hereditary factor VII deficiency is very rare in pregnancy (one in 500,000). However, obstetricians should consider this diagnosis whenever the prothrombin time is prolonged while the activated partial thromboplastin time is normal. The factor VII level increases in normal pregnancy, but the effect of pregnancy upon the factor VII level in deficient individuals is unknown. We report two cases of factor VII deficiency in pregnancy. In both, factor VII levels were 15% or less in the third trimester and were lower postpartum, suggesting that pregnancy does elevate factor VII in deficient individuals as well. Fresh frozen plasma is the treatment of choice. If the level is very low, fresh frozen plasma can be given prophylactically; otherwise, it should be given if blood loss becomes excessive.

Adolescent↗

Indirect antiglobulin test and anti-D prophylaxis in hereditary persistence of fetal hemoglobin.

The indirect antiglobulin test was used to assess the efficacy of anti-D prophylaxis in two Rh negative women with hereditary persistence of fetal hemoglobin. Saturation of all D sites by passive antibody should be followed by indirect antiglobulin positivity due to excess administered anti-D. In hereditary persistence of fetal hemoglobin, all red cells have fetal hemoglobin making the acid elution test (Kleihauer-Betke smear) to detect feto-maternal hemorrhage useless. The indirect antiglobulin test is recommended in patients with hereditary persistence of fetal hemoglobin.

Adult↗

Detection and quantitation of fetomaternal hemorrhage.

Failure to administer additional doses of Rh immune globulin (RhIG) to patients with excessive fetomaternal hemorrhage (FMH) is one of the causes of continued Rh isoimmunization. We compared the fetal cell ratio (FCR) with the other laboratory methods currently used at our hospital to quantitate FMH, ie, fetal cell preparation (FCP), RhIG cross-match with maternal serum, and indirect Coombs' testing 24 to 48 hours after administration of RhIG. The incidence of excessive FMH as detected by the various methods was 3.6% (cross-match), 9.6% (Coombs'), 18% (FCP), and 66% (FCR). The methods currently used do not accurately quantify FMH. The FCR is the most sensitive test but it has a high false-positive rate and thus does not appear to be clinically useful. We suggest that repeating the indirect Coombs' test may provide a practical alternative for determining the need for additional doses of RhIG.

Adolescent↗

Automated leukocyte differentials in trisomy 13.

We have reported a case of a 4-month-old girl who died with trisomy 13. Nuclear projections in polymorphonuclear leukocytes are diagnostically important in this syndrome and are not identified by automated leukocyte differential counting. The presence of polymorphonuclear leukocyte nuclear projections on the blood smear was identified only after the results of karyotyping were known. Recently, the importance of physician review of blood smears in anemia has been challenged. We believe this case, however, reaffirms the importance of physician blood smear review in suspected cytogenetic disease.

Adult↗

Identification of Giardia lamblia in peritoneal fluid.

Giardia lamblia, originally considered a commensal enteric organism, is now universally noted for its pathogenic potential in man. The mechanism of pathogenesis of giardiasis, however, remains unclear. We report a case of giardiasis diagnosed by analysis of peritoneal fluid obtained from a trauma victim. G lamblia trophozoite forms were found on microscopic examination of the fluid, and chemical studies of the fluid revealed bile and elevated amylase activity. The trophozoites may have entered the peritoneum through bowel perforation.

Ascitic Fluid↗

Densitometry and microchromatography compared for determination of the hemoglobin C and A2 proportions in hemoglobin C and hemoglobin SC disease and in hemoglobin C trait.

Using both densitometry and anion-exchange microchromatography, we measured hemoglobin C (Hb C) and Hb A2 proportions in 11 patients, eight of whom had Hb AC, two Hb SC, and one Hb CC. For one patient with Hb SC, we made the determinations before and after a transfusion. The mean (and SD) for the sum of Hb C + Hb A2 by densitometry and anion-exchange microchromatography for the nine patients with Hb AC and Hb CC were 45 (18) and 40 (18)%, respectively (p greater than 0.1, r = 0.98); for the three determinations involving the two Hb SC patients, the respective proportions were 40 (9.9) and 38 (6.6)% (r = 0.88). Electrophoretic analysis of microchromatographic eluates from the Hb AC and Hb CC patients showed that 6% of the absorbance of the late high-ionic-strength eluate was due to Hb C, which was responsible for the statistically insignificant difference between densitometric and chromatographic values for Hb C + A2 values. Electrophoresis on cellulose acetate of concentrated eluates of the Hb C + A2 fraction from the two Hb SC patients revealed no contamination by Hb S. Evidently, microchromatography can be used to determine Hb C + A2 in patients with Hb C or Hb SC disease or Hb C trait.

Anemia, Sickle Cell↗