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Lazy Leucocyte Syndrome-Disorder of the Granulocyte Membrane?

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Lazy Leucocyte Syndrome-Disorder of the Granulocyte Membrane? J Clin Pathol: first published as 10.1136/jcp.31.4.300 on 1 April 1978. Downloaded from Journal of Clinical Pathology, 1978, 31, 300-308 Lazy leucocyte syndrome-disorder of the granulocyte membrane? P. H. PINKERTON, JEAN B. ROBINSON, AND J. S. SENN From the Departments ofLaboratory Haematology and Medicine, Sunnybrook Medical Centre and Departments ofPathology and Medicine, University of Toronto, Toronto, Ontario, Canada SUMMARY An adult with long-standing neutropenia had the functional granulocyte abnormalities typical of the lazy leucocyte syndrome. Scanning electron microscopy of the patient's neutrophils showed alteration in the surface configuration of the cell with coarsening of the normal fine ruffles and the appearance of knob-like projections. Similar functional and anatomical changes were induced in normal neutrophils by treatment with vinblastine. The lazy leucocyte syndrome may be a consequence of altered membrane microfilamentous protein structure or function, and undue rigidity of the affected neutrophils may explain the clinicopathological features of the disease. Neutrophil microbicidal activity consists of a com- Rebuck and Crowley (1955), using a 12-mm diameter plex series of functions including migration, phago- round coverslip, replaced at two- or four-hourly cytosis of organisms, production of bactericidal intervals for a period of 20 hours, over an abraded substances and their release into the phagosome, and area on the volar aspect of the forearm. The cells digestion of the ingested material. A variety of adhering to the coverslips were stained with congenital and acquired disorders of these functions Romanowsky stain, examined by light microscopy, has been described (Baehner, 1974; Gallin and Wolff, and counted. Mobilisation of neutrophils from 1975; Miller, 1975; Quie, 1975), which may lead to marrow (Bishop et al., 1968; Cream, 1968) and an undue susceptibility to infection. One of the marginal pools (Athens et al., 1961; Joyce et al., http://jcp.bmj.com/ earliest congenital abnormalities of neutrophil 1976) was studied after stimulation with hydro- function to be reported was of defective mobility, cortisone sodium succinate (100 mg intravenously) both random and directed, associated with peripheral or adrenaline (0-5 ml of a 1/1000 dilution intra- neutropenia and normal numbers of mature neutro- muscularly) respectively. With hydrocortisone total phils in the bone marrow. The abnormality of and differential white cell counts were performed neutrophil movement was an intrinsic property of immediately before and hourly for six hours after the cells et This disorder has been (Miller al., 1971). administration. With adrenaline total and differential on October 2, 2021 by guest. Protected copyright. known as the 'lazy leucocyte syndrome' (LLS). We white cell counts were performed before and at 5, 15, describe a further case and report observations sug- 30, 45, and 60 minutes after administration. Random gesting that the pathophysiological basis of LLS lies mobility of leucocytes was measured according to in an alteration in the structure or function of the Bryant et al. (1966). microfilamentous protein of the granulocyte mem- For in-vitro studies of neutrophil function neutro- brane. phil suspensions were prepared from heparinised blood by collecting white cell-rich plasma after Methods sedimentation of red cells by adding a one-fifth volume of 6% dextran. After centrifugation of the Routine haematological investigations were carried white cell rich plasma the cell button was washed in out on a Coulter Counter Model S and by the Hanks's balanced salt solution (HBSS) and the cells methods of Dacie and Lewis (1975). Recruitment of were resuspended in HBSS at the required concen- neutrophils and monocytes to a site of inflammation tration. For experiments using vinblastine sulphate a was assessed by the 'skin window' technique of final concentration of 5 x 10-4M of the drug was used. Chemotaxis was studied by the methods of Boyden (1962), as modified by Clark and Kimball Received for publication 24 August 1977 (1971), with a 3-,u Millipore filter. Test and control 300 J Clin Pathol: first published as 10.1136/jcp.31.4.300 on 1 April 1978. Downloaded from Lazy leucocyte syndrome-disorder of the granulocyte membrane? 301 cell suspensions were adjusted to provide 5 x 106 The assay for granulopoietic colony formation in neutrophils/ml in the final suspension placed in the culture was similar to that of Iscove et al. (1971). upper chamber. Bone marrow cells were suspended in methyl cellulose Autologous and normal sera were activated with in alpha medium (Floro Laboratories) with 20% E. coli endotoxin (Difco Laboratories, Detroit, fetal calf serum in the presence or absence of 200 Mich, USA) and used as attractants in the lower leucocyte conditioned medium (LCM). After 14 days chamber. For studies with vinblastine (VBL) normal in culture at 37°C in a high humidity, 7.5% CO2 in neutrophils were incubated at 220C for 10 minutes air atmosphere granulocyte and macrophage colonies with 5 x 10-4M VBL before introduction to the were counted. Two plates were counted for each Boyden chamber. The chambers were incubated in group. In all experiments bone marrow cells were 5 % CO2 for three hours, when the filters were prepared as 'adherent' and 'non-adherent' (NA) recovered, stained, and examined for cells that had fractions before culture (Senn et al., 1974). passed through the membrane. Phagocytic capacity was examined according to El-Maalen and Fletcher (1976). Normal, patient, and vinblastine-treated cells Case report (see above) were incubated with Candida albicans at concentrations of 1 x 106/ml cells and 5 x 106/ml The patient, a 40-year-old Caucasian woman, was organism in HBSS at 370C. Aliquots were removed at referred for investigation of a neutropenia of at 3, 5, 10, 15, 20, 30, and 40 minutes, placed in cold least 13 years' duration. She gave a history of upper saline, centrifuged, and examined. After staining, respiratory tract infections; tonsillitis and repeated 800-1000 Candida were counted and the proportion sore throats (before and after tonsillectomy at 18 phagocytosed was expressed as a percentage. years of age); recurrent bouts of sinusitis treated with Bactericidal capacity was assessed using Staphylococ- antibiotics, antrostomy, and antrectomy; gingivitis; cus aureus 502A according to Quie et al. (1967). conjunctivitis; vaginitis; and pustular skin infections Mixtures oforganism and neutrophils were incubated since childhood. Her two brothers and her two at 370C for three hours. Samples were removed at children did not suffer unduly from infections. 20, 90, and 180 minutes, diluted, and used in a pour- Physical examination was unremarkable. plate of nutrient agar. Colony counts were per- The total leucocyte count was 1-5 x 109/1 (1500/ formed after 48 hours at 370C. Vinblastine-treated mm3) with 7 % neutrophils (0-105 x 109/1(105/mm3)). cells were tested both before and after washing to Absolute neutrophil counts subsequently were remove vinblastine, and little difference was seen in consistently less than 0 600 x 109/1 (600/mm3). The the results. bone marrow was of normal cellularity with normal http://jcp.bmj.com/ Candidacidal capacity was determined by the iron stores. Erythropoiesis was normoblastic. The method of Lehrer and Cline (1969) using patient, myeloid :erythroid ratio was normal (3:1) and a normal, and vinblastine-treated normal cells. Peroxi- differential count of 500 cells gave the following dase staining was carried out according to Kaplow proportions of neutrophils: myelocytes 18 %, meta- (1965) and nitroblue tetrazolium reduction was myelocytes 25-6y%, mature neutrophils and bands determined by the techniques of Park et al. (1968) 11-8y%. Megakaryocytes were unremarkable. Neu- and Park and Good (1970). trophil morphology in blood films and in bone mar- For scanning electron microscopy whole blood row was normal on light microscopy. Myelopero- on October 2, 2021 by guest. Protected copyright. from the patient and a normal individual was xidase activity and reduction of nitroblue tetrazolium layered over Hypaque-Ficoll and then centrifuged. were normal. The band of mononuclear cells was removed and the Tests of immunological function gave the follow- red cells and neutrophils were separated using ing results: Immunoelectrophoresis of patient's dextran, as described above. Normal and patient serum showed IgG 1476 mg/dl, IgA 225 mg/dl, neutrophils (> 95% pure) were resuspended in HBSS IgM 105 mg/dl (all within normal range), and IgE at a concentration of 5 x 106/ml. An aliquot of 375 IU (normal range 10-300 IU). Stimulation of normal cells was treated with vinblastine for two lymphocytes by phytohaemagglutinin, concanaval in hours at 37°C. The cells were then fixed in 1 % A, and pokeweed mitogen produced normal levels of glutaraldehyde in phosphate buffered saline (PBS), incorporation of tritiated thymidine. as described by Fujita et al. (1971). After fixation Investigations of in-vitro proliferation of granul- overnight at 4°C the cells were washed in PBS ocytes showed normal granulocyte and monocyte and dehydrated in graded alcohols. The final colony formation in both number and morphology. suspension in 100% alcohol was gold plated and Colony-stimulating activity (CSA) prepared from the examined on a Cambridge Stereoscan electron patient's leucocytes was capable ofstimulating colony microscope, Mark 2A (Kupari-Koby, 1976). formation (Table 1). J Clin Pathol: first published
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