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At least 19 recordsLinked to original sources

Diagnosis of leukocyte disorders with an automated haematology counter (Technicon H-1 analyser).

The differential leukocyte count is very important in the diagnosis of various leukocyte disorders. Newer automated haematology counters can now perform differential leukocyte count, thus reducing the dependence on experienced medical technologists. The Technicon H.1 analyser (Technicon Instruments Corp., New York, USA) can perform even more complicated tasks like flagging abnormal cells, generating cytograms based on cell size and cytochemistry. In this paper we report on the useful cellular characteristics of the various leukocyte disorders as measured by the Technicon H.1 analyser.

Evaluation Studies as Topic↗

Leukocyte function and nonmalignant leukocyte disorders.

This review summarizes recent literature regarding the clinical and molecular features of nonmalignant leukocyte disorders in children. Leukocyte adhesion deficiency and chronic granulomatous disease, two inherited disorders of neutrophil function, continue to be the best-characterized disorders with respect to specific molecular defects. The underlying causes of severe congenital neutropenia and cyclic neutropenia remain elusive. Clinical features of immune-mediated neutropenias and their differential diagnoses are also reviewed.

Child↗

Retinal blood velocity in patients with leukocyte disorders.

The blue light entoptic phenomenon was used to measure retinal blood velocity in eight patients with chronic granulocytic leukemia, six patients with leukopenia, and matched control subjects. The retinal leukocyte velocity of the leukemic patients was 0.53 +/- 0.26 (mean +/- SD) mm/s, whereas that of the matched control subjects was 0.46 +/- 0.14 mm/s. There was no significant difference between these two groups (power: 96% for a difference of 0.2 mm/s and 66% for 0.1 mm/s). There was also no significant difference between the leukocyte velocities of the leukopenic patients and control subjects (0.47 +/- 0.19 mm/s and 0.55 +/- 0.14 mm/s, respectively; 89% power for a difference of 0.2 mm/s, 59% for 0.1). There was a correlation between the leukocyte count and the number of leukocytes seen in the entoptoscope. The results suggest that retinal vascular autoregulation can compensate for changes in leukocyte numbers that might have been expected to alter retinal blood flow.

Adult↗

Deletion of leukocyte mitochondrial DNA in bipolar disorder.

Leukocyte mitochondrial DNA (mtDNA) was examined in 35 patients with bipolar disorder by the nested PCR method to explore whether or not the 4977 base-pair deletion (common deletion) is found. The PCR product corresponding to the common deletion was found in 2 of 35 (5.7%) patients and none of 29 normal controls. It was confirmed by the primer shift PCR method that this PCR product was amplified from deleted mtDNA. These results suggest that more than a small percentage of patients with bipolar disorder might have deleted mtDNA and that this aberrant mtDNA might relate to pathophysiology of a subtype of bipolar disorder.

Adult↗

Leukocyte glutamate dehydrogenase activity in patients with degenerative neurological disorders.

Leukocyte glutamate dehydrogenase (GDH) activity was measured in 39 normal subjects, 32 neurological controls, 66 patients with progressive ataxic disorders, 32 with multiple system atrophy, 40 with Parkinson's disease, eight with Steele-Richardson-Olszewski syndrome, eight with juvenile Parkinsonism and four with the dystonia-Parkinsonism syndrome. GDH activity was reproducible to within 10% in leukocyte pellets stored at -70 degrees C for up to 9 months, and did not vary with sex or age in control subjects. There was marked variation in the relative proportions of heat stable and heat labile forms of GDH between control subjects and on repeated assay in the same subject. Total leukocyte GDH activity was similar in normal subjects and neurological controls. Mean total GDH activity was reduced in all patient groups by between 15 to 29% compared with controls. Fourteen patients had total GDH activity below 50% of the control mean, but low values were not specific for any one disease (five had ataxic disorders, four Parkinson's disease, three multiple system atrophy, one juvenile Parkinsonism, and one dystonia-Parkinsonism). The heat labile fraction of GDH represented about 20% of total activity in control subjects, and 27% in the patients with reduced total GDH activity. Thus low GDH activity was not disease-specific in this study, and the heat-labile GDH fraction was not selectively affected. "Reduced" leucocyte GDH activity in some patients may represent no more than the lower end of a normal distribution.

Adult↗

Disorders of leukocyte chemotaxis.

The rapid accumulation of inflammatory cells at sites of microbial invasion or neoplastic transformation is a central event in immunologically-mediated host defense. The availability of methodology to accurately quantify leukocyte migration in vitro has allowed the disclosure of previously unrecognized clinical disorders, namely leukocyte dysmotility syndromes. Although this area of clinical investigation is in its infancy, one can identify several processes associated with abnormal leukocyte accumulation. Abnormalities of immune recognition, chemotactic factor production, cellular motility or inhibitors of chemotaxis have been identified in different human diseases. In the upcoming years, pharmacological intervention directed at correcting specific causes of leukocyte dysmotility may well enhance our ability to treat certain infectious, inflammatory, and neoplastic diseases.

Antibody Formation↗

WHIM syndrome: a genetic disorder of leukocyte trafficking.

PURPOSE OF REVIEW: WHIM syndrome (the association of warts, hypogammaglobulinemia, recurrent bacterial infections, and 'myelokathexis') is a rare congenital form of neutropenia associated with an unusual immune disorder involving hypogammaglonulinemia and abnormal susceptibility to warts. In this review, we describe the clinical, laboratory and genetic features of WHIM syndrome. RECENT FINDINGS: The identification of chemokine receptor CXCR4 as the causative gene of WHIM syndrome yields new interest in the study of this disease as a model for the comprehension of CXCR4 biology in humans and highlights the importance of the chemokine network for inducing effective immune responses and governing leukocyte trafficking. SUMMARY: CXCR4 participates in several biological processes (bone marrow hematopoiesis, cardiogenesis, angiogenesis, neurogenesis) and is implicated in different clinical pathologic conditions (WHIM, HIV infection, tumor metastatization, autoimmunity). Pharmacologic agents that modulate CXCR4 expression/function are already available and promise a wide range of future clinical applications.

Abnormalities, Multiple↗

Genetic disorders of leukocyte function: what they tell us about normal antimicrobial mechanisms of human phagocytic cells.

Analysis of three inherited defects of granulocyte function (Chediak-Higashi Syndrome, CHS; Chronic Granulomatous Disease, CGD; Myeloperoxidase Deficiency, MPO) has highlighted critical events for the antimicrobial function of these cells and placed others in perspective. Prompt phagosomal fusion may be more important for digestion of organisms rather than killing as indicated by the mild bactericidal defects in the CHS. The formation of O2- and H2O2 during the phagocytic respiratory burst is central for the broad antimicrobial activity of granulocytes. MPO, on the other hand, while perhaps normally participating in granulocyte antimicrobial action, appears to be essential only for the effective killing of eukaryotic organisms such as certain fungal strains. While the non-oxidative killing mechanism of neutrophils have stimulated much recent interest and were the first to be defined no specific inherited defects have been discovered which are clinically important. Genetic disorders of macrophage effector function remain to be clearly defined as do those of eosinophils. The lessons learned from the study of the granulocyte defects discussed have provided both the technology and approach to the analysis of the antimicrobial and cytocidal mechanisms of these important phagocytic cells.

Blood Bactericidal Activity↗