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Biomedical subjects

W J Thomas

Publications and source records attributed to W J Thomas.

At least 37 records · Page 2Linked to original sources
PubMed1989

Emergency use of uncrossmatched red cells in neonatal intensive care infants.

In very rare situations, a newborn will be delivered with severe unrecognized life-threatening anemia. Two infants with severe anemia with differing etiologies were deemed to require emergency uncrossmatched O-negative blood. In one infant, the blood transfused was incompatible for the minor antigen that had caused the isoimmune hemolytic anemia. We report on the indications, potential risks and benefits in giving uncrossmatched O-negative blood to term infants with life-threatening anemia.

Blood Grouping and Crossmatching↗
PubMed1986

Castleman's disease in children.

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph. His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castleman's disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 we could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients.

Child↗
PubMed1985

Severe congenital neutropenia with unique features of dysgranulopoiesis.

Congenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3-year-old girl with neutropenia (less than 500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30-50 microns) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly, immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony-forming cells, and normal colony-stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.

Agranulocytosis↗
PubMed1982

Morphologic observations of contact-induced lysis of EBV-infected B lymphocytes by autologous hand mirror T cells.

To study the possible immunologic role of hand mirror lymphocytes (HML) in the control of Epstein-Barr virus (EBV)-infected B lymphocytes in patients with infectious mononucleosis (IM), we studied the in vitro interactions of these cells by time-lapse video light microscopy. Peripheral blood T lymphocytes isolated from three patients in the early recovery phase of IM were mixed with their autologous EBV-infected B cells. Motile lymphocytes with their characteristic hand mirror shape were observed to attach by their uropods to the B cells. The HML remained attached for variable periods ranging from 45-75 min. Following detachment, B cells that were in contact with HML underwent lysis. Mixtures of T cells from healthy donors and EBV-infected cell lines exhibited only rare uropod formation with no attachment or lysis of B cells. The present experiment indicates that contact-induced lysis of EBV-infected B lymphocytes is operative in IM and that this process is mediated by the HML.

B-Lymphocytes↗
PubMed1982

Comparison of venipuncture blood counts with microcapillary measurements in screening for anemia in one-year-old infants.

The microhematocrit measurement of fingerstick blood in infants gives elevated values compared to venous hematocrits measured simultaneously in a Coulter Model S electronic counter. Thirty one-year-old infants had a mean microhematocrit of 36.6, significantly higher (p less than 0.001) than the mean venous Coulter S hematocrit of 34.6. Three children, who appeared to be normal by microhematocrit measurement, were found to be anemic by Coulter S measurement; in these children the microhematocrits were elevated 13.6%, 12.5%, and 5.1%, respectively, above venous Coulter S levels. Complete blood counts were measured concurrently on venipuncture specimens and on capillary samples. There was excellent correlation for mean cell volume (r = 0.98), but poor correlation for hemoglobin (r = 0.81) and hematocrit (r = 0.77). Four of 30 children had borderline low venipuncture hemoglobin values (less than or equal to 11.5 gm/dl) that were not detected by the capillary method. The inaccuracy of the fingerstick microhematocrit method and the discrepancy between the venipuncture and capillary Coulter S values supports the choice of venipuncture blood counts as the preferred method of screening for anemia in one-year-old infants.

Anemia↗
PubMed1981

Successful treatment of metastatic extragonadal endodermal sinus (yolk sac) tumor in childhood.

Two children with metastatic extragonadal endodermal sinus tumor who had failed VAC chemotherapy were treated with combination chemotherapy consisting of cis-platinum, vinblastine, doxorubicin HCl (Adriamycin) and bleomycin (cis-VAB). There was a complete response in both patients with one child remaining tumor-free 3 1/2 years after metastasis and 2 1/2 years after stopping therapy. The main toxic reactions encountered were associated with the use of cis-platinum, but these were manageable and resolved upon completion of therapy. Cis-VAB appears to be a safe and effective regimen for the treatment of metastatic endodermal sinus tumor. A study of the efficacy of cis-VAB in a larger group of children with endodermal sinus tumor of poor prognosis seems indicated.

Adolescent↗
PubMed1981

Chronic myelomonocytic leukemia in childhood.

A four-year-old child with recurrent infections and increasing hepatosplenomegaly over a three-year period was evaluated. Increased numbers of myeloid precursors packed the bone marrow and infiltrated the peripheral blood. A diagnosis of chronic myelogenous leukemia (CML) was considered but could not be confirmed by laboratory studies appropriate for the types of CML usually observed in childhood. Examination of the patient's peripheral blood smears revealed many atypical monocytoid cells with unipolar hairy projections. Scanning electron microscopy showed these to be leukemic monoblasts with characteristic broad-based ruffles on the cell surface. A population of myeloid precursors possessing narrow ridge-like profiles was also observed. Progressive infiltration of the spleen caused hypersplenism which necessitated splenectomy. Subsequently, massive liver and bone marrow involvement led to the patient's death. Terminally, the proliferating blast cells were demonstrated to be leukemic monoblasts by analysis of cytochemical staining patterns, surface immunoglobulins, serum lysozyme levels, and monocyte-mediated antibody-dependent cellular cytotoxicity studies. The findings in this case are most compatible with a diagnosis of chronic myelomonocytic leukemia (CMML), a condition not previously described in childhood. Several myeloproliferative disorders with prolonged survival have been reported in children, but special studies were not performed to determine which cell lines were abnormally proliferating. The similarities between these children and our patient with CMML suggest that monocyte studies may be useful in the diagnosis of these unusual disorders, provide insights into their pathogenesis, and aid in the selection of appropriate therapy.

Antibody-Dependent Cell Cytotoxicity↗
PubMed1981

Acute lymphoblastic leukemia--hand mirror variant--viral immune interrelationship as demonstrated by ultrastructural studies.

Acute lymphoblastic leukemia--hand mirror variant--was extensively restudied in a 22-year-old white female who survived for 22 months without therapy. Immune complexes to the baboon endogenous virus (BaEV) were found in the bone marrow plasma of the relapse specimen in 1977, but not in the bone marrow plasma from the terminal state in 1979. Immunoperoxidase-tagged IgM antibody prepared from the patient's bone marrow plasma revealed BaEV antigen on the tip of the uropod of the HMC at the time immune complexes were found in the marrow. Absence of immune complexes in the marrow. Absence of immune complexes in the bone marrow in the terminal state suggested a failure of the patient's immune surveillance system and/or possible immune suppression by chemotherapy.

Adult↗
PubMed1981

Pseudoleukemia. Ineffective regulation of granulopoiesis.

A neonate with Down's syndrome confirmed by chromosomal analysis had leukocytosis, with a WBC count of 48,000/cu mm, at age 13 days. Differential counts showed 70% to 90% blast forms by light microscopy. The blast-cell granules stained negatively with Sudan black B and alpha-napthyl butyrate esterase. Myeloperoxidase activity evaluated light and electron microscopy was negative. The granules stained positively with PAS, beta-glucuronidase, and tartrate-sensitive acid phosphatase. Indirect immunofluorescence for terminal-deoxynucleotidyl-transferase was negative. Our results show that the blast cells of pseudoleukemia may be of lymphoid origin.

Bone Marrow Cells↗
PubMed1980

Infectious mononucleosis and acute lymphoblastic leukemia--hand mirror cells: a qualitative and quantitative ultrastructural study.

The hand mirror cell (HMC) in infectious mononucleosis (IM) is a T cell which appears at the time of improvement and apparently has an important role in immune surveillance. Fifty electron micrographs of HMC from an unusual untreated patient with ALL-HMC variant were compared to 50 electron micrographs of HMC from five patients beginning to recover from IM. Quantitative differences between the IM-HMC and ALL-HMC were observed in multivariate analysis of variance (p < 0.001), nuclear perimeter (p < 0.0001), nuclear length (p < 0.0001), cytoplasmic length (p < 0.0001), total cell area (p < 0.0001), cytoplasmic area (p < 0.0001) and nuclear cytoplasmic ratio (p < 0.004), and numbers of mitochondria (p < 0.001). The qualitative differences included greater nuclear irregularity in the IM-HMC, and prominent nucleolonema in some of the ALL-HMC. In addition, the IM-HMC showed more polyribosomes, parallel tubular arrays and microtubules, but much less glycogen. Since the IM-HMC apparently enter effectively into immune surveillance, and some cases of ALL have HMC, the ultrastructural similarities and differences above contribute to a better understanding of the importance of this unique cell configuration in benign and malignant disorders.

Genetic Variation↗
PubMed1980

Acute lymphoblastic leukemia: hand mirror variant.

The presence of numerous hand mirror cells in the bone marrow of patients with acute leukemia may be related to a good prognosis. To further evaluate this relationship, the bone marrows of 21 consecutive patients with a diagnosis of acute lymphoblastic leukemia were reviewed for the presence of greater than 40% hand mirror cells. Four of these 21 patients (19%) demonstrated this finding during the course of their illness. These patients were characterized by ease of remission and/or prolonged survival. Three (75%) patients are alive and well at the time of this writing. At the present time, of the 17 patients without hand mirror cells, seven (41%) are alive and ten (59%) are dead. Although not statistically significant, our findings suggest a positive relationship between the presence of greater than 40% hand mirror cells in the bone marrow ans a favorable prognosis. Future larger prospective studies are indicated to further understand the importance of this complex, apparently immunologically stimulated cell.

Adult↗
PubMed1980

Hand-mirror lymphocytes in infectious mononucleosis.

The identification of a morphologically unique lymphocyte in the peripheral blood of patients with infectious mononucleosis (IM) led to additional studies. Examination of Wright-stained smears of EDTA anticoagulated blood obtained from 25 patients with IM at presentation revealed significantly increased (p less than 0.0001) percentages of hand-mirror lymphocytes (HML) (mean 9.2%) compared with normal controls (mean 2.7%) or controls with nonspecific viral syndromes (mean 2.2%). Follow-up blood samples obtained on 10 of these patients demonstrated a marked increase in the HML count (mean 12.1%) that coincided with the onset of recovery. E-rosette separation of Ficoll-Hypaque-derived peripheral blood lymphocytes from 5 patients with early recovery IM showed the HML to be present almost exclusively in the T-cell population, representing about 25% of the T cells. Identical procedures on 5 controls showed less than 5% HML in the T-cell sample. Cytochemistries supported a T-cell derivation for HML. Electron microscopic examination of HML in IM demonstrated that these cells have unique ultrastructural features that may be related to functions of cellular attachment and cytotoxicity.

Humans↗
PubMed1979

Acute lymphoblastic leukemia--hand mirror variant. An analysis of a large group of patients.

One hundred and thirty-four initial bone marrows and 102 peripheral blood smears were evaluated for hand mirror cells (HMC) on pediatric patients with acute lymphoblastic leukemia (ALL). Twenty (15%) of the 134 patients were found to have greater than 5% HMC in their bone marrows. Only rarely were a few HMC noted in the peripheral blood. Survival did not appear to be related to the number of HMC in the bone marrow. Also, the median survival was not significantly greater in those HMC patients with greater than 40% HMC in their bone marrows when compared to the non-HMC group. From the available data in this study, it would appear that the number of HMC does not affect prognosis in the bone marrows of children with ALL. However, the prognostic significance of the HMC has not been clearly established in adults.

Adolescent↗
PubMed1979

Persistence in humans of antibody after immunization with four alphavirus vaccines.

Immunization with attenuated VEE virus vaccine resulted in persistence of neutralizing antibody for 12 years. Immunization with inactivated WEE vaccine converted 83% of the subjects, killed EEE vaccine converted 27% and killed Chikungunya vaccine induced no significant titers. Antibody formed as a result of immunization with inactivated vaccines was of short duration, i.e. less than 1 year. Attenuated VEE was responsible for some heterologous antibody rises to the other three alphaviruses. Among the inactivated vaccines WEE and Chikungunya vaccines produced one heterologous rise each to EEE virus.

Alphavirus↗